Maria Emília Paiva

Congenital composite hemangioendothelioma: case report and reappraisal of the hemangioendothelioma spectrum.

Background:  Composite hemangioendothelioma is the most recently described entity of the hemangioendothelioma (HE) spectrum. To the best of our knowledge, only eight cases of this entity have been hitherto described. All of the previous cases affected adults; local recurrences were observed in three cases, and one case showed lymph node metastasis. We herein describe a new and previously unreported congenital case of this rare vascular tumor, arising on the acral extremity of the left forearm, which was diagnosed when the patient was 23 years old.

Composite adenomatoid tumor and myelolipoma of adrenal gland: report of 2 cases.

Adenomatoid tumor and myelolipoma are benign, hormonally inactive tumors that are often incidental findings in the adrenal glands. Myelolipoma is more common than adenomatoid tumor in this location but both are rare, and as yet, the pathogenesis of both remains unclear. We report 2 cases of composite adenomatoid tumor and myelolipoma, incidentally found in the adrenal gland on investigation for other diseases. To our knowledge, composite adenomatoid tumor and myelolipoma of adrenal gland has not been previously reported.

Dermatofibrosarcoma Protuberans: A Histological and Ultrastructural Study of 11 Cases with Emphasis on the Study of Recurrences and Histogenesis

Eleven cases of dermatofibrosarcoma protuberans (DFSP) (6 male: 5 female; mean age +/- SD: 48.7 +/- 16.1 years) were reviewed in an attempt to correlate the histological and ultrastructural findings with the recurrence pattern of these tumours and to shed light on their histogenesis. Six (54.5%) of the tumours recurred: 3 with a similar appearance to the primary tumours, 2 with fibroblastic malignant fibrohistiocytic pattern (F-MFH), and one with fibrosarcomatous features (F), confirmed ultrastructurally. In one primary tumour with associated F features there were two recurrences. The ultrastructural findings of 6 primary tumours (2 with and 4 without recurrences) showed a predominance of primitive mesenchymal cells. The comparison between the features (sex, age, site, necrosis, mitotic rate size) of recurring DFSP vs DFSP without recurrences did not yield any significant difference. At follow-up (range: 11-108 months) all patients were alive and well. These findings support the assumption that DFSP originates from primitive mesenchymal cells that are able to show, at least, F and F-MFH differentiation, and corroborate the low-grade malignancy of DFSP regardless of their macroscopic, histologic and ultrastructural characteristics.

Tumor-in-Tumor of the Thyroid With Basaloid Differentiation: A Lesion With a Solid Cell Nest Neoplastic Component?

This study describes an encapsulated thyroid tumor having 3 different concentric appearances in a 70-year-old man. The most peripheral neoplasm showed features of a microfollicular adenoma; the intermediate lesion displayed typical features of the follicular variant of papillary thyroid carcinoma (FVPTC); and the main, central neoplasm, showed a basaloid configuration. At variance with the other neoplasms, the latter component exhibited high mitotic activity. Necrosis, angio-invasion, or capsular invasions were not observed. The centrally located neoplasm was immunoreactive for several cytokeratins, p63, carcinoembryonic antigen, and galectin-3, and negative for thyroid transcription factor 1, thyroglobulin, calcitonin, CD5, and CK20, featuring a phenotype similar to that of thyroid solid cell nests. A N-RAS mutation was found both in the basaloid and in the FVPTC components. The clinicopathological and immunohistochemical data ruled out the alternative possibilities of intrathyroidal metastasis and tumor from ectopic (thymic, parathyroid, or salivary gland) tissues.

August 2003: 47‐year‐old female with a 7‐year history of osteomalacia and hypophosphatemia

The August 2003 COM. A 47-year-old woman presented with a long history of muscle pain, weakness, and visual disturbances. Over the last year, she developed diplopia and left sixth nerve palsy. No other neuro-ophthalmologic abnormalities were found. Past medical and family history was unremarkable. Laboratory investigation disclosed hypophosphatemia, phosphaturia, elevated serum alkaline phosphatase activity, and normal serum calcium levels. CT scans showed a lobulated mass arising on the meningeal surface of the cavernous sinus, measuring 3x 2 x 2 cm. The lesion was partially resected and microscopic examination revealed a highly vascularized tumor composed of primitive mesenchymal cells arranged whether in a patternless-pattern or surrounding thin-walled, branching vascular spaces and thick-walled hyalinized vessels. Other eye-catching features were microcystic areas, multinucleated osteoclastic-like giant cells, scattered islands of mature adipocytes, foci of hemorrhage, thrombosed medium-sized-to-large vessels, and hemosiderin-laden macrophages. After surgery, the patient recovered from the muscle pain and weakness, with a significant improvement of serum calcium and alkaline phosphatase levels and phosphate blood levels. Taken together, the clinical and pathologic features were consistent with a diagnosis of phosphaturic mesenchymal tumor (mixed connective tissue variant) of the cavernous sinus in a setting of oncogenic osteomalacia. No further treatment was carried out. The patient has been followed for the last 4 years with no evidence of recurrent disease. Oncogenic osteomalacia is a rare clinical entity characterized by hypophosphatemia, phosphaturia, and a low concentration of 1,25-dihydroxyvitamin D(3) caused by a neoplasm. Pathologists should be aware of this entity, because surgical excision of the tumor is usually curative.

