Primary synovial sarcoma of the heart. A clinicopathologic study of one case and review of the literature.

Primary synovial sarcomas of the heart are aggressive and extremely rare tumors. Only thirteen cases have been reported previously. We report the fourteenth case and review the previous ones. Our patient is a 45 year-old man who presented with congestive heart failure, which was related to a right atrial pedunculated tumor. The tumor was resected completely and the patient has remained disease-free after 5 years. The histopathologic examination and immunohistochemistry study confirmed the diagnosis. Thirteen cases are reported in the literature (11 men, 2 women) with a median age of 37.38 years (13 to 53 years). Most of the tumors occur in the right side (in 8 cases) particularly in the right atrium (in 5 cases). Ten patients died, seven within one year of diagnosis. However, in our case, the patient is still alive and free of disease.

[Metastatic benign leiomyoma in a 46-year-old woman]. / Léiomyome bénin métastasiant.

Chest X-ray in a 46-year-old woman with a history of hysterectomy for a uterine mass revealed a nodular opacity measuring 3 cm. Computed tomography identified 3 other nodules. Histology examination of the lung lesions identified the same configuration as that of the hysterectomy specimen performed two years earlier: benign metastatic leiomyoma. Approximately 40 cases have been reported in the literature.

Melanin production in medullary thyroid carcinoma.

A case of melanin-producing medullary thyroid carcinoma is reported in a 51-year-old man. Histologically, the tumour had a typical pattern of medullary thyroid carcinoma with numerous scattered pigmented cells which contained large amounts of melanin pigment as confirmed by bleached Fontana-Masson stain. Immunohistochemical staining revealed positivity of almost all tumour cells for calcitonin and chromogranin, whereas S-100 protein and HMB-45 staining was positive only in the pigmented tumour cells. This finding confirms the ability of medullary thyroid carcinoma cells to have multidirectional differentiation, although melanocytic differentiation remains an exceptional phenomenon.

[Clinical and evolutive aspects of nasopharyngeal T4 N0 neoplasms]. / Aspects cliniques et évolutifs des T4 N0 du nasopharhnx.

Nasopharyngeal carcinoma (NPC) is the primary cancer of the head and neck localisations in the Salah Azaiz Institute (Tunisia). From 1970 to 1987, 80 patients with histologically proven T4 N0 NPC, were treated with exclusive radiation (70-75 Gy to the primary lesion and 50-55 Gy to cervical lymph nodes). The T4 N0 represents 7% of all NPC and 16% of the T4 treated in our Institute. Ninety percent of the patients are over 20 years old with a mean age of 52 years. The sex-ratio was 4:1. Extension to the brain was observed in 55% of the cases. Local control was 70% at 2 months after the end of irradiation. The actuarial survival at 5 years was 47%. Distant metastasis represent 13% (30% of all NPC). The main failure of treatment was local recurrence. The T4 N0 is probably a particular entity concerning the age, the response to radiotherapy and the low rate of distant metastasis.

Inflammatory symptoms in breast cancer. Correlations with growth rate, clinicopathologic variables, and evolution.

Based on two pretreatment evaluations, doubling time (DT) was calculated in 75 cases of invasive breast cancer (BC). The cases studied were more or less equally distributed between three DT groups: fast-growing tumors (DT less than 90 days), intermediate cases (DT between 90 and 180 days), and slow-growing tumors (DT greater than 180 days. A correlation was found to exist between DT and patient age and, to an even greater extent, between DT and pathologic prognostic indicators such as histologic grading and nuclear grade. Inflammatory symptoms were not associated with DT, but were closely related to the size of the tumor and regional lymph node involvement. The date of detection of distant metastases depended heavily on the DT of the BC:BC with shorter DT = earlier metastatic spread. The presence of inflammatory signs was also decisive: within each DT category, inflammatory BC metastases were both more frequent and precocious.

[Pseudotumoral pelvic actinomycosis in a woman who had been using an IUD for 15 years]. / Actinomycose pelvienne pseudo tumorale chez une femme porteuse d'un DIU depuis 15 ans.

PIP: A 56-year-old woman was referred to a hospital in Tunis for a tumor of the right ovary. The patient had had 11 term pregnancies and had been using an IUD with no medical supervision for 15 years. She complained of recent constipation and weight loss. The uterus was of normal size but of hard consistency and fixed to the sacrum. A cervical smear was normal. The patient was found to be anemic and the sedimentation rate was accelerated. A diagnosis of probably malignant tumor of the right ovary was made and a hysterectomy with bilateral ovarectomy was performed after laparotomy. The patient left the hospital after receiving a prescription for ampicillin and was lost to follow-up. Histological examination showed the mass to be comprised of inflammatory fibrous and granulomatous tissue with numerous abscesses of a filamentous, strongly eosinophilic character suggesting actinomycosis. Pelvic actinomycosis is a rare chronic infection caused by a gram positive anaerobic bacteria, Actinomyces Israeli. The clinical signs usually include anemia, weight loss, and pelvic mass. Clinical diagnosis is difficult, and diagnosis is usually based on histological and bacteriologic examination. Observation of the symptoms in association with IUD use suggests actinomycosis. The treatment of choice combines surgery with antibiotic therapy for 2 months. Cervical smears should be regularly scheduled for IUD users not only to screen for prenoplastic and neoplastic lesions but to indicate actinomycosis at an early stage when treatment will allow serious complications to be avoided.^ieng

High interferon titer and defective NK-cell activity in the circulation of nasopharyngeal carcinoma patients.

