Two-stage resection of a disseminated mixed endometrial stromal sarcoma and smooth muscle tumor with intravascular and intracardiac extension.

OBJECTIVE: Mixed endometrial stromal and smooth muscle tumor (MESSMT)-a rare mesenchymal uterine tumor of the uterus with atypical clinical symptoms-is susceptible to misdiagnosis and missed diagnosis. We report a case of a disseminated MESSMT with intravenous and intracardiac extensions treated with staging surgery and review previously documented cases of such tumors with intracardiac extension. CASE REPORT: The case involves a 45-year-old woman with disseminated MESSMT that originated in the uterus and progressed through the iliac vein, inferior vena cava, right atrium, and into the right ventricle, which closely resembled intravenous leiomyomatosis (IVL) grossly and microscopically. She presented with a 1-year history of dyspnea on exertion. IVL was highly suspected preoperatively based on computed tomography and magnetic resonance imaging findings. Two-stage surgeries were performed successfully. The postoperative pathology indicated a disseminated MESSMT. CONCLUSION: This case illustrates the important role of pathology and immunohistochemistry in the differential diagnosis of a rare tumor that mimics the characteristics of IVL with intracardiac involvement and demonstrates the therapeutic strategy for this rare entity.

Mixed endometrial stromal and smooth muscle tumor: report of a case with focal anaplasia and early postoperative lung metastasis.

A rare case of a mixed endometrial stromal and smooth muscle tumor arising in the uterus of a 74-year-old woman is reported. The patient underwent hysterectomy for an enlarging uterine mass, and a large intramural tumor, showing marked central hyaline necrosis with calcification, was found. The tumor consisted of an admixture of a low-grade endometrial stromal sarcoma (ESS) and a fascicular proliferation of spindle cells suggesting smooth muscle differentiation, and a characteristic 'star-burst' appearance was found. In the ESS region, there were a few small foci of anaplasia where large polygonal cells with atypical nuclei and abundant eosinophilic cytoplasm proliferated, and the proliferative activity was locally increased in these foci. A small metastatic nodule appeared in the lung nine months after the hysterectomy, and the resected metastatic lesion showed features of anaplastic spindle cell sarcoma which was immunoreactive for CD10 but not for smooth muscle markers. Mixed endometrial stromal and smooth muscle tumors should be regarded as malignant neoplasms with the potential for hematogenous metastasis, particularly when they contain foci of cellular anaplasia.

Multifocal osteolytic lesions within lumbar spine in a middle-aged Chinese woman: a benign metastasizing leiomyoma?

STUDY DESIGN: Case report. OBJECTIVE: To describe a case presented as osteolytic lesions involving 2 inconsecutive lumbar, which was pathologically proved to be of smooth muscular origin. SUMMARY OF BACKGROUND DATA: Smooth muscle tumor of uncertain malignant potential (STUMP) could be diagnosed when the neoplasm showed some malignant behavior, whereas its histologic image revealed very low malignance. A 47-year-old woman presented with low back pain and numbness of the left lower extremity attributable to osteolytic lesions in the second and fourth lumbar vertebral bodies, which was subsequently diagnosed as STUMP. A benign metastasizing leiomyoma was highly suspected. METHODS: Clinical, pathologic, and radiologic characters of the patient with STUMP confined within the lumbar spine were retrospectively reviewed. A mini review was also conducted. RESULTS: We describe the first spine-confined case of STUMP. CONCLUSION: Significant new information in the growing literature of this rare and newly identified diagnosis has addressed the need for orthopedists to consider the STUMP diagnosis.

Uterine smooth-muscle tumors with unusual growth patterns.

Uterine smooth-muscle tumors with unusual growth patterns include a variety of neoplastic lesions characterized histologically by their similarity to adult smooth muscle tissue. The 3 primary neoplasms are intravenous leiomyomatosis, benign metastasizing leiomyoma, and leiomyomatosis peritonealis disseminata. We present a review of the recent literature along with the clinical and pathologic features of 5 illustrative cases from Mayo Clinic Arizona, Scottsdale.

Malignant perivascular epithelioid cell tumor ('PEComa'): a case report and literature review of cutaneous/subcutaneous presentations.

Perivascular epithelioid cell (PEC) tumors, also called 'PEComas,' are distinct tumors showing PEC differentiation with characteristic histologic and immunophenotypic features. PEComas are rare tumors documented in the literature presenting in numerous anatomic sites including the thorax, abdomen, pelvis, soft tissue and skin. Criteria for malignancy does not exist for the subset of PEComas that pursue an aggressive clinical course. Herein, we present an unusual case of a malignant PEC tumor presenting as a scalp nodule in a patient with a prior diagnosis of 'melanoma' based upon the immunophenotypic profile of an excised enlarged cervical lymph node. The purpose of this case presentation is to further describe the rare clinical manifestations of a subcutaneous PEC tumor, emphasize the malignant potential of this entity, and review the literature focusing upon clinicopathologic features of cutaneous/subcutaneous PEComas.

Epstein-Barr virus-associated smooth muscle tumour mimicking bilateral vocal process granuloma.

A case is presented of a 36-year-old Chinese woman with a renal transplant for end-stage renal failure due to Goodpasture's syndrome. She presented with a year's history of throat discomfort and acid regurgitation into her throat. Videolaryngoscopy revealed bilateral vocal process granuloma, presumed to be due to gastroesophageal reflux. A four-week course of high dose omeprazole was prescribed. On follow up a month later, the granulomas had enlarged, and laser excision was undertaken. Histological and immunohistochemical staining was consistent with Epstein-Barr virus-associated smooth muscle tumour. This is believed to be the first reported case in the English literature of such a tumour affecting the vocal process. The aim of this paper is to present the pathogenesis, clinical behaviour and treatment of Epstein-Barr virus-associated smooth muscle tumour, and to review the literature concerning the differential diagnosis of polypoid vocal process lesions.

Single-stage resection of a mixed endometrial stromal sarcoma and smooth muscle tumor with intracardiac and pulmonary extension.

A case of mixed endometrial stromal sarcoma and smooth muscle cell tumor of the uterus with intravenous extension into the right heart and pulmonary artery is presented. The current literature and diagnostic and therapeutic strategies of pelvic tumors with intravenous extension are reviewed.