Malignant extragastrointestinal stromal tumor presenting as a vaginal mass: report of an unusual case with literature review.

The clinical and pathologic features of a malignant extragastrointestinal stromal tumor presenting as a vaginal mass are discussed. A 66-year-old female presented with copious vaginal bleeding and spontaneous passage of tumoral tissue per vagina. Histologic assessment showed a mitotically active spindle cell neoplasm. Immunohistochemical analysis demonstrated the neoplasm to be positive for CD117 (transmembrane tyrosine kinase) and CD34, consistent with a malignant extragastrointestinal stromal tumor. Subsequent clinical examination revealed an 8-cm posterior vaginal wall mass, with probable origin from the rectovaginal septum. This case is unique based on the primacy of presenting gynecologic complaints, and the unusual anatomic location of the lesion. A literature review of the pathologic features of extragastrointestinal stromal tumors and factors predictive of biologic behavior are discussed. Correct tumor diagnosis is emphasized given the effective treatment possible with imatinib for patients with unresectable tumors.

Interdigitating dendritic cell sarcoma. A report of four cases and review of the literature.

To better define the clinical and pathologic features of interdigitating dendritic cell sarcoma (IDCS), we report 4 cases, including the first reported in the tonsil. There were 2 male and 2 female patients (mean age, 70 years). Sites of tumor included 1 case each in the right cervical lymph node, left axillary lymph node, right tonsil, and right inguinal lymph node. Histologically, all showed diffuse effacement of the lymphoid tissue by pleomorphic round to spindled cells with convoluted nuclei and abundant eosinophilic cytoplasm. All were immunoreactive for S-100, CD68, lysozyme, and vimentin. CD45 was positive in 3 cases and CD1a in 1 case. Fascin was positive in 3 cases. Other immunostains, including CD3, CD20, CD21, CD30, actin, cytokeratin, and HMB-45, were negative. Ultrastructurally, the tumor cells were elongated and showed indented nuclei, variable numbers of lysosomes, and interdigitating cytoplasmic processes. Follow-up was available for all cases. One patient died of widespread disease 2 months after diagnosis. One was alive with metastatic lung disease at 12 months. Two patients were disease free at 5 and 9 months.

Pulmonary and mediastinal glomus tumors--report of five cases including a pulmonary glomangiosarcoma: a clinicopathologic study with literature review.

Pulmonary and mediastinal glomus tumors are rare lesions, with four previously reported primary pulmonary cases and three mediastinal cases. The authors report one mediastinal glomus tumor, a locally infiltrative type, and four pulmonary glomus tumors, including the first case of primary pulmonary glomangiosarcoma. These tumors show a variety of clinical and pathologic differences from the more common cutaneous variety, including later age at presentation, larger size, and more frequent atypical/malignant features. Mediastinal and pulmonary glomus tumors both have an average patient age at presentation of 45 years. However, compared with their pulmonary counterparts, mediastinal glomus tumors are less common, more often symptomatic, and are larger (average size, 5.4 cm). Additionally, mediastinal glomus tumors more often demonstrate malignant or atypical features. Pulmonary glomus tumors average 3.3 cm in greatest dimension, with the majority measuring less than 2.5 cm. The pulmonary glomangiosarcoma presented was large, measuring 9.5 cm, and showed increased mitotic count (9 mitoses/10 high-power fields), necrosis, cytologic atypia, and was associated with disseminated disease. Regardless of clinical symptoms, histologic features, and even metastases, the vast majority of all benign and malignant glomus tumors are indolent and cured surgically, with adjuvant therapy needed only for occasional patients with more advanced disease. The four patients with glomus tumors reported are currently alive and free of disease as of last follow up. The patient with the glomangiosarcoma developed widespread metastases and died of disease 68 weeks after initial therapy.

Collision of uterine rhabdoid tumor and endometrioid adenocarcinoma: a case report and review of the literature.

Extrarenal malignant rhabdoid tumors have been reported in a variety of anatomic sites but infrequently in the female genital tract. In the uterus, they have been described as a pure tumor, in association with endometrial stromal sarcomas, and as a component of a malignant mullerian mixed tumor. This study reports an unusual uterine neoplasm in a 49-year-old woman, in which a malignant rhabdoid tumor occurred as a collision tumor with a well-differentiated endometrioid adenocarcinoma. The tumor was a 14-cm polypoid mass that filled the endometrial cavity. The two neoplastic components were distinct on microscopic and immunohistochemical examination. Ultrastructural examination confirmed the rhabdoid phenotype of the sarcomatous component. The patient died of disease 4 months after diagnosis with progression of the malignant rhabdoid tumor. The highly aggressive behavior of the rhabdoid (i.e., nonepithelial) component in this collision tumor lends support for a distinction of this neoplasm from a malignant mullerian mixed tumor, with which it may be confused.

The columnar cell variant of thyroid papillary carcinoma. Case report and discussion of an unusually aggressive thyroid papillary carcinoma.

The columnar cell variant of thyroid papillary carcinoma is an aggressive tumor associated with widespread dissemination and a fatal outcome. We report a case of a 29-year-old white woman who presented with a large thyroid mass extending into the mediastinum with local and distant metastases. The histologic features included focal papillary growth with columnar cells and nuclear stratification. However, the histologic picture was dominated by cells with a clear to vacuolated-appearing cytoplasm similar to that seen in association with secretory-type endometrium. In addition, areas of solid growth and organoid or glandular features were also identified. This tumor followed an aggressive course with widespread metastatic disease unresponsive to all therapeutic intervention. The patient died 7 months after diagnosis. We believe this tumor represents the columnar cell variant of thyroid papillary carcinoma. This is only the second reported case of columnar cell carcinoma to occur in a female. Other than the unusual occurrence in a woman, the clinical and pathologic features of this case are similar to those previously reported, including an aggressive behavior followed rapidly by the death of the patient.