:: LMS Chat ::

:: Leiomyosarcoma (LMS) Defined ::

Leiomyosarcoma (Gr. Leio- =smooth, myo- = muscle, sarcoma = fleshy growth) is a type of sarcoma which is a neoplasm of smooth muscle. (When a uterine neoplasm is benign, it is a leiomyoma.) Smooth muscle cells make up the involuntary muscles, which are found in most parts of the body: in uterus, stomach and intestines, walls of all blood vessels, and skin. It is therefore possible for leiomyosarcomas to appear at any site in the body. It is however most commonly found in the stomach, small intestine and retroperitoneum.

Leiomyosarcoma is a very rare cancer. It makes up 5-10% of soft tissue sarcomas, which are in themselves rare cancers.

:: About Leiomyosarcoma ::

Leiomyosarcoma is a form of cancer that affects the smooth muscle of the body. It spreads through the blood stream and can affect the lungs, liver, blood vessels, or any other soft tissue in the body.

LMS is a type of sarcoma which is a neoplasm of smooth muscle. Smooth muscle cells make up the involuntary muscles, which are found in most parts of the body: in the uterus, stomach and intestines, walls of all blood vessels, and skin.

Leiomyosarcoma is a very rare cancer. It makes up 7% of soft tissue sarcomas; in all, LMS affects 4 out of 1,000,000 people.

Presently there is no cure. Remission can be attained, but this rare cancer can reappear at any time. Because of its rarity, few doctors know how to treat it and it attracts very little research.

Monday, September 21, 2009

Jordan to have cancer scan

Celebrity Spy | pictures | now magazine | celebrity gossip
Jordan is not looking forward to the cancer tests

Glamour model advised to get checked out after previous scare

Saturday, 19 September 2009

Jordan is to have tests to see if she has cancer.

The glamour model - real name Katie Price - had leiomyosarcoma, a form of the disease that attacks the smooth muscle cells, removed from her finger in 2002.

‘I'm not looking forward to it,' says Jordan. 'It takes three hours and they inject you with iodine.

‘The doctor said I should have one because the cancer I had before was linked to stress and because I'm stressed again he told me to get it done.'

But Jordan, 31, plans to keep quiet about the results.

‘I'm not going to say any more about it,' she writes in her OK! diary.

‘I know people will twist it and say I'm talking about it to get sympathy or something.'

Saturday, August 29, 2009

‘Better Than Ever’ looking for new ‘Sole-Mates’

By Mike Touzeau, Special to the Green Valley News
Published: Tuesday, August 25, 2009 4:14 PM MST
You probably know someone who has battled cancer — perhaps even someone who lost.

The disease affects virtually everyone, directly or indirectly, which is why “Better Than Ever” (BTE) begins its 10 year at the forefront of a local campaign that has attracted hundreds of participants while raising hundreds of thousands of dollars for the Arizona Cancer Center.

It’s likely most people in the Green Valley/Sahuarita area have already heard about the BTE “Sole-Mates,” a group that continues to promote fitness as prevention for cancer, but may not be aware they too can join them in the fight.

Leaders Suzie Cherry, Barbara Powell, Sandy Bird and Kathy Wong are once again inviting anyone who wants a chance to join their army of “battling BTE’s” to enjoy their kickoff orientation Tuesday, Sept. 1, at 5:30 p.m. at Arizona Family Restaurant in Green Valley.

Training sessions in a “welcoming, non-competitive atmosphere” begin a week later, so it’s an opportunity for those who want to “have fun, be fit, and fight cancer,” as their brochures state, while at the same time get a chance to meet some courageous and dedicated supporters of today’s cutting edge treatments and tomorrow’s cures.

Virtually every “Sole-Mate” has lost someone to cancer, and that reality was driven home with even more agonizing certainty when one of their most active members succumbed to leiomyosarcoma in March.


“She fought a long, hard battle,” said Wong of her good friend Chris Portmann, who was only 58. “She was the epitome of living with her cancer, and living life to the fullest.”

Portmann finished the 2007 Tucson half-marathon, one of BTE’s major fund-raising events, while struggling with the disease — no small feat.

“Unfortunately, she was one of those in a million who get this type of cancer,” Bird said, “and she was one-in-a-million as a person.”

Bird had never been in any kind of fitness or walking program before she joined the group last year about this time, but because of the support from her training leaders and friends, she was able to finish the half-marathon in 2008.

“I didn’t think I would ever make it,” she admits, but she recalls the times that people just like Portmann encouraged her to keep going when she wanted to turn back, which is why she wants to continue to participate to remember her friend.

“It was first in her honor,” Bird said, “and now it’s in her memory.”

BTE trains participants in a 90-day program to run or walk their choice of events and distances, which include the CATwalk 10k (6.2 miles) on Saturday, Nov. 14, the Tucson half-marathon of 13.1 miles on Sunday, Dec. 13 (a Catalina State Park event in April and a Cinco de Mayo event), or they can cycle in the El Tour de Tucson Saturday Nov. 21.

Biking was just added three years ago to the BTE program in Tucson.

“We’d really like a biking group to get started in Green Valley/Sahuarita with BTE,” Wong said.

BTE’s program is designed for walkers, runners, and cyclists of all ages, and they include group workouts, coaching, a lecture series on healthy living, social functions, and a weekly newsletter with tips and information.

All money raised goes directly to research grants for the Arizona Cancer Center.

BTE raised $100,000 in 2008-09, Wong said.

The training sessions start every Saturday from either the Desert Hills or Santa Rita Springs recreations centers, she said, adding that they all head out for breakfast afterward.

“It’s a great source of camaraderie for the women,” she declared, “and we’d like to have more men out there.”

Since August of 2000, BTE has raised more than $1,000,000 and 2,800 participants of all ages have crossed the finish line — like Alice Slater, 79, a breast and colon cancer survivor who still does most of the events.

“I’m so grateful that I’m able to do this at all, considering how my health has been,” Slater said.

If you would like to join in the fight, Wong said, just call her at 625-0159 or Sandy Bird at 399-1511, and you can get more information at www.arizonabte.org.

Mike Touzeau is a freelance writer for the Green Valley News.

Co-workers plan benefit for man who lost arm to cancer

By Christina Chapman - cchapman@morrisdailyherald.com

A local retailer’s employees are banding together to help a sick co-worker. They are hoping the community will join their efforts.