Hodgkin's disease with predominant infradiaphragmatic involvement and massive invasion of the bone marrow. A necropsic study of nine cases

The authors report the results obtained in the necropsic study of nine cases of the so‐called acute, visceral form of Hodgkins disease (HD). Most of the patients (six men and three women, ranging from 42 to 74 years of age) lacked peripheral lymphadenopathies and had fever, weight loss, abnormality of hepatic function, and pancytopenia. Mixed cellularity was diagnosed in two, diffuse fibrosis in four, and reticular subtype of lymphocyte depletion in three cases. Despite the predominant infradiaphragmatic involvement, supradiaphragmatic lymph nodes were involved in six and tonsils in three cases. Spleen and bone marrow were involved in eight cases and the liver was involved in seven cases. In four cases there were also lesions in other extralymphoid organs. The involvement of the bone marrow was widespread and showed concurrent myelofibrosis and/or other signs of hematopoietic disturbance. There was a close relationship between the presence of vascular invasion (seven cases) and the extent of HD spread. It is concluded that despite its peculiarity, this form of HD fits the classic model of unicentric origin, lymphogenic contiguous spread, and hematogeneous dissemination, and should not be identified with any particular histologic type of HD.

Primary gastric adenosquamous carcinoma in a Caucasian woman: a case report

IntroductionMost gastric tumors are adenocarcinomas. Primary gastric adenosquamous carcinoma is a rare malignancy, mostly associated with Asian populations. It constitutes less than one percent of all gastric carcinomas and its clinical presentation is the same as adenocarcinoma. It occurs more frequently in the proximal stomach, usually presents with muscular layer invasion and tends to be found in advanced stages at diagnosis, with a worse prognosis than adenocarcinoma.Case presentationWe report the case of an 84-year-old Caucasian woman with an adenosquamous carcinoma extending to her serosa with lymphatic and venous invasion (T3N1M1). Nodal and hepatic metastasis presented with both cellular types, with dominance of the squamous component.ConclusionsAdenosquamous gastric cancer is a rare diagnosis in western populations. We present the case of a woman with a very aggressive adenosquamous carcinoma with a preponderance of squamous cell component in the metastasis. Several origins have been proposed for this kind of carcinoma; either evolution from adenocarcinoma de-differentiation or stem cell origin might be possible. The hypothesis that a particular histological type of gastric cancer may arise from stem cells might be a field of research in oncological disease of the stomach.

Round cell sarcoma after clear cell sarcoma remission

STEGER et al. [l] reported a relapse of round cell sarcoma after a complete remission of a clear cell sarcoma recurrence induced by treatment with interferon alphazb (IFN-a&. We have recently studied a similar case that was not treated with IFN-(wZh, thus raising new insights on the possible role of biological response modifiers in the natural course of clear cell sarcoma of tendons and aponeurouses (CSSTA), as well as providing additional evidence on the neural crest origin of CSSTA. The patient, a 26-year-old woman, presented with a slowgrowing tumour on the left popliteal region that she had noticed 6 years before (1982). The surgical specimen consisted of a whitish and fleshy mass, 10 cm in its largest dimension, infiltrating the musculo-aponeurotic tissues on the periphery. The surgical margins were free of tumour. Light microscopic study disclosed a fairly typical clear cell sarcoma without melanin pigmentation (Fig. la). The neoplastic cells were diffusely immunoreactive for S 100 protein and non-reactive with cytokeratin. Immunoreactivity for neuron specific enolase (NSE) was equivocal. Staging procedures failed to demonstrate lymphnode, visceral and bone metastases. She received local radiotherapy over 1 month (6400 Rads), followed by ten sessions every 28 days of a polychemotherapy protocol (day 1: 70 mg doxorubicin + 400 mg dacarbazine; days 2-5: 400 mg dacarbazine) . 15 months later the patient presented with two subcutaneous nodules on the right deltoid (1.5 cm) and left thoracic (2.1 cm) regions. Staging showed multiple bone metastases (head, ribs, dorsolumbar vertebra and pelvis). There were neither nodal and visceral metastases nor any signs of local recurrence of the tumour. Light microscopy of the subcutaneous nodules revealed a round cell sarcoma with Hommer-Wright rosettes (Fig. lb), immunoreactive for SlOO protein and NSE. Palliative chemotherapy (25 mg actinomycin + 2 mg vincristine + 6 g ifosfamide) was given. The patient died 29 months after initial surgery. Necropsy was not done. As in the recurrence of the case reported by Steger et al. the subcutaneous metastases of our patient displayed the typical features of a round cell sarcoma consistent with a primitive neuroectodermal tumour. However, in contrast to this case, Fig. 1. (a) Clear cell sarcoma with typical pattern in the primary tumour (haematoxylin-eosin x 125); (b) monomorphic proliferation of round cells with scanty cytoplasm displaying Hommer-Wright rosettes in the subcutaneous metastasis (haematoxylin-eosin, X 250).