Humans ; Sarcoma, Alveolar Soft Part/secondary* ; Lung Neoplasms/pathology* ; Soft Tissue Neoplasms/pathology*

Alveolar soft-part sarcoma is a rare soft tissue sarcoma of young adults with unknown histogenesis, and the organ most frequently involved in metastasis is the lung. We report the CT findings of three patients of pulmonary metastases of alveolar soft-part sarcoma, which manifested as clearly enhanced pulmonary nodules or masses. On enhanced scans, some of the masses were seen to contain dilated and tortuous intratumoral vessels.
Adult ; Case Report ; Female ; Human ; Lung Neoplasms/blood supply/*radiography/*secondary ; Male ; Sarcoma, Alveolar Soft Part/blood supply/*radiography/*secondary ; *Tomography, X-Ray Computed

Adenocarcinoma ; metabolism ; pathology ; Adult ; Desmin ; metabolism ; Diagnosis, Differential ; Humans ; Lung Neoplasms ; secondary ; Lymphatic Metastasis ; Male ; Melanoma ; metabolism ; pathology ; Rhabdomyosarcoma, Alveolar ; metabolism ; pathology ; surgery ; Sarcoma, Alveolar Soft Part ; metabolism ; pathology ; Testicular Neoplasms ; metabolism ; pathology ; surgery ; Testis ; metabolism ; pathology

Alveolar soft part sarcoma (ASPS) is a rare malignant soft-tissue neoplasm of unknown histogenesis. The two main sites of occurrence are the lower extremities in adults and the head and neck in children. We report the first case of pleural ASPS occurring in a 58-yr-old man who presented with progressive dyspnea. A computed tomographic scan of the thorax revealed a large enhancing pleural mass with pleural effusion in the left hemithorax. Wide excision of the pleural mass was performed. Histologically, the tumor consisted of organoid nests of large polygonal cells, the cytoplasm of which had eosinophilic and D-PAS positive granules. Immunohistochemical staining showed that the tumor cell nuclei were positive for transcription factor 3 (TFE3). The pleural ASPS with multiple bone metastases recurred 1 yr after surgery and the patient died of acute pulmonary embolism 1.5 yr after diagnosis.
Bone Neoplasms/diagnosis/secondary ; Dyspnea/etiology ; Humans ; Immunohistochemistry ; Male ; Middle Aged ; Pleura/physiopathology ; Positron-Emission Tomography and Computed Tomography ; Pulmonary Embolism/diagnosis ; Sarcoma, Alveolar Soft Part/*diagnosis/pathology/radiography ; Soft Tissue Neoplasms/*diagnosis/pathology/radiography ; Transcription Factor 3/metabolism

OBJECTIVETo investigate the clinical feature, suitable treatment and prognosis of alveolar soft part sarcoma.

METHODSThe clinical data of 58 such patients treated at our hospital from 1967 to 2006 were retrospectively analyzed. Fifty-three patients except 6 with distant metastasis underwent surgical resection including 33 extensive resection and 19 local resection. Furthermore, 19 patients received postoperative adjuvant radiotherapy or chemotherapy.

RESULTSEleven of 50 patients who underwent complete resection developed local recurrence. Thirty-One (53.4%) was found to have lung metastasis. The overall 3-, 5-, 10-year survival rate was 89.5%, 74.1% and 57.7%, respectively. The median survival time of the patients was 125 months. The 3-, 5-, 10-year survival rate was 100.0%, 81.6% and 65.3% for female patients, but it was 79.6%, 67.2% and 49.7% for male patients, respectively.

CONCLUSIONAlveolar soft part sarcoma usually grows slowly. Though local recurrence is rarely seen, distant metastasis is frequently observed. Lung is the most common organ of metastasis. However, survival can still be long even with lung metastasis. To achieve microscopically complete resection is quite critical to localized alveolar soft part sarcoma. No survival advantage was observed when adding adjuvant radiotherapy and/or chemotherapy. The phenomena of female patient or patient with X chromosome translocation showing better prognosis than the male or other patients needs further investigation.