Ryan Lechtenberg is expected to return to work this week at a local big-box hardware store after recovering from surgery earlier this month to have his left arm amputated.

Lechtenberg has been battling a very rare form of cancer, called Leiomyosarcoma, for more than 3 years.

After undergoing at least five surgeries, continuous chemotherapy and radiation therapy, Lechtenberg finally had to have his arm amputated in hopes of preventing the cancer from spreading, said Dan Lupton, co-worker and one of the benefit organizers.

“Through past surgeries, they were able to save his arm, but the tumor kept growing,” Lupton said.
Lechtenberg has about six inches left of his arm, Lupton said. He is expected to be able to continue work, but will need assistance.

Because Lechtenberg’s insurance does not cover all of his treatment, his co-workers are trying to raise funds to help him and his family. Lechtenberg is 37 years old and married with two young children.

“He’s so young to have to be dealing with this,” Lupton said. “He’s a very private person, but so helpful to everyone else.”

Lechtenberg’s co-workers are holding a benefit for him at 6:30 p.m. Friday at Chapin’s Restaurant, 701 N. Liberty St., Morris. A donation of $20 a person is being asked in exchange for appetizers and a fun evening filled with live music, raffles and silent auction items.

“We’re so thankful for Scott and Dianne (Breslar, Chapin’s owners) for their generosity,” Lupton said. “We wouldn’t be able to throw this benefit without them.”

Leiomyosarcoma is a form of a rare and aggressive cancer called sarcoma. A sarcoma is a cancer of the connective or supportive tissues of the body, such as bone, cartilage, fat, muscle and blood vessels, according to lmsdr.org.

The cause of Leiomyosarcoma is unknown, but studies have indicated there are genetic and environmental risk factors associated with the cancer.

“Most oncologists only see one or two cases of this in their careers,” Lechtenberg co-worker Trish Vidito said.

Another co-worker working on the benefit is Lisa Buhle.

Vidito said Lechtenberg plans to get a prosthetic arm, but he is unsure how much of the cost his insurance will cover ,so funds raised could go toward that also.

“Cancer is so common now, but to have to have a limb removed . . . We know how overwhelming it is for him and want to help as much as we can,” Vidito said.

For more information on the benefit, call Dan Lupton at (815) 325-3070.

Racing toward cancer research

Organization that raises money for Penn cancer research gets ad space from NASCAR

Along sleek sides of a TRG Motorsports Chevrolet screams David Gilliland's usual slew of sponsors: the Ford Racing team, the Web site freecreditreport.com, candy bars Milky Way and Twix and Combos pretzel snacks.

But this past weekend, one slogan on the number 17 cobalt blue race car didn't read like gas station advertising.

In addition to his normal array, Gilliland also sported the phrase "Sarcoma, The Forgotten Cancer" in bright green lettering across his car during last Monday's Sunoco Red Cross Pennsylvania 500 at Pocono Raceway in Long Pond, Pa.

The National LeioMyoSarcoma Foundation - a group that has raised almost $1 million to finance research at both Penn and Stanford University - partnered with Gilliland's TRG Motorsports to raise awareness about leiomyosarcoma (LMS), a rare form of cancer.

The project began after North Carolina resident and NASCAR fan Amy Elliott fought the extremely rare, aggressive soft-tissue cancer and vowed to raise awareness upon her recovery.

Just last week, Elliot approached TRG Motorsports General Manager Mike Brown in hopes of fulfilling that mission. Brown, in turn, offered Elliot and the LMS foundation rights to a prime advertising spot that typically costs $50,000 to $100,000.

The new partnership, and the recognition and awareness it generated will, in turn, make research like the projects currently underway at Penn and Stanford possible.

Penn Pathology professor John Brooks currently relies on both monetary and physical donations (in the form of tissue samples) to study the role of LMS at Penn, while Pathology professor Matt van de Rijn runs a similar LMS lab at Stanford.

Beyond fundraising, the ultimate goal of the "Sarcoma" message is to grab the attention of legislators and the general public to ensure that the many forms of sarcoma are properly studied, said Brooks.

Since the very aggressive LMS affects only four individuals out of every 1 million, patients, and thus the tissue samples required for research, are extremely rare.

As a result, tissue banks such as the ones at Penn and Stanford often face difficulties raising enough samples to conduct adequate research.

Unlike more common cancers, Brooks says that those studying rare tumors don't have luxury of the "press and research dollars" to which other research groups have access.

Because of this lack of funding, "there has been little progress on our understanding of the molecular genetic mechanisms driving [LMS], nor on treating it."

However, with the help of the LMS foundation and 300 donated samples, van de Rijn and his Stanford lab team were able to perform the most extensive research done on the deadly disease so far.

While the Stanford team is still in the process of analyzing the results, Brooks and the LMS Repository Tissue Bank are working to store the samples and more recently donated tissues for future use.

Circumferential resection of the inferior vena cava for primary and recurrent malignant tumors - Abstract

27 August 2009

University of South Florida, Tampa, Florida, USA.

Circumferential vena caval resection is occasionally performed in patients with advanced malignancy. We explored the oncological effectiveness of inferior vena caval resection, as determined by margin status, cancer recurrence and survival. Also, we addressed the morbidity associated with inferior vena caval obstruction and resection, and determined indications for inferior vena caval reconstruction.

A total of 18 patients underwent attempted inferior vena caval resection from 1999 to 2008. Primary tumor type was renal cell carcinoma in 7 patients, metastatic testicular cancer in 5, leiomyosarcoma in 3, and adrenal cortical carcinoma, primary retroperitoneal germ cell tumor and upper tract transitional cell carcinoma in 1 each. Data reviewed included preoperative and postoperative sequelae of inferior vena caval obstruction, postoperative complications, pathological results, cancer recurrence, graft requirements and functional outcomes.