The interferon (IFN) activity of sera from 19 patients with nasopharyngeal carcinoma (NPC) was determined by the plaque-reduction assay with vesicular stomatitis virus (VSV) in HeLa cells and compared to that of sera from matched healthy controls. High titers of interferon were detected in the sera of the NPC patients with a geometric mean titer (GMT) of 43 +/- 25 U/ml. The interferon activity of the patients' sera was acid- and heat-labile (pH = 2 and 56 degrees C for 1 hr) and could be neutralized by a goat antiserum to human IFN-gamma. Interferon titers of the patients, in contrast, to normal controls, were not correlated with natural killer (NK) activity which was abnormally low in the NPC patients. On the other hand, a high percentage of circulating cells co-expressing the LGL marker (HNK-I) and the OKT8 antigen was detected in parallel with high IFN levels in NPC patients.

Immunoproliferative small intestinal disease without alpha-chain disease: a pathological study.

Biopsy specimens taken during exploratory laparotomy provided the material for a pathological study of 23 cases of nonsecreting immunoproliferative small intestinal disease (Mediterranean lymphomas without alpha-chain disease). The distinctive pathological feature of immunoproliferative small intestinal disease, i.e., a diffuse lymphoid infiltration, was present in the mucosa and submucosa of all or a major part of the small intestine. It was composed of a low- or intermediate-grade malignant lymphoid proliferation associated in 19 of 23 cases with benign-appearing follicular lymphoid structures. These follicular figures were surrounded and partially destroyed by the lymphoma cells. This association strongly resembles the newly described non-Hodgkin's lymphoma entities of perifollicular or parafollicular cell origin. Gross tumors of the small intestine were found in association with the diffuse lymphoid infiltration in 10 cases. They often constituted foci of lymphoma with a higher grade of malignancy. Mesenteric lymph node involvement was frequent and generally in direct ratio to the severity of intestinal involvement. A comprehensive study of the lesions observed in these cases led to the hypothesis that nonsecreting immunoproliferative small intestinal disease could result from the malignant change of perifollicular B cells; during an initial period the tumoral cells retain circulating and homing properties that explain their infiltrative and extensive method of spreading. The possible subsequent emergence of more aggressive subclones of noncirculating malignant cells could then explain the associated inconstant fungating tumor foci. Further studies using more sophisticated immunohistochemical techniques are necessary to establish the meaning of the hyperplastic lymphoid follicles, the possible etiologic role of benign nodular hyperplasia, the exact identification of the tumor cells, and the relationship of nonsecreting immunoproliferative small intestinal disease to closely related alpha-chain disease.

Prognostic value of EBV markers in the clinical management of nasopharyngeal carcinoma (NPC): a multicenter follow-up study.

Between December 1979 and April 1982, 373 patients with untreated, undifferentiated carcinoma of the nasopharynx (NPC), 99 in Hong Kong, 120 in Tunis and 154 in Villejuif, entered a longitudinal study aimed at determining the clinical prognostic value of EBV serology after radiotherapy. A minimum of 3 years' follow-up was achieved for 319 patients (83 in Tunis, 95 in Hong Kong and 141 in Villejuif) who had regular clinical and serological testing at intervals of 6-8 months. No significant difference in initial serology (i.e., before any treatment) or variations of antibody titers at time of first follow-up was observed between patients who achieved complete remission after radiotherapy and those who did not. This included IgG and IgA antibodies to VCA, EA or EBNA. However, when patients with confirmed clinical remission 1 year after completion of radiotherapy were studied, the value of IgG/EA and mainly of IgA/EA increasing titers became highly significant for prediction of relapse, regardless of the initial titers. This demonstrated the clinical usefulness of EBV serology for NPC patients who have confirmed clinical remission after radiotherapy.

Results of laparotomy in immunoproliferative small intestinal disease.

Immunoproliferative small intestinal diseases (IPSID), previously known as Mediterranean Lymphomas, constitute more than 60% of the non-Burkitt's small intestinal lymphomas in Tunisia. A multidisciplinary study of IPSID was undertaken by the Tunisian/French Lymphoma Study Group in 1980 to reach a better understanding of the two subgroups of the disease: secreting IPSID (essentially alpha-chain disease [ACD]); and nonsecreting IPSID (NS-IPSID) (extensive small intestinal lymphomas without gammopathy). The results of initial exploratory laparotomy performed in 38 cases of IPSID (17 ACD and 21 NS-IPSID) are described here, and show notable similarities between the two groups: the extensive pattern of the abnormal cell infiltrate along the major part of the small intestine; frequent and extensive involvement of the mesenteric lymph nodes; the existence of several degrees of severity in small intestinal mesenteric lymph nodes; and other intraabdominal organ involvement. Certain differences also were observed: the relatively high degree of tropism of the NS-IPSID towards the gastric mucosa which was absent in the ACD of this series; and the more frequent involvement of the entire length of the small bowel in ACD. In spite of these discrete differences, the IPSID form a homogeneous group which is definitely distinguishable from the segmentary small intestinal lymphomas known as Western-type, and which account for approximately one third of our non-Burkitt's small bowel lymphomas.