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Leiomyosarcoma of the Soft Tissue

Leiomyosarcomas are the most common mesenchymal tumors of the lung presenting as an endobronchial lesion with symptoms of obstruction. Peripheral lesions are asymptomatic.

 Abstract:

Primary leiomyosarcomas of the lung: a clinicopathologic and immunohistochemical study of 18 cases. Mod Pathol. 1997 Feb;10(2):121-8

We studied 18 patients with primary malignant smooth muscle tumors of the lung (7 women and 11 men, 5-76 yr old, with a mean age of 50 yr). Lesions varied from 1.7 to 10 cm in greatest diameter. The tumors were classified as low (4 cases), intermediate (2), or high grade (12). Low-grade lesions were characterized by an orderly proliferation of fascicles of spindle cells that intersected at right angles and showed oval-to-spindle cells with cigar-shaped nuclei, minimal pleomorphism, and low mitotic activity, without hemorrhage or necrosis. Intermediate-grade lesions retained the fascicular configuration but showed increased cellularity with atypia and dense chromatin pattern, occasional pleomorphism, and mild increase in mitotic activity. High-grade lesions showed high cellularity, marked pleomorphism and atypia, frequent areas of hemorrhage and necrosis, and high mitotic activity. Immunohistochemical studies in 16 cases showed positive staining of tumor cells with smooth muscle actin in 12, desmin in 5, and coexpression of actin and/or desmin and keratin in 3. Six patients with low- and intermediate-grade lesions were alive and well from 2 to 12 years after diagnosis (mean follow-up time, 6 yr); 8 with high-grade lesions died of their tumors with widespread metastases from 1 to 24 months after diagnosis (median survival time, 5 mo). One patient whose tumor showed features of high-grade leiomyosarcoma was alive and well 12 years after surgery. Three patients with high-grade tumors were lost to follow-up. Our findings suggest that histologic grade may be the most reliable prognostic parameter for predicting clinical behavior in primary leiomyosarcoma of the lung and that smooth muscle actin is the most sensitive immunohistochemical marker for establishing the diagnosis in these tumors. Primary leiomyosarcoma should be considered in the differential diagnosis of pulmonary spindle cell neoplasms; histologic grading may be of value in the planning of therapy and assessment of prognosis for these lesions.

A case of primary pulmonary leiomyosarcoma with history of pneumonia 5 years before.Nihon Kokyuki Gakkai Zasshi. 2004 May;42(5):419-2

A 35-year-old woman with past history of pneumonia in the right lung field 5 years before was admitted to our hospital because of fever and cough. Chest radiographs showed a pulmonary tumor with atelectasis of the right lower lung. Chest CT also revealed a round clear-edged tumor at the right S6 with atelectasis of the right lower lung lobe. Bronchoscopic findings showed a yellowish endobronchial tumor in the right truncus intermedius, which proved to be leiomyosarcoma. We could not find any other malignant lesion, and therefore, on a diagnosis of primary pulmonary leiomyosarcoma, right middle and lower lobectomy was performed with lymph node excision. Retrospective examination of the chest radiographs revealed not only that the original region of the leiomyosarcoma seemed to be near the site of the earlier pneumonia, but also that the atelectasis-like findings 2 years before were similar to the findings on this admission. It was reported that, if an operation could not be performed at an early stage, the prognosis might be poor. In the follow-up of the abnormal chest radiographic findings, the clinic physician should observe the symptoms from the same viewpoint as hospital doctors. It is important to keep an active relationship between clinic and hospital. We might have reached our final diagnosis earlier if we had been more active in seeking an examination for abnormal chest radiographic findings, without attaching too much importance to the patient's age.

Primary pulmonary leiomyosarcoma. Report of a case diagnosed by fine needle aspiration cytology.Acta Cytol. 2002 Sep-Oct;46(5):912-6.

BACKGROUND: Primary pulmonary leiomyosarcoma is a rare but important entity. We report a case diagnosed by fine needle aspiration cytology. CASE: A 73-year-old male presented with an asymptomatic, right, pulmonary, subpleural nodule detected by computed tomography during follow-up for chronic obstructive pulmonary disease. Fine needle aspiration cytology showed cellular smears with numerous single or loosely cohesive groups of spindle-shaped to round cells. The tumor cell nuclei were blunt ended (cigar shaped), with fine to fine-granular chromatin, prominent nucleoli and an irregular nuclear rim. The tumor cells were positive for desmin and negative for cytokeratin and S-100 protein by immunocytochemistry. Right upper lobectomy with lymph node dissection was performed. Pathologic diagnosis after microscopic, immunohistochemical and electron microscopic studies was leiomyosarcoma. CONCLUSION: To our knowledge, this is the first reported case of primary pulmonary leiomyosarcoma arising in the subpleural region diagnosed by fine needle aspiration cytology. Immunocytochemistry was useful in establishing the diagnosis in this case.

Radiologic findings in primary pulmonary leiomyosarcoma.J Thorac Imaging. 2000 Apr;15(2):151-2.

Leiomyosarcomas are uncommon smooth muscle tumors most commonly encountered in the female genital organs, gastrointestinal tract, and soft tissues. They are rarely encountered as primary pulmonary neoplasms. Herein we describe the conventional radiographic and computed tomographic findings in a patient with a pulmonary leiomyosarcoma.

