Benign schwannoma of the digestive tract: a clinicopathologic and immunohistochemical study of five cases, including a case of esophageal tumor.

We report five cases of schwannomas of the digestive tract. The patients were two men and three women, whose ages ranged from 56 to 74 years. Three cases arose in the stomach, one in the ascending colon, and one in the esophagus; the latter was a hitherto unreported location for this tumor. The schwannomas ranged from 2 to 11 cm in diameter. They were well circumscribed but not encapsulated, with interlacing bundles of spindle cells, nuclear atypia and no mitosis, interspersed with collagenous strands. Inflammatory cells were scattered throughout the tumors and a peripheral cuff of lymphoid aggregates was observed in all cases. Intracellular periodic acid-Schiff (PAS)-positive crystalloids were found in three cases; no skeinoid fibers were seen. A diffuse and intense positivity for vimentin and S-100 protein was detected in all five cases together with a variable and sometimes focal positivity for glial fibrillary acidic protein and neuron-specific enolase. None of the tumors showed expression of CD34 or the smooth muscle antigens tested. The four cases with a sufficient follow-up had a favorable outcome without any recurrence or metastasis. The morphologic and immunohistochemical features of digestive schwannomas were compared with those of other gastrointestinal stromal tumors. Schwannomas have many differences. Digestive schwannomas can be readily recognized on histologic and immunohistochemical examination. They are spindle cell tumors without epithelioid features, with a peripheral cuff of lymphoid tissue. Specific intracellular needle-shaped PAS-positive crystalloids are found in some cases, whereas skeinoid fibers are not. These tumors always express S-100 protein in a diffuse and strong manner, and they express glial fibrillary acidic protein but not express CD34. Digestive schwannomas usually are gastric tumors and have never been reported in the small bowel. They pursue a benign course and are far rarer than gastrointestinal autonomic nerve tumors.

Detection of Epstein Barr virus in an hepatic leiomyomatous neoplasm in an adult human immunodeficiency virus 1-infected patient.

We report the first case of a human immunodeficiency virus (HIV)-related primary hepatic leiomyoma in an adult patient. The diagnosis was made at autopsy and confirmed by immunohistochemistry. Epstein Barr virus (EBV) was identified in tumour cells by in situ hybridization. Review of the literature revealed 13 cases of visceral myogenic tumours occurring in acquired immunodeficiency syndrome children, and only 2 cases in adults. One was a spinal epidural leiomyoma, the other multiple smooth muscle tumours of the colon and adrenal gland. This is the first report of EBV in smooth muscle neoplastic cells in an HIV-infected adult patient.

Is it possible to have an international program of pregraduate pathology?

The idea of an international program of pregraduate pathology is attractive, but this program might be difficult to establish for several reasons. 1) Even if one admits that the student has no need to know pathology for itself and must only know that part of pathology useful for medical practice, it is still difficult to decide what is really useful. As a matter of fact, this varies in a great extent from country to country. 2) A program considered far to extensive by some schools would be considered ridiculously limited by others. 3) Overall, one might be concerned that an international program would be inaccurate in most cases.

An European pathologist?

The European pathologist already does exist, at least in the regulations of European communities. On the 16th of June 1975, the Council of the European Community stated: 1. The post-graduate course of pathology can only be undertaken by students graduated following a course of 6 years. 2. The post-graduate course of pathology does necessarily require 4 years of full time theoretical and practical teaching, in a University hospital or Institute. The training must include participation of the student in activities and responsibilities of the department. The Council must reexamine the situation this year and consider the possibility of part-time training. Nothing is said about a terminal examination, although this examination is legally required in some countries. Nothing states that the student has to spend the four years of residency in the same place and he could probably train in different countries of the community and change every year. The students of countries which are not in the community at present are not mentioned. If a student has the opportunity to achieve his education in different countries of Europe would this be enough to define an European pathologist? I do not think so, because there is no common doctrine of pathology among Europeans. For instance, some topics in German pathology simply have no equivalent, neither in the thought, nor in the language of a French pathologist, and viceversa. However, the common language of pathologists throughout Europe will certainly be English. This must contribute to unifying the ideas, but the ideas will probably not be specifically European.

Endometrial stromal sarcoma of the rectosigmoid colon arising in extragonadal endometriosis and revealed by portal vein thrombosis.

Malignant transformation is an infrequent complication of endometriosis. The ovary is the primary site in 76% of cases, and extragonadal sites are identified in 24%. Endometrioid carcinoma is the most common histologic type; sarcoma is very rare. We report a case of low-grade endometrial stromal sarcoma of the rectosigmoid colon presenting with epigastric pain due to portal vein thrombosis. This tumor arose from extragonadal endometriosis in a 61-year-old woman and was treated by surgical resection. The main differential diagnosis of this unusual colonic neoplasm includes primary mesenchymal tumors, such as gastrointestinal stromal tumors.