Mean followup in the entire patient cohort was 24 months. Inferior vena caval resection was completed in 15 of 18 patients, of whom 12 (80%) had negative surgical margins. Of the patients 50% presented with symptoms of venous hypertension, including lower extremity edema with or without venous thrombosis, or abdominal wall varicosity. After inferior vena caval resection symptoms resolved in half of them, likely due to the ongoing formation of collateral vessels. Five asymptomatic patients with incomplete inferior vena caval occlusion underwent reconstruction with inferior vena caval vascular grafts of polytetrafluoroethylene (4) or Dacron (1). The polytetrafluoroethylene grafts remained patent. A total of 12 patients underwent simultaneous nephrectomy and/or left renal vein ligation in the same setting with acceptable alterations in postoperative renal function and no need for permanent dialysis. Cancer recurred locally in 4 of 15 patients who underwent resection. Five of 15 patients in the resection group died of disease or were lost to followup compared to all 3 in whom resection was aborted or macroscopically incomplete (mean followup 19.2 vs 4.3 months).

Local cancer control and potentially increased cancer specific survival can be achieved with successful complete circumferential resection of the inferior vena cava as a component of multimodality care in select patients with locally advanced malignancy. Polytetrafluoroethylene is the preferred prosthetic material when inferior vena caval replacement is indicated. The most common postoperative complications are renal insufficiency and lower extremity edema, which are generally transient.

Written by:
Caso J, Seigne J, Back M, Spiess PE, Pow-Sang J, Sexton WJ. Are you the author?

Reference:
J Urol. 2009 Sep;182(3):887-93.
doi:10.1016/j.juro.2009.05.015

PubMed Abstract
PMID:19616230

Friday, June 19, 2009

:: Music ::

http://www.code7music.com/store/images/bliss-SM.jpg

In one of those moods so I thought I would share some music that I have been listening to while thinking about myself and others with this sobering disease. -M.

Temple Of The Dog - Say Hello To Heaven

Please, mother mercy
Take me from this place
And the long winded curses
I keep hearing in my head
Words never listen
And teachers never learn
Now I'm warm from the candle
But I feel too cold to burn
He came from an island
And he died from the street
He hurt so bad like a soul breaking
But he never said nothing to me
So say hello to heaven

New like a baby
Lost like a prayer
The sky was your playground
But the cold ground was your bed
Poor stargazer
Shes got no tears in her eyes
Smooth like whisper
She knows that love heals all wounds with time
Now it seems like too much love
Is never enough, you better seek out
Another road cause this one has
Ended abrupt, say hello to heaven

I never wanted
To write these words down for you
With the pages of phrases
Of things well never do
So I blow out the candle, and
I put you to bed
Since you cant say to me
Now how the dogs broke your bone
Theres just one thing left to be said
Say hello to heaven

:: LISTEN ::

Thursday, June 4, 2009

:: General Update ::

http://www.foxnews.com/i/new/fn-header.jpg

WASHINGTON — Sen. Edward Kennedy's battle with a malignant brain tumor is likely to put a dramatic personal stamp on a health care cause he first championed nearly 40 years ago: The nation's war on cancer.

Kennedy had already begun work on an overhaul of the 1971 National Cancer Act when his tumor was diagnosed, and advocates hope the fact that Kennedy has fallen victim to this disease will generate public support and lend new urgency to the need to update the bill.

"People think of Ted Kennedy as a fighter and as someone who has always been there for everyone," said Daniel E. Smith, president of the American Cancer Society Cancer Action Network, the American Cancer Society's advocacy arm. "The fact that he now is fighting this disease is a jolt. It's a wake-up call to everyone."

"With that diagnosis, the irony for us is Senator Kennedy has been one of our great champions," Smith said.

The 76-year-old Kennedy has been a prominent and passionate advocate of cancer research and other health care issues throughout his long tenure in the Senate.

Prospective Randomized Trial

A prospective randomized trial comparing patient-
controlled epidural analgesia to patient-controlled
intravenous analgesia on postoperative pain control
and recovery after major open gynecologic cancer
surgery

http://snipurl.com/jf4ae [www_sciencedirect_com]

Methods
A randomized controlled clinical trial included
women undergoing laparotomy for a gynecologic
disorder. Patients were randomized to postoperative
IV morphine PCA (control arm) or to postoperative
morphine–bupivacaine PCEA (treatment arm).
Postoperative outcomes such as pain, and length of
hospital stay were compared with an intention-to-
treat analysis. The primary endpoint was
postoperative pain at rest and when coughing —
assessed by a 10-point visual analog scale (VAS).

Results
Between 9/04 and 6/07, 153 patients were randomized
and 135 were evaluable (PCEA = 67; PCA = 68). Over
75% of the women in this study had gynecologic
cancer. Patients in the PCEA arm had significantly
less postoperative pain at rest on Day 1 and during
the first 3 postoperative days when coughing
compared to the PCA arm (P < 0.05). The mean pain
score at rest on Day 1 was 3.3 for the PCEA group
compared to 4.3 for the PCA group (P = 0.01).
Overall, postoperative pain at rest and while
coughing in the first 6 days was less in women
treated with PCEA compared to PCA (P < 0.003).

Conclusions
PCEA offers superior postoperative pain control
after laparotomy for gynecologic surgery compared to
traditional IV PCA. Women requiring major open
surgery for gynecologic cancer should be offered
PCEA for postoperative pain management if there are
no contraindications.

Tuesday, June 2, 2009

Leiomyosarcoma of the Adrenal vein: a novel approach to surgical resection

Another great study is also listed here.

Here is an excerpt:
This is a 64-year old woman who presented with a 13 × 6.5 × 6.6 cm heterogeneous mass arising in the region of the right adrenal gland and extending into the inferior vena cava (IVC) and the right atrium. Biochemical evaluation excluded a functional tumor of the adrenal gland, and multiple tumor markers were negative. We present the novel use of deep hypothermic circulatory arrest (DHCA) in the resection of an adrenal vein leiomyosarcoma extending into the right atrium. The patient remains free of disease ten months after surgery. DHCA afforded a bloodless operative field for optimal resection of disease from within the IVC.

Leiomyosarcoma is a soft-tissue tumor that differentiates from smooth muscle. Primary leiomyosarcomas of vascular origin are relatively rare and frequently arise within the IVC [1-3]. Rarely, leiomyosarcomas may arise from the renal or adrenal vein. We report the case of a leiomyosarcoma of the adrenal vein and a novel approach to surgical resection involving the use of deep hypothermic circulatory arrest (DHCA).

(Figure 1).

thumbnail

Figure 1. Sagittal view of abdominal MRI. Tumor (arrow) extends from the superior pole of the right kidney to the right atrium.

