A case of sinus histiocytosis with massive lymphadenopathy (SHML) in which cranial nerve function was impaired is described. Severe paraparesis due to an epidural block at the C, and C,-T, levels was also present. Treatment with cytotoxic agents resulted in a dramatic decrease in the size of the involved lymph nodes and disappearance of neurological symptomatology. A review of the literature revealed that, although the disease is benign in its clinical course, the process is not restricted to the lymph nodes. The case reported here is the second case in which the epidural space was affected. Cancer 42:77-80, 1978.

Breast cancer is the most frequent malignant tumor in women, whereas it is rare in men. In our own case series the ratio is 175:1. The present paper deals with an evaluation of clinical and morphological findings from a series of 54 de novo male breast cancers observed in our institution from 1978 to 1996 and a comparative discussion of 528 female breast cancers from the same geographic area. We should like to focus on the following observations: At the time of histopathological diagnosis, male patients with breast cancer were on average 67 (34-87) years old and thus 5 years older than women. Below the age of 40, breast cancer is very rare in men. The lag time between first symptoms and surgery was on average 42 weeks, i.e. twice as long as in women. In the vast majority of cases palpation of a retromamillary nodule was the leading diagnostic symptom. Mamillary secretion appeared to be an early symptom with favorable relation to prognosis by tumor size whereas diffuse breast swelling was an unfavorable late symptom. Bilateral carcinoma and double cancer (breast and prostatic cancer) was observed in one case each. Three patients (3/51 = 6%) had a positive family history (breast cancer in 1st and 2nd degree relatives). The average invasive tumor size was nearly identical with 23 mm (s11.02) in men and 25 mm (s13.48) in women. Men presented more frequently with regional lymph node metastases (53% versus 45%), which tended to develop earlier. pT4 cancers were twice as frequent in men compared to women. In situ cancers were found in 2% (1/54) in men and 4% in women. Similar to females, male breast cancers are predominantly of ductal histological type (NOS-cancers), classical lobular carcinoma with LCIS-components were not observed; special forms (tubular, papillary, mucinous) are slightly more common in men. When reviewing our series, need for revision of the origin of tumor was not found in any of the cases. Metastases of prostatic cancer were never misinterpreted as primary breast cancer. In case of isolated NSE-reaction, cancers with carinoid differentiation pattern are to be found in nearly every second tumor. However, when multiple markers were used (chromogranin A or synaptophysin) only 10% displayed such pattern, which corresponded to a positive hormone receptor status in each case. Quantitative (enzyme immunoassay) and semiquantitative (immunohistochemistry) analysis of steroid hormone receptor status was positive in 86% of 35 cases in men and in 75% in women. In contrast to female breast cancer, hormone status proved to be independent of age in males. The average levels of estrogen and progesterone were higher in men. Overlapping results were found only when cases were compared with postmenopausal women. The Nottingham prognostic index, a product of primary tumor size, axillary lymph node status and grading allows an approximative estimate of the course of the disease; its predictive value is higher than that of isolated tumor markers.

A 77-year-old man with chronic obstructive pulmonary disease was treated with low-dose methotrexate (7.5-15 mg per week). After 15 months a diagnosis of urothelial carcinoma of the bladder was made; after a further 6 months pneumonitis and pancytopenia developed. The patient died due to massive pulmonary hemorrhage. A malignant teratoma was diagnosed in a 65-year-old asthmatic man 16 months after initiation of methotrexate therapy (15 mg per week). The patient died 4 months later due to fulminant progression of the neoplasm. A third malignant neoplasm (dermal squamous cell carcinoma) was seen in a 64-year-old woman with rheumatoid arthritis after 13 months treatment with 7.5 mg methotrexate per week. These three cases, while obviously not proving a causal relationship between long-term treatment with low-dose methotrexate and development of malignant neoplasm, do call for stringent treatment criteria, close surveillance, and prospective studies.

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Molecular genetic analysis indicates that the BRCA2 gene plays an important role in familial male breast cancer. To determine a possible involvement of this tumor suppressor gene in sporadic male breast cancer, we examined 30 sporadic male breast carcinomas for loss of heterozygosity (LOH) at two loci on chromosome 13q12-13, a region that spans the BRCA2 locus. Sixteen of 24 (67%) informative cases showed LOH in at least one marker on chromosome 13q12-13. The affected cases included both invasive ductal carcinomas and other types of invasive breast carcinoma and were detected preferentially in patients who were 50 years or older, in patients with lymph node metastasis, and in progesterone receptor-negative cases. We report, for the first time, a high frequency of LOH at chromosome 13q12-13 in sporadic male breast cancer and its association with factors indicating a poor prognosis for this tumor (e.g., lymph node metastasis and negative progesterone receptor status). These findings suggest an important role for BRCA2 in the development and progression of sporadic male breast cancer.

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