[Atypical decubitus fibroplasia: a recent entity. Apropos of a case of an adolescent girl]. / La fibroplasie atypique de décubitus: une entité récente. A propos d'un cas chez une adolescente.

Atypical decubital fibroplasia (FAD) occurs especially in elderly and physically debilited or immobilized patients. We report one observation which is peculiar due to the patient's young age and its circumstances. The painless mass is situated in hyperpressure areas (shoulder, posterior or lateral chest wall, sacrum). The lesion is situated in the deep subcutis and has ill defined limits; it is characterized by zones of fibrinoid necrosis and fibrosis and a prominent myxoid stroma. The differential diagnoses includes mesenchymatous malignant tumors and non neoplastic fibroblastic proliferations such as proliferative fasciitis and decubitus ulcer. The prominent underlying factor and the initial event contributing to its pathogenesis seems to be ischemia. Although some recurrent cases have been reported, FAD is a benign lesion whose treatment is surgical removal.

[Malacoplakia. Apropos of a case of retroperitoneal site]. / Malacoplasie. A propos d'une observation avec localisation rétropéritonéale.

Malacoplakia is a granulomatous inflammatory disease caused by a disorder of macrophage bactericidal function. The disease, histologically characterized by the presence of Michaelis-Gutmann bodies, has a preference for the urogenital tract and less frequently affects the gastrointestinal tract and the retroperitoneal region. We report the unusual case of a male patient who presented with retroperitoneal pseudotumoral malacoplakia associated with vesical malacoplakia. The main pathophysiological, diagnostic and therapeutic aspects of this rare disease are described.

[Retiform hemangioendothelioma]. / Hémangioendothéliome rétiforme.

Hemangioendotheliomas are vascular tumors of intermediate malignancy considered as low-grade angiosarcomas. The authors report two cases of a distinctive type, named retiform hemangioendothelioma because of its particular morphologic features, and recently delineated by Calonje and al. This tumor of the skin recurs frequently but has a very low metastatic rate.

[Primary leiomyosarcoma of bone: a clinico-pathologic and immunohistochemical study of 3 cases]. / Léiomyosarcomes primitifs de l'os: étude anatomo-clinique et immunohistochimique de 3 observations.

Primary leiomyosarcoma of bone is rare. Herein we describe 3 cases with immunohistochemical study. All 3 cases were positive for smooth muscle actin and/or desmin. None was positive for cytokeratin. Differential diagnosis includes metastatic spindle cell carcinoma, other sarcoma (fibrosarcoma, malignant fibrous histiocytoma) and metastatic extra-osseous leiomyosarcoma, mostly from uterus or digestive tract.

[Carcinosarcoma arising in eccrine spiradenoma. A morphologic and immunohistochemical study]. / Carcinosarcome développé sur un spiradénome eccrine. Etude morphologique et immuno-histochimique.

Malignant change in a benign eccrine spiradenoma is a rare but definite phenomenon. Since it was first reported by Dabska, 23 cases have been reported. All cases present recent changes in a long standing cutaneous nodule which, when excised and examined microscopically, disclosed carcinoma associated and arising from benign eccrine spiradenoma. Eight patients died of disseminated tumours. Histologic features, in three fatal cases, showed carcinosarcoma and particularly osteosarcoma in one case. A further fatal case of carcinosarcoma arising in spiradenoma, is reported in a Melanesian man aged 38, who died after 8 months. Histologic examination showed squamous cell carcinoma associated with osteosarcoma in the recurrence.

[Cellular angiofibroma. A rare vulvar tumor. Report of a case]. / L'angiofibrome cellulaire, une tumeur vulvaire rare. A propos d'un cas.

Cellular angiofibroma is a rare tumor. We report a vulvar case in a 37 year old woman. This nodular, well circonscribed tumor consists of bland spindle cells, numerous thin or thick often hyalinized vessels and adipocytes. The stromal cells are positive for vimentin and negative for CD34, protein S100, smooth muscle actin, desmin, epithelial membrane antigen and cytokeratin. Cellular angiofibroma is a benign tumor that has to be differentiated from aggressive angiomyxoma, angiomyofibroblastoma, glomangiopericytoma, spindle cell lipoma, solitary fibrous tumor and perineurioma.

[Tumor of the peripheral nerve sheath with rhabdomyoblastic differentiation arising in a ganglioneuroma: neurilemmoma? A fortuitously detected case]. / Tumeur des gaines des nerfs périphériques à différenciation musculaire survenant sur un ganglioneurome: tumeur de Triton?