A leiomyosarcoma of the adrenal vein was first reported in 1981, in a 50-year old patient with a 12 cm leiomyosarcoma arising from the left adrenal vein [4]. Since that time, there have been only eight other reported cases. We report a ninth case, using the novel approach of surgical resection under DHCA

More About LMS

Surgical Treatment of Leiomyosarcoma of the Distal Duodenum
R. Orda, J. Sayfan, I. Wasserman

Department of Surgery 'A', Assaf Harofeh Medical Center, Sackler Faculty of Medicine, Tel-Aviv University, Tel-Aviv, Israel

http://www.scielo.br/img/revistas/ibju/v29n1/Lemos_ing_43_44_figure2.jpg

Malignant tumors of the small intestine are rare. An uncommon finding of leiomyosarcoma located in the fourth part of the duodenum was diagnosed by gastrointestinal contrast studies, CT and angiography. Although malignant lesions of the small bowel are usually diagnosed late and thus are far advanced, curative resection was possible in our case. The location and histology of the tumor permitted a 'pancreas-preserving segmental duodenectomy'. The operative approach and exposure using the Cattell maneuver is described. It is emphasized that the more extensive pancreatoduodenectomy should be reserved for adenocarcinomas or lesions situated in the proximal part of the duodenum. Thirteen years following the operation, the patient is asymptomatic while CT and gastrointestinal contrast studies reveal no evidence of disease.

Alternative Names
Mass; Neoplasm

Definition Return to top

A tumor is an abnormal growth of body tissue. Tumors can be cancerous (malignant) or non-cancerous (benign).

Causes Return to top

In general, tumors appear to occur when there is a problem with the dividing of cells in the body. Typically, the division of cells in the body is strictly controlled. New cells are created to replace older ones or to perform new functions. Cells that are damaged or no longer needed die to make room for healthy replacements.

If the balance of cell division and death is disturbed, a tumor may form.

Problems with the body's immune system can lead to tumors. Tobacco causes more deaths from cancer than any other environmental substance. Other causes include:

  • Benzene and other chemicals and toxins
  • Drinking excess alcohol
  • Excessive sunlight exposure
  • Genetic problems
  • Inactivity (sedentary lifestyle)
  • Obesity
  • Radiation

Certain viruses can play a role in the development of tumors, such as cervical cancer (human papillomavirus) and hepatocellular carcinoma (hepatitis B virus).

Some tumors are more common in one gender than the other. Some are more common among children or the elderly. Others vary with diet, environment, and your family history.

Symptoms Return to top

Symptoms depend on the type and location of the tumor. For example, lung tumors may cause coughing, shortness of breath, or chest pain, while tumors of the colon can cause weight loss, diarrhea, constipation, iron deficiency anemia, and blood in the stool.

Some tumors produce no symptoms, but symptoms that may occur with tumors include:

Exams and Tests Return to top

Like the symptoms, the signs of tumors vary based on their site and type.

When a tumor is found, a biopsy is performed to determine if the tumor is non-cancerous (benign) or cancerous (malignant). Depending on the location of the tumor, the biopsy may be a simple procedure or a serious operation.

Most patients with tumors undergo CT scans or MRI to determine the exact location of the tumor and how far it has spread. More recently, positron emission tomography (PET) scans have been used to find certain tumor types.

Other tests include:

Treatment Return to top

Treatment also varies based on the type of tumor, whether it is benign or malignant, and its location. If the tumor is benign (meaning it has no potential to spread) and is located in a "safe" area where it will not cause symptoms or affect the function of the organ, sometimes no treatment is needed.

Sometimes benign tumors may be removed for cosmetic reasons, however. Benign tumors of the brain may be removed because of their location or harmful effect on the surrounding normal brain tissue.

If a tumor is malignant, treatments include:

  • Surgery
  • Radiation
  • Chemotherapy
  • A combination of these methods

If the cancer is in one location, the goal of treatment is usually to remove the tumor with surgery. If the tumor has spread to local lymph nodes only, sometimes these can also be removed. If all of the cancer cannot be removed with surgery, the options for treatment include radiation and chemotherapy, or both. Some patients require a combination of surgery, radiation and chemotherapy.

However, lymphoma is rarely treated with surgery. Chemotherapy and radiation therapy are most often used for treating lymphoma.

Support Groups Return to top

The diagnosis of cancer often causes a lot of anxiety and can affect a patient's entire life. There are many resources for cancer patients (see cancer resources).

Outlook (Prognosis) Return to top

The outlook varies widely among different types of tumors. If the tumor is benign, the outlook is generally very good. However, there are some instances where a benign tumor can cause significant problems, for instance, in the brain.

If the tumor is malignant, the outcome depends on the stage of the tumor at diagnosis. Some cancers can be cured. Some that are not curable can still be treated and patients can live for many years with the cancer. Still other tumors are quickly life-threatening.

Possible Complications Return to top

Complications can occur if a tumor is located in a region of the body where it affects the function of the normal organ. If the tumor is malignant, it can also cause complications if it spreads (metastasizes).

When to Contact a Medical Professional Return to top

Call your health care provider if you notice any suspicious lumps or bumps on your body, or if you notice a new or changing mole on your skin.

Prevention Return to top

You can reduce the risk of cancerous (malignant) tumors by:

  • Eating a healthy diet
  • Exercising regularly
  • Limiting alcohol
  • Maintaining a healthy weight
  • Minimizing exposure to radiation and toxic chemicals
  • Not smoking or chewing tobacco
  • Reducing sun exposure if you burn easily
Update Date: 8/3/2008

Leiomyosarcoma of the maxillary sinuses: report of two cases.

Leiomyosarcoma is a malignant smooth-muscle tumor that has a predilection for the gastrointestinal tract and the female genital tract. It is locally fast-spreading and highly aggressive, and the prognosis is poor. We report two cases of leiomyosarcoma of the maxilla maxilla /max·il·la/ (mak-sil´ah) pl. maxil´las, maxil´lae [L.] the irregularly shaped bone that with its fellow forms the upper jaw. max´illary

max·il·la
n. pl.
in patients who sought treatment for maxillary swelling, nasal obstruction, and epistaxis; one patient also had orbital involvement and cervical lymph node metastasis. Both patients underwent radical surgery followed by chemo- and/or radiotherapy. The patient with orbital involvement died shortly thereafter, but the other was disease-free at 18 months.