Adolescent ; Adult ; Chemotherapy, Adjuvant ; Child ; Female ; Follow-Up Studies ; Humans ; Lung Neoplasms ; secondary ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; Neoplasm Staging ; Radiotherapy, Adjuvant ; Retrospective Studies ; Sarcoma, Alveolar Soft Part ; drug therapy ; radiotherapy ; secondary ; surgery ; Sex Factors ; Soft Tissue Neoplasms ; drug therapy ; pathology ; radiotherapy ; surgery ; Survival Rate ; Young Adult

OBJECTIVETo analyze the clinical features, treatment and prognosis of Stage IV alveolar soft part sarcoma.

METHODSTo analyze the clinical and pathological features, therapeutic methods and follow-up results in 21 patients with stage IV alveolar soft part sarcoma. There were 11 males and 10 females, in the age of 26-57 years (average 37.0 years old). All the 21 patients had metastasis: nine cases had multiple pulmonary metastasis, three cases had multiple pulmonary and brain metastasis, two cases had multiple brain metastasis, two cases had multiple pulmonary and bone metastasis, two cases had single pulmonary metastasis, one case had single bone metastasis, one case had single brain metastasis and one case had single soft tissue metastasis. Eight patients were treated by surgical operation, including five cases of complete resection for the primary and (or) metastatic tumor and 3 cases of palliative operation for the primary tumor. All patients received chemotherapy, including seven cases of CAVD regimen and 14 cases of MAID regimen treatment. One patient with single bone metastasis and five patients with multiple brain metastasis received post-operative whole brain radiation therapy.

RESULTSAll the eight patients with surgical operation had healing by first intention, and pathological examination showed that seven patients achieved R0 surgical margin and one case with R2 status. One patient with single brain metastasis had recurrence after operation. The toxic and adverse reactions of all patients treated with chemotherapy were tolerable. Among them, 17 cases had stable disease and 4 cases had disease progression after chemotherapy. The disease control rate (DCR) was 81.0%. The DCR of patients with CAVD regimen chemotherapy was 85.7% and that of patients treated with MAID regimen was 78.6% (P = 0.862). All patients were followed up for 8 - 86 months (average 32.4 months). The median survival time of all patients was 32.6 months. The 2-year survival rate was 55.1% and the 5-year survival rate was 21.8%. The median survival time in the patients with complete resection was 60.0 months, and that in patients with palliative operation was 27.0, showing a significant difference between them (P = 0.048). The median progression-free survival in patients with complete excision was 57.2 months and that in patients with palliative operation or without operation was 19.6 months, with a significant difference (P = 0.029). The median survival time in patients who received CAVD regimen chemotherapy was 30.0 months, and that in patients with MAID regimen was 51.0 months, with a non-significant difference (P = 0.511). The median progression-free time in patients with CAVD regimen chemotherapy was 13.0 months, and that in patients with MAID regimen was 38.0 months, also with a non-significant difference (P = 0.066).

CONCLUSIONSAlveolar soft part sarcomas are rarely seen and highly malignant tumors, and the prognosis of stage IV ASPS is poor. Complete resection of all tumors is the key of successful treatment of Stage IV ASPS, and the site and number of tumor metastasis are important factors affecting prognosis. The curative effects of radiotherapy and chemotherapy for ASPS need to be further investigated.

Adult ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Bone Neoplasms ; radiotherapy ; secondary ; surgery ; Brain Neoplasms ; radiotherapy ; secondary ; surgery ; Dacarbazine ; therapeutic use ; Disease-Free Survival ; Doxorubicin ; therapeutic use ; Female ; Follow-Up Studies ; Humans ; Ifosfamide ; therapeutic use ; Lower Extremity ; Lung Neoplasms ; secondary ; surgery ; Male ; Mesna ; therapeutic use ; Middle Aged ; Neoplasm Recurrence, Local ; Neoplasm Staging ; Particle Accelerators ; Remission Induction ; Salvage Therapy ; Sarcoma, Alveolar Soft Part ; drug therapy ; radiotherapy ; secondary ; surgery ; Soft Tissue Neoplasms ; drug therapy ; radiotherapy ; surgery ; Survival Rate