Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report a rare case of a young female with primary brain fibrosarcoma, and to the best of our knowledge, we believe that only <50 cases have been reported or described worldwide so far. Fibrosarcoma is a malignant neoplasm, in which histologically the predominant cells are fibroblasts that divide excessively without cellular control and they can invade local tissues or metastasize. Primary central nervous system fibrosarcomas are very aggressive neoplasms and generally have a poor prognosis. This tumor is either from sarcomatous transformation of a meningioma or arises de novo within the brain parenchyma. Our patient, a 48-year-old woman, who presented with progressive speech disorder over the period of 4 months, showed a left temporoparietal lesion with surrounding edema and local mass effect. Total surgical resection was achieved. Histopathology revealed classical fibrosarcoma features and secondary screening revealed no other distant lesion as diagnosis of primary brain fibrosarcoma was established. This case is deemed to be extremely rare because most reports claim that recurrence is within 6 months with poor prognosis; however, this patient is currently recurrence-free at 3 years. This would suggest of the possibility for a relook into this disease's course and recurrence rate when complete excision is achieved. Due to extreme rarity of these tumors, more comparative studies will be needed to improve the disease outcome.
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PMID:Primary Central Nervous System Fibrosarcoma. 2893 84

Fibrosarcoma represent a rare group of soft tissue malignancies derived from fibrous connective tissue and immature proliferating fibroblasts or undifferentiated anaplastic spindle cells. It affects patients in the fourth and fifth decade of life. Fibrosarcomas can be classified in subtypes such as low-grade fibromyxoid sarcoma (LGFMS) and sclerosing epithelioid fibrosarcoma (SEF), and others. Histological features that overlap between types of fibrosarcomas is well known and reported in the literature. We report the case of a 53-year-old patient who presented a tumor in the axillary fossa, which was initially diagnosed as a solitary fibrous tumor. Due to recurrence of the lesion, as well as the presence of distant metastases, the histological revision considered the diagnosis of breast metaplastic carcinoma, since the tumor expressed the p63 antigen and estrogen and progesterone receptors. Unexpected resistance to chemotherapy motivated the diagnosis re-evaluation, which was due to MUC4 expression and morphological characteristics concluded by a hybrid LGFMS-SEF tumor. The authors call attention to the difficult diagnosis in cases of soft tissue tumors. A broad panel of immunohistochemical research is required as the clinical course is essential to the final diagnosis.
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PMID:Fibrosarcoma: a challenging diagnosis. 3152 15

Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal tumor with unique histological and immunohistochemical characteristics. Malignant renal cell carcinoma is even rarer and its biological behavior is still not clear. We report here a case of malignant PEComa in a male patient. The first symptom of the patient was intermittent hematuria. Ultrasound and computed tomography scan showed a solid mass in the left kidney. Fibrosarcoma was initially diagnosed after radical resection of the tumor. Six years later, the patient had a tumor recurrence and underwent a second operation, through which the diagnosis of renal malignant PEComa was made. Since then, the patient received chemotherapy, a third operation, and interventional treatment because of recurrence and metastasis of the tumor, which prolonged the life of the patient for another 6 years. Malignant PEComa involving the kidney is very rare; it seems to develop slowly but will recur and metastasize. At present, surgical resection remains the best treatment.
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PMID:Perivascular epithelioid cell tumor (PEComa) of the kidney: an overview of its management and outcomes. 3307 Jun 94


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