Case reports
Patient 1. A 27-year-old woman sought treatment for a 6-month history of painful swelling over the right maxillary area that was accompanied by nasal obstruction, epistaxis, proptosis proptosis /prop·to·sis/ (prop-to´sis) forward displacement or bulging, especially of the eye.

prop·to·sis
n. pl.
, and loose teeth.
Clinical examination revealed that the diffuse swelling measured 5 x 4 cm and involved the orbit (figure 1). The tumor extended inferiorly to the angle of the mouth, laterally to the front of the tragus, and medially to the dorsum of the nose. It obliterated the nasolabial fold and involved the skin at the root of the nose. The skin over the swelling appeared to be stretched. Movement of the right eyeball was restricted. Intraorally, the swelling destroyed the hard palate and alveolus. On anterior rhinoscopy, the mass could be seen filling the entire nasal cavity. Findings on postnasal postnasal /post·na·sal/ (-na´z'l) posterior to the nose.
post·na·sal
adj.
1. Located or occurring posterior to the nose or the nasal cavity.
2.
examination were normal. Examination of the neck revealed that multiple cervical lymph nodes Cervical lymph nodes are lymph nodes found in the neck. Anterior cervical nodes
The anterior cervical nodes are a group of nodes found on the anterior part of the neck.
at levels I and II were hard, nontender, and mobile.

[FIGURE 1 OMITTED]
We arrived at a clinical diagnosis of a malignancy of the right maxilla with orbital infiltration and metastasis to the cervical lymph nodes. Computed tomography (CT) revealed a large soft-tissue density in the right maxillary sinus that had destroyed all of the maxillary walls except the posterior wall and that had involved the orbit (figure 2). There was no intracranial extension.

[FIGURE 2 OMITTED]
Analysis of the biopsy specimen revealed that the tumor tissue was made up of fascicles of spindle-shaped cells with eosinophilic eosinophilic /eo·sin·o·phil·ic/ (-fil´ik)
1. readily stainable with eosin.

2. pertaining to eosinophils.

3. pertaining to or characterized by eosinophilia.
cytoplasm and oval to elongated and blunt-ended vesicular nuclei (figure 3). Mitosis was conspicuous. Areas of necrosis, fibrosis, hyalinization, and inflammatory infiltrates were seen. The reticulin reticulin /re·tic·u·lin/ (re-tik´u-lin) a scleroprotein from the connective fibers of reticular tissue.
re·tic·u·lin
n.
stain showed abundant reticulin fibers around individual tumor cells. The van Gieson's and Masson's trichrome stains showed smooth-muscle differentiation in the spindle cells. These features were those of a leiomyosarcoma.

[FIGURE 3 OMITTED]
The patient underwent radical maxillectomy with orbital exenteration exenteration /ex·en·ter·a·tion/ (ek-sen?ter-a´shun)
1. surgical removal of the inner organs; evisceration.

2. in ophthalmology, removal of the entire contents of the orbit.
and radical neck dissection Radical Neck Dissection Definition

Radical neck dissection is an operation used to remove cancerous tissue in the head and neck.
Purpose
. A temporary obturator obturator /ob·tu·ra·tor/ (ob´tu-rat?er) a disk or plate, natural or artificial, that closes an opening.
ob·tu·ra·tor
n.
1.
was inserted. The patient underwent chemo- and radiotherapy for 3 days, but died of aspiration pneumonia.

Patient 2. A 42-year-old woman reported a 2-month history of swelling over the left maxillary region, nasal obstruction, epistaxis, and loose teeth and a 1-month history of swelling in the left upper jaw. The jaw swelling arose after she had had three teeth extracted by a dentist.

Clinical examination revealed that the diffuse swelling over the left maxillary area measured 3 x 2 cm. The swelling extended superiorly to the infraorbital region, inferiorly to the angle of the mouth, laterally to the malar bone malar bone
n.
See zygomatic bone.
, and medially to the ala of the left nostril, where it obliterated the left nasolabial fold. The skin over the swelling was normal. The same swelling was seen intraorally, and it had destroyed the hard palate and extended to the alveolus, where it had obliterated the gingivobuccal sulcus sulcus /sul·cus/ (sul´kus) pl. sul´ci [L.] a groove, trench, or furrow; in anatomy, a general term for such a depression, especially one on the brain surface, separating the gyri. . The surface of the mass in the oral cavity was smooth, and the mucous membrane was intact. Anterior rhinoscopy revealed that a smooth mass in the nasal cavity had filled the left nostril. Findings on postnasal examination were normal. The orbit and the movement of the left eyeball were also normal. There were no palpable lymph nodes.

We arrived at a clinical diagnosis of a malignancy of the left maxilla without orbital infiltration. CT showed a large soft-tissue density in the maxillary sinus that had destroyed the anterior floor and the medial wall of the left maxillary sinus (figure 4). Biopsy analysis identified the lesion as a leiomyosarcoma. The patient underwent a total maxillectomy followed by radiotherapy, and she received a permanent prosthesis. At the 18-month follow-up, she was symptom-free (figure 5).

[FIGURES 4-5 OMITTED]
Discussion

The first case of maxillary leiomyosarcoma was reported in 1958. (1) Since then, only about 65 cases have been reported throughout the world. (2)

Incidence. Leiomyosarcomas account for 6.5% of all soft-tissue sarcomas, and only 3% of them arise in the head and neck. (3) When they occur in the sinonasal tract, the most common sites are the nasal cavity, the maxillary sinus, and the ethmoid sinus, in that order.

