Epithelioid hemangioendothelioma is an uncommon malignant vascular tumor of intermediate grade that occurs in a variety of organs and soft tissues. Diagnosis of epithelioid hemangioendothelioma is often complicated by the rarity of the tumor, and because the tumor shares many morphologic features with other peritoneal neoplasms. This report presents 3 cases of epithelioid hemangioendotheliomas arising as primary tumors of the peritoneum and reviews 7 previously reported cases to establish the demographic, clinical, morphologic, immunohistochemical, and ultrastructural features of this neoplasm. These cases demonstrate that the light microscopic features are very similar to epithelioid hemangioendothelioma arising at more conventional sites, such as the liver, that immunohistochemical analysis provides a reliable approach for confirming or establishing the diagnosis, and that at least one endothelial marker (either CD31, CD34, or factor VIII) should be positive for a definitive diagnosis. This series identifies the characteristics of peritoneal epithelioid hemangioendothelioma that can be reliably used for diagnosis.
We report the features in fine-needle aspiration biopsy (FNAB) of thymic basaloid carcinomas. This is a rare neoplasm, of which there are only three documented in our hospital files. To the best of our knowledge, this is the first fine-needle aspiration (FNA) report on basaloid carcinoma of the thymus. This is a tumor in which the FNA diagnosis is difficult and the differential diagnosis is broad. We describe the cytologic features encountered in the three cases, and immunohistochemical and ultrastructural findings so as to raise awareness of this entity in the differential diagnosis of thymic neoplasms on FNABs. The cases studied included three male patients, aged 73, 65, and 50, who presented with anterior mediastinal masses, with no primary tumor elsewhere. FNAB was performed on two cases, followed by thymectomy. One case, additionally, had metastasis to a cervical lymph node, and the other two were associated with thymic cysts. The diagnoses on all three cases were thymic basaloid carcinoma.
A case of diffuse-type tenosynovial giant cell tumor arising in the left upper extremity is reported in a 23-year-old man with neurofibromatosis type 1 (NF1)-Noonan syndrome. The predominately mononuclear cellular proliferation with psammomatous calcifications had the immunohistochemical and ultrastructural features of a fibrohistiocytic neoplasm. This uncommon type of soft tissue neoplasm occurring in this unique clinical setting served to open an inquiry into the subject of non-neurogenic tumors in association with NF1 and Noonan syndrome, both manifested in our patient. Nonossifying fibroma of bone as a presumptive fibrohistiocytic tumor is an uncommon but well-documented manifestation in NF1, whereas in Noonan-like syndrome, both giant cell granuloma and pigmented villonodular synovitis are recognized as associated lesions with histologic and immunophenotypic similarities with the diffuse-type tenosynovial giant cell tumor.
Some patients with bland smooth muscle tumors in the uterus have synchronous or asynchronous smooth muscle tumors in the peritoneum and/or the retroperitoneum. It is usually assumed that the uterine tumor is the primary lesion, and the extrauterine neoplasm represents its metastasis. Thus, they are designated as low-grade leiomyosarcomas because they lack the diagnostic features of a conventional spindle cell leiomyosarcoma. Nineteen such cases were retrieved from the files of the Department of Pathology at The University of Texas M.D. Anderson Cancer Center, covering a period of 18 yr. Institutional Review Board approval was obtained before the initiation of this study. In addition, 31 cases of conventional uterine leiomyosarcomas of a high grade were reviewed for comparison. Clinicopathologic features such as patients' age, tumor location, histologic features, stage, treatment, and follow-up were recorded. Immunohistochemical stains for estrogen receptor (ER), progesterone receptor (PR), p53, Ki-67, and WT-1 were performed in the initially detected tumor and the subsequent neoplasm of all cases with available material in the low-grade group and selected cases in the high-grade group. Compared with high-grade leiomyosarcomas, the low-grade group cases were found at an early age (45 vs. 52.8 yr), had a longer median time of "recurrence" (42 mo for the low-grade group vs. 12 mo for high-grade leiomyosarcomas), longer median survival (165 mo for the low-grade group vs. 41 mo for the high-grade group), and a much better overall survival (84% vs. 13%). Three (16%) patients died of disease in the low-grade group versus 27 (87%) patients in the high-grade leiomyosarcoma group. We also found a difference in the location of the extrauterine tumors. Most cases of low-grade tumors were found in the pelvis, abdomen, or retroperitoneum, whereas most high-grade leiomyosarcomas involved the lung. In the low-grade tumors, there were some differences in the immunophenotype between the uterine and the extrauterine neoplasms, but in the high-grade tumors, there were no differences in the immunohistochemistry between the primary tumor and the metastasis. In addition to these differences between the 2 groups in the age of the patients, sites of recurrences, and the immunophenotype of the uterine and extrauterine tumor, neither the uterine nor the extrauterine low-grade lesions had histologic features of malignancy. On the basis of these differences, the possibility that the extrauterine lesions in the low-grade group represent independent primaries involving the secondary mullerian system is proposed.
Neuroendocrine carcinomas of the endometrium are rare tumors that can be pure, combined with endometrioid adenocarcinoma, or a component of malignant mixed müllerian tumor. Recently, a case of combined small cell carcinoma and papillary serous carcinoma of the endometrium was described for the first time. We report the first case, to our knowledge, of combined large cell neuroendocrine carcinoma and papillary serous carcinoma of the endometrium, with an unusual pagetoid spread of the neuroendocrine component into normal endometrial and endocervical glands. The endometrial carcinoma had a small serous component, but most of the tumor was characterized by solid sheets of medium to large cells with abundant mitotic figures, numerous apoptotic bodies, and foci of necrosis. This component was diffusely positive for neuroendocrine markers. Following surgery, the patient was treated with radiation therapy and chemotherapy. She was without evidence of progression at 5 months of follow-up.
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