Non-alcoholic duct destructive chronic pancreatitis: a histological, immunohistochemical and in-situ apoptosis study of 18 cases.

AIMS: To assess retrospectively pancreatic changes in non-alcoholic duct-destructive chronic pancreatitis and to investigate the role of apoptosis in duct destruction. METHODS AND RESULTS: Eighteen patients (mean age 46 years, nine women and nine men) underwent pancreatic resection for suspected pancreatic tumour and were diagnosed as having non-alcoholic duct-destructive chronic pancreatitis. We performed a morphological study either semiquantitatively (fibrosis and inflammation) or quantitatively (CD3+ intraepithelial lymphocytes, M30 and TUNEL+ apoptotic cells). The results were compared with those obtained in 10 cases of chronic alcoholic pancreatitis and nine cases of chronic obstructive pancreatitis. Pancreatic changes were diffuse and heterogeneous in 14 cases, but segmental in four cases. The main pancreatic lesions were ductal epithelial alteration, periductal inflammation and fibrosis. There were no cysts or calcifications. We found a marked increase in mast cells in the infiltrate, a slight increase in TiA1+ lymphocytes and in intraepithelial lymphocytes compared with other types of chronic pancreatitis. No significant increase in ductal apoptosis was observed. CONCLUSION: Non-alcoholic duct-destructive chronic pancreatitis is a well-defined pathological entity, distinct from alcoholic and obstructive chronic pancreatitis. Our results indicate that apoptosis probably does not play a major role in ductal alterations.

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.

[Thyroid insular carcinoma]. / Le carcinome insulaire thyroïdien.

This review article illustrates the several histological and immunohistochemical patterns of thyroid insular carcinoma and their associated disease. Differential diagnosis are also discussed. Poorly differentiated thyroid carcinomas are overviewed. The interest of transgenic mice models is presented.

[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.

Solitary fibrous tumor of the adrenal gland.

Solitary fibrous tumors are rare neoplasms, most commonly involving the pleura, recently described in various other locations. We report a solitary fibrous tumor of the right adrenal gland in a 42-year-old woman, discovered incidentally during abdominopelvic ultrasonographic examination. Pathologic and immunohistologic features of the tumor were identical to those of other solitary fibrous tumors. Three-quarters of this unencapsulated infiltrating tumoral mass presented foci of hemorrhage and were made of small, round, epithelioid-like cells that expressed the CD34 antigen more weakly than do the typical spindle cells usually observed in solitary fibrous tumors. Despite hemorrhage and poor limitation, the tumor behaved in a innocuous manner; the mass remaining unchanged for more than 5 years before the patient agreed to surgical intervention, which was recommended because of a sudden enlargement of the mass.

[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.

[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.

Clear cell sarcoma of the pre-parotid region: an initial case report.

An unusual case of clear cell sarcoma (CCS) found in the parotid region of a 75-year-old man with a long history of chronic lymphoid leukemia is reported. Treatment of the patient included a total parotidectomy with preservation of the facial nerve. The tumor was pathologically consistent with a clear cell sarcoma. Since CCS originates from the neural crest and is melanin producing, we suggest that this particular tumor originated from the superficial musculo-aponeurotic system (SMAS). To our knowledge, this is the first case of CCS that has been reported in the parotid region.

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.

[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.

[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.

Primary liposarcoma of the heart.

We report the eleventh illustrated case of primary liposarcoma of the heart in a 28-year-old man. The tumor arose from the mitral valve and the left ventricle and was revealed by a solitary brain metastasis 1 year before. The primary tumor and the metastasis were surgically removed. The cardiac and brain neoplasms were myxoid liposarcoma, as confirmed by histochemical, immunohistochemical, and ultrastructural studies. Although review of the literature points out the poor survival of patients with malignant liposarcoma of the heart, no recurrence or metastasis was observed after a 6-month follow-up.

[Anatomopathological aspects of arterial angiodysplasias]. / Aspects anatomopathologiques des angiodysplasies artérielles.

Arterial angiodysplasias constitute an ill-defined entity comprising conditions of apparently very variable natures. Apart from arterial fibrodysplasia, the clinical features of which, if not the etiology, are well established, there exists a number of different arterial abnormalities, stenotic or aneurysmal, the significance of which remains unclear (dolicho-arteries, the Moya-Moya syndrome, etc.). However, the role of genetic factors and metabolic abnormalities in these conditions is uncontestable. They demonstrate both the authenticity of this group of diseases and the importance of metabolic disorders of the interstitial tissues in the majority of these vascular abnormalities.

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.