Clinical features. Leiomyosarcomas of the sinonasal tract are more common in men than in women. (4) The average age at diagnosis is 50 years. (5) Initial symptoms in order of decreasing frequency include nasal obstruction, epistaxis, facial pain, and facial swelling. Sinonasal tract leiomyosarcoma is characterized as locally aggressive rather than as having high metastatic potential. (1) Any degree of orbital invasion, which occurred in our patient 1, is disastrous. Leiomyosarcoma with regional lymph node involvement is rare, but cervical node metastasis has been reported in 15% of leiomyosarcomas that arise in the oral cavity. (6)

Histopathology. On histologic examination, the tumor is made up of interlacing fascicles of spindle-shaped cells that have elongated, blunt-ended nuclei and eosinophilic cytoplasm. Numerous mitotic figures are present. The cytoplasm of the tumor cells stains red in Masson's trichrome medium. (7)

Radiology. On CT, leiomyosarcomas appear as bulky masses, and they are frequently associated with extensive necrotic or cystic changes. These tumors do not contain calcifications, but they do cause frank bony destruction, as occurred in both of our patients. On magnetic resonance imaging magnetic resonance imaging (MRI), noninvasive diagnostic technique that uses nuclear magnetic resonance to produce cross-sectional images of organs and other internal body structures. , leiomyosarcomas show intermediate enhancement with contrast and intermediate to high signal intensity on T2-weighted imaging. (3)

Differential diagnosis. Depending on the patient's age and the location of the tumor, the differential diagnosis includes malignant schwannoma, malignant fibrous histiocytoma malignant fibrous histiocytoma
n.
A deeply situated tumor, especially on the extremities of adults, frequently recurring after surgery and metastasizing to the lungs.
, fibrosarcoms, amelanotic melanoma, malignant lymphoma, squamous cell carcinoma squamous cell carcinoma
n.
A carcinoma that arises from squamous epithelium and is the most common form of skin cancer. Also called cancroid, epidermoid carcinoma.
, extramedullary plasmacytoma, metastatic tumor, glandular tumor, chondroma chondroma /chon·dro·ma/ (kon-dro´mah) pl. chondromas, chondro´mata a benign tumor or tumor-like growth of mature hyaline cartilage. , chondrosarcoma, osteogenic sarcoma, and inverted papilloma. (3,6)

Treatment. Although regional lymph node involvement is rare, when it does occur, resection of the primary tumor with wide margins and neck dissection is the treatment of choice. Recent studies show that combination therapy with mesna, doxorubicin, ifosfamide, and dacarbazine or combination therapy with cyclophosphamide cyclophosphamide /cy·clo·phos·pha·mide/ (-fos´fah-mid) a cytotoxic alkylating agent of the nitrogen mustard group; used as an antineoplastic, as an immunosuppressant to prevent transplant rejection, and to treat some diseases , doxorubicin, vincristine vincristine /vin·cris·tine/ (vin-kris´ten) an antineoplastic vinca alkaloid; used as the sulfate salt in the treatment of various neoplasms, including Hodgkin's disease, acute lymphocytic leukemia, non-Hodgkin's lymphoma, Kaposi's , and dacarbazine is effective against soft-tissue sarcomas.

The clinical features of leiomyosarcoma in our 2 patients differed in that patient 1 exhibited orbital involvement and cervical lymph node metastasis. Her prognosis was very poor and despite radical surgery and postoperative chemo- and radiotherapy, she died of complications. Patient 2, who had less extensive disease, responded well to treatment.

References
(1.) Konrad HR. Nasal neoplasms. In: English GM, ed. Otolaryngology. Philadelphia: J.B. Lippincott, 1993:1-11.
(2.) Ortega JM, Gomez-Angulo JC, Aragones P, et al. [Leiomyosarcoma of the paranasal sinuses with intracranial involvement: Report of a clinical case and review of the literature]. Neurocirugia (Astur) 2001;12:331-7.
(3.) Tanaka H, Westesson PL, Wilbur DC. Leiomyosarcoma of the maxillary sinus: CT and MRI CT and MRI
Two high technology methods of creating images of internal organs. Computerized axial tomography (CT or CAT) uses x rays, while magnetic resonance imaging (MRI) uses magnet fields and radio-frequency signals. Both construct images using a computer.
findings. Br J Radiol 1998;71: 221-4.
(4.) Carran RL, Myers EN. Neoplasms of the nose and paranasal sinuses. In: Bailey BJ, ed. Head and Neck Surgery: Otolaryngology. 2nd ed. Vol. 2. Philadelphia: Lippincott-Raven, 1998:1450.
(5.) Rice DH, Stanley RB. Surgical therapy of tumors of the nasal cavity, ethmoid sinus and maxillary sinus. In: Thawley SE, Panje WR, eds. Comprehensive Management of Head and Neck Tumors. 2nd ed. Vol. 1. Philadelphia: W.B. Saunders, 1999:571.
(6.) Sumida T, Hamakawa H, Otsuka K, Tanioka H. Leiomyosarcoma of the maxillary sinus with cervical lymph node metastasis. J Oral Maxillofac Surg 2001;59:568-71.
(7.) Izumi K, Maeda T, Cheng J, Saku T. Primary leiomyosarcoma of the maxilla with regional lymph node metastasis. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1995;80:310-19.

From the Department of Otolaryngology (Dr. Prasad, Dr. Alva, and Dr. Madhu) and the Department of Pathology (Dr. Khadilkar), Kasturba Medical College, Mangalore Kasturba Medical College (KMC), Mangalore, is a medical college based in Mangalore, Karnataka, India.

It is a constituent college of Manipal University(Formerly known as MAHE), which is a deemed University recognized by an Act of UGC.
, India.

Reprint requests: Kishore Chandra Prasad, MS, DLO, Nethravathi Bldg., 1st Floor, Balmatta, Mangalore 575 001, Dakshina Kannada, Karnataka State, South India.

Phone: 91-824-244-5858, ext. 5498; fax:91-824-242-8379; e-mail:kishorecprasad@yahoo.com

New findings from Mayo Clinic describe advances in leiomyosarcoma

http://www.jeffersonhospital.org/obgyn/fibroid/leiomyosarcoma.jpg

2009 APR 20 -- According to recent research from the United States, "Inferior vena cava (IVC) leiomyosarcoma is a rare tumor of smooth muscle origin. It is often large by the time of diagnosis and may involve adjacent organs."

"A margin-free resection may be curative, but the resection must involve the tumor en bloc with the affected segment of vena cava and locally involved organs. IVC resection often requires vascular reconstruction, which can be done with prosthetic graft. Case presentation: We describe a 39-year-old man with an IVC leiomyosarcoma that involved the adrenal gland, distal pancreas, and blood supply to the spleen and left kidney.

http://www.jeffersonhospital.org/obgyn/fibroid/leiomyosarcoma2.jpg

Data on gastrointestinal stromal tumors described by D. Pasku et al

http://www.webpathology.com/slides/slides/Ext.Genitalia_SpermaticCordLMSGross.jpg
Leiomyosarcoma of Spermatic Cord

This was a 4.0 cm leiomyosarcoma arising in the spermatic cord of a 61 year old man. Paratesticular leiomyosarcoma is an extremely rare neoplasm. For a recent review of this tumor, refer to: Fisher C et al. Am J Surg Pathol 2001 Sep; 25(9):1143-9.