                 

Pulmonary leiomyosarcoma.Chin Med Sci J. 1997 Jun;12(2):129-31

Three cases of pulmonary leiomyosarcoma were presented. The characteristic clinical features were described with review of literature. In comparison with bronchogenic carcinoma, the leiomyosarcoma has some characteristics: 1) On chest X-ray, it usually appears as a sharply demarcated, even density round mass, growing rapidly within the lung, it unusually accompanies with hilar or mediastinal lymph node metastasis; 2) The preoperative cytological or pathological diagnosis is difficult either by sputum smear or by bronchoscopic biopsy or by fine needle percutaneous aspiration biopsy; 3) Pathological differential diagnosis of leiomyosarcoma of lung from anaplastic lung cancer is difficult. In conclusion, the primary pulmonary leiomyosarcoma is a rare malignant tumor, detecting the present illness seriously, paying attention to the chest X-ray films characterize, early surgical resection is the only way to get diagnosis and effective treatment method.

Pulmonary leiomyosarcoma--report of three cases.Chin Med Sci J. 1996 Sep;11(3):191-4

Three cases of pulmonary leiomyosarcoma were presented. The characteristic clinical features were described with review of literature. In comparison with bronchogenic carcinoma, the leiomyosarcoma has some characteristics: 1) On chest X-ray, it usually appears as a sharply demarcated, even density round mass, growing rapidly within the lung, it rarely accompanies with hilar or mediastinal lymph node metastasis. 2) The preoperative cytological or pathological diagnosis is difficult either by sputum smear or by bronchoscopic biopsy or by fine needle percutaneous aspiration biopsy. 3) Pathological differential diagnosis of leiomyosarcoma of lung from anaplastic lung cancer is difficult. In conclusion, the primary pulmonary leiomyosarcoma is a rare malignant tumor, detecting the present illness seriously, paying attention to the chest X-ray films characterize, early surgical resection is the only way to get diagnosis and effective treatment method.

Pulmonary leiomyosarcoma.Arch Bronconeumol. 1994 Nov;30(9):465-7

We present the case of a young patient with leiomysarcoma of the lung that was at first diagnosed as a hydatid cyst. Pathological tissue analysis was needed for firm diagnosis. This rare tumor, of which fewer than 100 cases have been reported worldwide in the literature, needs to be considered as a differential diagnosis when lung cancer is suspected, given that leiomysarcoma has a better prognosis. Treatment is mainly surgical.

A case of pulmonary leiomyosarcoma followed up for five years.Nippon Kyobu Geka Gakkai Zasshi. 1992 Nov;40(11):2066-9

A 70-year-old woman with leiomyosarcoma, followed up for 5 years as tuberculoma, is described. The tumor, in the left lower lobe, had enlarged from 1.5 x 1.5 cm to 3.4 x 3.4 cm in 5 years, and doubling time was calculated to be 540 days. Another tumor (1.8 x 1.8 cm) was also recognized in the right lower lobe at the later time. Since not only a benign tumor but metastatic disease was suspected, thoracotomy was performed, and the intraoperative pathologic diagnosis was leiomyosarcoma. However, only partial lung resection was performed because of the low growth rate of the tumor, the patient's advanced age, limited lymphatic metastasis and favorable prognosis with only partial resection. DNA analysis of the tumor based on flow cytometry showed a diploid pattern, and this was thought to account for the long doubling time of the tumor. The postoperative course was satisfactory without any signs of recurrence at the operation site.

Two cases of pulmonary leiomyosarcoma.Nippon Kyobu Geka Gakkai Zasshi. 1990 Jul;38(7):1203-8

Pulmonary sarcoma is a rare tumor of the lung. There are 81 cases in the literature for review, including our two cases. Our first case who was a thirty-seven year old male who was admitted to our hospital with complaints of left back pain. His chest X-ray showed an 11 X 14 cm tumor shadow in the lower left lung area. A left upper lobectomy was performed, and the histological diagnosis was leiomyosarcoma of the pulmonary vein. He died due to mediastinal recurrence 29 months later. Our other case was a fifty-six year old female. Her chest X-ray showed an abnormal shadow that was a tumor shadow in the right central lung area of 4 cm in diameter. As the diagnosis of the transcutaneous lung biopsy was benign tumor, partial resection of lung was performed. The histological diagnosis was leiomyosarcoma of the bronchus, and she is alive and well at 29 months after the operation. As the rate of diagnosis in leiomyosarcoma detected by transcutaneous lung biopsy is low, the most acceptable method of diagnosis is a total examination, including cytology of sputum, bronchoscopic findings, and roentgenoscopy. Probe thoracotomy would also be necessary if malignancy could not ruled out. The treatment of lobectomy should include lymph node resection.

Primary pulmonary leiomyosarcoma. Review of the literature apropos of a case.J Chir (Paris). 1987 Apr;124(4):272-5

A 55-year-old patient with primary pulmonary leiomyosarcoma was treated by pneumonectomy. The diagnosis was established by histology of the operative piece. Bronchoscopy and cytology of expectorations were negative. This rare tumor (fewer than 100 cases described) differs from bronchial carcinoma by its method of spread, mainly hematogenic, and its better prognosis. clinical and radiographic data do not provide differential diagnoses. Treatment is essentially surgical since the tumor is only weakly radio-sensitive and responds poorly to chemotherapy.

 
Soft Tissue Pathology;

Myxoid Tumours of Soft Tissue Classification of Soft Tissue Tumour;  Gross examination of soft tissue specimen ;  A practical approach to histopathological reporting of soft tissue tumours Grading of soft tissue tumours ; Lipomatous tumours ;Neural tumours ; Myogenic tumours ;Vascular tumours ;Fibroblastic/Myofibroblastic tumours ; Myofibroblastic tumours ;  Fibrohistiocytic tumours ; ChondroOsseous tumours ; Soft TissueTumours of Uncertain Differentiation ; Notochordal Tumour -Chordoma ;Extra-adrenal Paraganglioma ; Gastrointestinal Stromal Tumour ;

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MESOTHELIOMA-ONLINE

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CONGENITAL SMOOTH MUSCLE HAMARTOMA

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