rare kind of tumor-sarcoma

[yippychan]

My mom has a very rare kind of cancer called myxoid fibro sarcoma in her left temporal (head) area.  My mom has had multiple surgies and the tumor always comes back.  Her recent biopsy showed a low grade recurrance of the sarcoma again.  Is there any other treatments other than surgery?  Today, my mom told me that she has right abdominal pain with bloated feeling for about 1 week.  Would it be possible that the tumor has spread?  Can anyone tell me what to expect? Thank you.

[rof]

I have a 3.4year old sun who has been diagnosed with a sarcoma in his trachea, it approximately 1cm in width. Not much is known about it? he has been given  vincristine ,actinomycin-D ect so far the tumar has reduced is this a good sign?

[Enoch Choi, MD]

Sorry i'm not familiar with myxoid spindle cell fibromyxoid sarcoma, you'd need to discuss with her oncologist.  Good luck!

[yippychan]

Thanks so much for your information.  I took my mom in to see her PCP and an ultrasound of the abdomine was ordered, the result should be out in about one week.  During her visit to her PCP, she told the doctor the pain and bloated sensation was in her right lower abdominal area.  Her PCP also ordered some blood work plus some urine test.  I hope the result will be negative.  Have you heard of myxoid spindle cell fibromyxoid sarcoma?  Is it the same as the original myxoid fibrosarcoma?  Thanks a lot for taking your time to answer my question?

[Enoch Choi, MD]

Your mother's right abdominal pain needs immediate evaluation.

Fibrosarcomas are often of high histologic grade and often recurr. Treatment can include some combination of surgery and radiation therapy, and chemotherapy. The prognosis for this disease is poor. In a small study of 9 patients, eight patients died with a median survival of 7.5 months, with local recurrence in eight patients and distant recurrence in six patients.  See the 2 references below:


TI Primary intracranial fibrosarcoma.  
AU Bisogno G; Roganovic J; Carli M; Scarzello G; Calderone M; Faggin R; Perilongo G  
SO Childs Nerv Syst. 2002 Nov;18(11):648-51. Epub 2002 Aug 15.  
  
  INTRODUCTION: Primary fibrosarcomas of the brain are very rare tumors, so that information regarding the treatment is scarce. We report the contributions that different therapeutic options made to the treatment of a child with one of these aggressive tumors. CASE REPORT: A 13-year-old boy underwent a complete resection of a left temporo-parietal mass that had been diagnosed as a fibrosarcoma by two independent pathologists. Adjuvant chemotherapy with vincristine, actinomycin-D, ifosfamide and Adriamycin was started, but after 3 months tumor relapse was evident. The boy subsequently received radiation therapy during which there was evidence of progressive tumor shrinkage. A second surgery was performed 6 months after radiotherapy and a small enhancing lesion, revealed to be gliosis, was resected. The child remains alive and well 44 months after diagnosis. CONCLUSION: Our experience supports the importance of total resection followed by radiation therapy, and radiotherapy should be started as soon as possible after surgical resection, rather than administering chemotherapy first.  
  
AD Hematology/Oncology Division, Department of Pediatrics, Via Giustiniani, 3, 35128 Padua, Italy. gianni.***@****  



TI Primary cerebral fibrosarcomas. Clinicopathologic study and review of the literature.  
AU Gaspar LE; Mackenzie IR; Gilbert JJ; Kaufmann JC; Fisher BF; Macdonald DR; Cairncross JG  
SO Cancer. 1993 Dec 1;72(11):3277-81.  
  
  BACKGROUND. Primary of the brain and meninges are uncommon tumors. Information regarding optimum treatment is limited due to their rarity, and the best form of therapy is not yet known. METHODS. Nine patients between the ages of 22 and 61 years with primary fibrosarcomas confined to the brain and meninges were studied clinicopathologically. Tumors were superficially located in five patients and intracerebrally or deep in four patients. Treatment consisted of maximum feasible surgical resection (seven patients, gross total; one, subtotal; one, no surgery before death) and radiation therapy (45-60 Gy conventional fractionation) in eight of nine patients. RESULTS. Fibrosarcomas were moderate or high grade in seven of nine patients (78%). Immunohistochemical reactions with antisera to glial fibrillary acid protein (GFAP), cytokeratin, vimentin, desmin, and S-100 demonstrated vimentin positivity in five of nine patients and cytokeratin positivity in two of nine patients. Individual GFAP-positive cells were seen in two cases, thought to represent trapped reactive astrocytes. None were S-100-positive or desmin-positive. Eight patients have died, with a median survival time of 7.5 months (range, 1 day-96 months). Local recurrence developed in eight patients and distant recurrence in six patients. Systemic metastases developed in four patients (50%) and meningeal seeding in four patients (50%). Longer survival was observed in superficially located tumors (range, 7.5 months-96 months) compared with intracerebral tumors (range, 1 day-9 months). CONCLUSIONS. Primary fibrosarcomas of the brain are uncommon tumors, usually of high histologic grade, with a high rate of local recurrence. Their propensity for meningeal and distant relapse distinguishes them from tumors of glial origin. Immunohistochemistry is of limited diagnostic value, although it may facilitate exclusion of other diagnoses. Because the prognosis after conventional surgery with 50-60 Gy external beam radiation therapy is relatively poor, the authors recommend more aggressive therapy with maximal feasible resection followed by external beam radiation therapy to doses of 64-66 Gy. When effective chemotherapy is established for soft tissue sarcomas of the extremities, this should be evaluated in view of the high incidence of distant metastases.  
  
AD London Regional Cancer Centre, Ontario, Canada



Dr. Enoch Choi, MD
Palo Alto, CA