According to a study from Greece, "The location of gastrointestinal stromal tumors (GIST) outside of the gastrointestinal system is a rare event. Case presentation: A 56-year old woman presented with a GIST of the pelvis was misdiagnosed and treated as a uterine leiomyosarcoma."

"The diagnosis was made after the CD117 (KIT) positivity in the biopsy of the excised bowel mass four years from the first presentation. During this period she presented a bilateral muscle and subcutaneous metastasis in the gluteal area. The correct diagnosis of the extra-gastrointestinal stromal tumor is a challenge even for experienced pathologists. CD117 (KIT)...

Sunday, May 17, 2009

Study findings on leiomyosarcoma are outlined in reports from University of Vienna, Medical Department



2009 JAN 5
-- "Uterine leiomyosarcoma during pregnancy is an extremely rare event. The incidence of meningioma during pregnancy is comparable with that in nonpregnant women of the same age group," investigators in Vienna, Austria report.

"We report a case of both - a primary uterine leiomyosarcoma and additionally all atypical meningioma of the brain both diagnosed during pregnancy. The patient was admitted with generalised seizures at 31 weeks of gestation. A tumoural mass was detected and initial conservative treatment was started. The patient delivered her infant via caesarean section, at 34 weeks of gestation. During caesarean section a pedunculated uterine fibroid was removed and total gross resection due to the brain tumour was also performed. Histopathological diagnosis of both tumours revealed an atypical meningioma of the brain and a uterine leiomyosarcoma. The patient underwent laparatomy and received six cycles of adjuvant chemotherapy. We are the first to report a case of a woman with two separate primary neoplasms both diagnosed during pregnancy," wrote B. Bodneradler and colleagues, University of Vienna, Medical Department.

The researchers concluded: "Treatment options seem to be reduced in pregnant women and mainly depend on the patient's condition as well as the gestational age at presentation."

Bodneradler and colleagues published their study in Anticancer Research (Primary Uterine Leiomyosarcoma and Primary Atypical Meningioma Diagnosed during Pregnancy. Anticancer Research, 2008;28(5B):3083-3085).

For additional information, contact B. Bodneradler, University of Vienna, School Medical, Dept. of Obstetrics & Gynecology, Wahringer Gurtel 18-20, A-1090 Vienna, Austria.

The publisher of the journal Anticancer Research can be contacted at: International Institute Anticancer Research, Editorial Office 1ST km Kapandritiou-Kalamou Rd. Kapandriti, PO Box 22, Athens 19014, Greece.

Source

New findings from M.C. Cercato and co-authors in the area of carcinoma published



2009 JAN 5 -- "Multiple primary malignant neoplasms (MPMN) ore not uncommon, however, finding more than three primary malignancies in one individual is unusual. Surviving five malignancies is considered exceptional," scientists in Rome, Italy report.

"Two patients surviving five primary malignant neoplasms for 12 and 18 years are reported: a 55-year-old woman with a squamous cell carcinoma of the larynx, two carcinomas of the breast, a carcinoma of the kidney and an adenocarcinoma of the colon, and a 75-year-old woman with a sarcoma of the myometrium, a carcinoma of the thyroid, an adenocarcinoma of the rectum a leiomyosarcoma of the colon and a bronchial carcinoid. Only twelve other reported cases with five or more primary infiltrating malignancies involving more than three sites. diagnosed while the patient was alive have been found," wrote M.C. Cercato and colleagues.

The researchers concluded: "Relevant features were that colon cancer was quite often present more than once and survival was longer than expected for the stage (median overall survival, 20 Years: 95% confidence interval: 12-28 years)."

Cercato and colleagues published their study in Anticancer Research (Report of Two Cases of Quintuple Primary Malignancies and Review of the Literature. Anticancer Research, 2008;28(5B):2953-2958).

For additional information, contact M.C. Cercato, Italian National Cancer Institute Regina Elena, Dept. of Epidemiology, Via Elio Chianesi 53, I-00144 Rome, Italy.

The publisher's contact information for the journal Anticancer Research is: International Institute Anticancer Research, Editorial Office 1ST km Kapandritiou-Kalamou Rd. Kapandriti, PO Box 22, Athens 19014, Greece.

Source

PET Scans and LMS

http://www.steinfurthinstruments.com/pictures/LMS_Gesamtansicht_co_jn.jpg

Fluorodeoxyglucose positron emission tomography in leiomyosarcoma:
imaging characteristics.

Nucl Med Commun. 2009 May 13. [Epub ahead of print]

Punt SE, Eary JF, Oʼsullivan J, Conrad EU.
aDepartments of Radiology and Orthopedics, University of Washington,
Seattle, Washington, USA bDepartment of Statistics, University
College, Cork, Ireland.

OBJECTIVE: Leiomyosarcoma, a malignant neoplasm of smooth muscle,
accounts for 7% of the sarcomas. Patients with leiomyosarcoma tumors
have an average survival of 5 years. These tumors, which are derived
from mesenchymal tissues, are difficult to diagnose, and treatment
options remain controversial. The relatively rare incidence of this
soft tissue sarcoma subtype has limited the number of patients
available for studies and research. This study examines whether the
imaging characteristics of positron emission tomography (PET) with
radiolabeled fluorodeoxyglucose (FDG) provide a reliable, noninvasive
means to predict tumor behavior in patients with leiomyosarcomas.

METHODS: [F]-FDG-PET was performed on the tumors of participating
patients before the neoadjuvant chemotherapy or resection, and a
maximum tumor standard uptake value (SUVmax) was calculated.

RESULTS: The SUVmax was correlated with tumor grade (P=0.001) and
tumor size as greatest dimension (P=0.004). Analysis of these data
indicated the potential effectiveness of FDG-PET imaging in predicting
tumor grade.

CONCLUSION: In leiomyosarcoma, the SUVmax from FDG-PET is a likely
predictor of tumor behavior. The results of this study suggest that a
large (by greatest dimension) intermediate grade tumor is expected to
have the same predicted outcome as a high-grade tumor and should be
treated in the same manner, as they share the same prognosis by
definition of tumor grade. Improvements made in the clinical treatment
of leiomyosarcomas by use of FDG-PET imaging data may lead to an
increase in patient survival.