An otherwise healthy 21-year-old man with no evidence or family history of Von Recklinghausen's neurofibromatosis presented a posterior mediastinal mass detected on routine chest radiographs. The findings of standard light microscopy, ultrastructural examination and immunohistochemical studies suggested the working hypothesis of an unusual malignant "Triton" tumour: -arising in a pre-existing de novo ganglioneuroma; -fortuitously detected; -predominantly made of multinucleated spindle cells with only ultrastructural and immunohistochemical rhabdomyoblastic differentiation.

[Rare tumors of the prostate or seminal vesicles. Report of a case of leiomyosarcoma]. / Les tumeurs rares de la prostate ou des vésicules séminales. A propos d'un cas de lélomyosarcome.

An unusual case of leiomyosarcoma of the prostate presented as a recurrent pelvic cystic mass. Prostatic sarcoma are rare in adults with a poor prognosis. It is often difficult to determine a definite origin. The authors reviewed the differential diagnosis with pseudosarcoma, inflammatory fibrosarcoma and others rare tumors of the prostate and the seminal vesicle.

[Botryoid rhabdomyosarcoma of the cervix. Clinico-pathologic study of a case]. / Rhabdomyosarcome botryoïde du col utérin. Etude anatomo-clinique d'un cas.

The botryoid rhabdomyosarcoma of the cervix is a rare tumour occurring in young woman or during genital activity. It mainly causes vaginal bleeding or appears as a polypoid grape-like mass with a gelatinous cut-surface. The diagnosis is based on the presence of a submucosal cambium layer and a rhabdomyoblastic differentiation, corresponding to an intracytoplasmic double cross-striation. Metaplastic cartilaginous islands are sometimes observed. The immunostaining ensures the muscular origin of the tumour, characterized by the expression of actin and desmin. Apart from mullerian adenosarcomas, the main differential diagnosis is represented by the benign polypoid formations of the cervix: the genital rhabdomyoma and the fibroblastic lesion, called fibro-epithelial polyp with atypical stroma. The treatment actually includes both chemotherapy and surgery which is often limited to a conization.

[MRI appearance of a primary leiomyosarcoma of bone with epiphyseal involvement]. / Aspect IRM d'un cas de léiomyosarcome osseux primitif de localisation épiphysaire.

Primary leiomyosarcoma of bone is a rarely reported tumor of elderly subjects. It usually shows an aggressive osteolytic pattern on plain radiographs and involves predominantly the metaphyses of long bones. We report a case of primary leiomyosarcoma of bone, which is atypical by its epiphyseal location, a non-aggressive pattern on plain radiographs and its MR imaging features.

[Peroperative colposcopic examination of conization tissue. Determination of the complete or incomplete nature of the cone]. / Colposcopie per-opératoire d'une pièce de conisation. Mise en évidence de son caractère complet ou incomplet.

The authors describe an original technique which makes it possible for them to determine during the operation whether conisation has been complete or incomplete. The tissue removed is examined during the operation colposcopically after it has been stained with an aqueous solution of toluidine colour blue. Pathological epithelium can be recognized easily and it is possible to measure the distance between the edge of this epithelium and the tissue that has been removed. If it is inadequate more can be cut out immediately. The distance should be at least 5 mm from the edge of the pathological epithelium.

[Mixed corticomedullary tumor]. / Tumeur mixte corticomédullaire.

BACKGROUND: Pheochromocytoma and primary hyperaldosteronism rarely occur simultaneously. Few cases have been reported in the literature. CASE REPORT: A patient explored for hypertension was found to have hypokalemia related to primary hyperaldosteronism. Pathology examination of the ablated adrenal showed a co-existing pheochromocytoma suspected at history taking although urine catecholamines were normal. DISCUSSION: Different pathogenic hypothesis have been proposed. Such dual tumors could be a simple coincidence, occur in a particular genetic setting, be related to direct contact between cortical and medullary tissue leading to reactional cortical hyperplasia, pheochromocytoma produced factors stimulating aldosterone synthesis, or factor X, a substance produced by cortical adenomas and favoring growth of the pheochromocytoma.

[Imaging of ORL diseases in acquired immunodeficiency syndrome]. / Imagerie des pathologies ORL au cours du syndrome d'immunodéficience acquise.

More than 50% of patients with acquired immunodeficiency syndrome (AIDS) present a lesion affecting parotid gland, lymph nodes, paranasal sinuses, pharyngo-larynx or temporal bone. In about 20% out of cases affected patients present different head and neck lesions at the time of evaluation. Most often, clinical examination and endoscopy are sufficient to perform diagnosis and to manage the disease. Imaging studies (CT or MRI) are indicated when the nature of the disease is unknown or when a map of the process is mandatory for therapeutic approach. Some imaging features are strongly suggestive of HIV infection: parotid cysts associated with hyperplasia of the nasopharynx and cervical lymph nodes enlargement; labyrinthitis and multinevritis; and head and neck squamous cell carcinomas in non alcoolo-tobacco addicted patients. Such diseases suggest the need for knowledge of the patient's seropositivity status.