PMID: 19440162

Ginger Found to Ease Nausea of Cancer Treatment

http://1001resepi.com/wp-content/uploads/2008/05/ginger.jpg

Grandma was right when she recommended ginger for an upset stomach — at least for cancer patients.

A randomized clinical trial has confirmed what many people suspect — that ginger can decrease nausea caused by chemotherapy. The effect goes beyond that provided by standard anti-vomiting drugs.

The results will be presented at the annual meeting of the American Society of Clinical Oncology, which begins May 29 in Orlando, Fla. Abstracts of most of the studies to be presented at the conference were made public Thursday.

NYTimes Full Article

Myocardin gene amplification in most human retroperitoneal LMS

Strong smooth muscle differentiation is dependent on myocardin gene
amplification in most human retroperitoneal leiomyosarcomas.

Cancer Res. 2009 Mar 15;69(6):2269-78. Epub 2009 Mar 10.

Pérot G, Derré J, Coindre JM, Tirode F, Lucchesi C, Mariani O, Gibault
L, Guillou L, Terrier P, Aurias A.
Genetics and Biology of Cancers, Institut Curie, Paris, France.

Myocardin (MYOCD), a serum response factor (SRF) transcriptional
cofactor, is essential for cardiac and smooth muscle development and
differentiation. We show here by array-based comparative genomic
hybridization, fluorescence in situ hybridization, and expression
analysis approaches that MYOCD gene is highly amplified and
overexpressed in human retroperitoneal leiomyosarcomas (LMS), a very
aggressive well-differentiated tumor. MYOCD inactivation by shRNA in a
human LMS cell line with MYOCD locus amplification leads to a dramatic
decrease of smooth muscle differentiation and strongly reduces cell
migration. Moreover, forced MYOCD expression in three undifferentiated
sarcoma cell lines and in one liposarcoma cell line confers a strong
smooth muscle differentiation phenotype and increased migration
abilities. Collectively, these results show that human retroperitoneal
LMS differentiation is dependent on MYOCD
amplification/overexpression, suggesting that in these
well-differentiated LMS, differentiation could be a consequence of an
acquired genomic alteration. In this hypothesis, these tumors would
not necessarily derive from cells initially committed to smooth muscle
differentiation. These data also provide new insights on the cellular
origin of these sarcomas and on the complex connections between
oncogenesis and differentiation in mesenchymal tumors.

PMID: 19276386

Early Outcomes of Soft Tissue Sarcomas Presenting With Metastases and Treated With Chemotherapy

Am J Clin Oncol. 2009 May 8.

King JJ, Fayssoux RS, Lackman RD, Ogilvie CM.
From the *Department of Orthopedic Surgery, Drexel University,
Philadelphia, PA; and daggerDepartment of Orthopedic Surgery,
University of Pennsylvania, Philadelphia, PA.

OBJECTIVES:: The purpose of this study is to describe outcomes for
patients with metastatic soft tissue sarcoma treated with
chemotherapy.

METHODS:: We retrospectively reviewed the records of 383 soft tissue
sarcoma patients treated at our institution from 1997 to 2006.
Thirty-five patients met the inclusion criteria-metastatic disease on
presentation; primary tumor outside the abdomen; treatment with
chemotherapy; and minimum follow-up of 3 months. Treatments were
individualized and determined by a multidisciplinary oncology team.
Patient survival was calculated from the diagnostic biopsy to the date
of death or last follow-up using the Kaplan-Meier method.

RESULTS:: Mean age was 47 years (range 17-74 years).

Most common tumors involved were MFH and leiomyosarcoma.

Sixteen of 35 (46%) patients had died at last follow-up. Mean patient
survival was 24 months (range 4-102). The 2-year and 5-year survival
rates were 64% and 24%, respectively. Twenty-nine patients (83%)
presented with pulmonary metastasis, 6 with bone metastases (17%), 4
with regional node metastases (11%), and 4 with soft tissue metastases
(11%). Survival was the same for those with pulmonary and only
extrapulmonary metastases. The 10 patients with complete resection or
complete chemotherapy response of all detectable lesions had longer
mean survival (34 months) than the 25 patients who had partial/no
resection or response (20 months) (P = 0.02).

CONCLUSIONS:: Soft tissue sarcoma patients presenting with metastatic
disease have a low survival rate, but complete eradication of tumor
correlated with longer survival. Longer-term studies especially those
tracking the outcome of complete responders and those completely
resected will help determine the efficacy of chemotherapy.

PMID: 19433963 [PubMed - as supplied by publisher]

Uterine sarcomas in Norway. A histopathological and prognostic surve

Uterine sarcomas in Norway. A histopathological and prognostic survey
of a total population from 1970 to 2000 including 419 patients.

Histopathology. 2009 Feb;54(3):355-64.

Abeler VM, Røyne O, Thoresen S, Danielsen HE, Nesland JM, Kristensen GB.
Division of Pathology, Radiumhospitalet, University Hospital, Oslo,
Norway. vera.maria.abeler@...

AIMS: To determine the frequency and survival of the various types of
uterine sarcoma in the total population of Norway and evaluate
histopathological prognostic factors in order to identify risk groups.

METHODS AND RESULTS: Histopathological review of all uterine sarcoma
cases reported to the Norwegian Cancer Registry during 1970-2000 was
undertaken. Survival dates were provided by The Cancer Registry.
Kaplan-Meier survival curves were generated. The log rank test was
used for univariate analysis and a Cox proportional hazards regression
model for multivariate evaluation of survival. Stage of disease was
the most important prognostic factor for all tumour types.

Tumour size and the mitotic index (MI) were significant prognostic
factors (P < 0.0001) in leiomyosarcomas confined to the uterus and
allowed for separation into three risk groups with marked differences
in prognosis.

The prognosis of endometrial stromal sarcomas confined to the uterus
was related to MI (P < 0.0001) and tumour cell necrosis (P < 0.004).
Combining these parameters allowed for separation into three risk
groups with marked difference in prognosis. In adenosarcomas, tumour
cell necrosis was the only significant prognostic factor.

CONCLUSIONS: There are marked differences in survival between uterine
sarcoma types. Leiomyosarcomas and endometrial stromal sarcomas can be
divided into different groups.

PMID: 19236512