Limited Wegener's granulomatosis. Unusual cutaneous, radiographic, and pathologic manifestations.

A case of limited Wegener's granulomatosis is presented with unusual black linear and circular digital infarcts, unilateral bursitis and subcutaneous nodules, interstitial pneumonitis, and unusual pulmonary pathology. Response to cyclophosphamide was curative.

Synovial sarcoma: a clinical, pathological, and ultrastructural study of 26 cases supporting the recognition of a monophasic variant.

Twenty-six cases of synovial sarcoma (14 biphasic, 12 monophasic) were subjected to a clinicopathological study that included electron-microscopic examination of six tumors. Monophasic tumors were composed predominantly of uniform, densely packed, small spindle cells with scant cytoplasm identical to those of the "stromal" elements of typical biphasic tumors. The arrangement of these cells into narrow interlacing fascicles, forming tight whorls and showing little collagenization, was distinctive for this tumor. Major clinical differences between the two types of synovial sarcoma were the tendency for monophasic tumors to arise in distal extremity locations (seven of 10), and the poorer prognosis of monophasic tumors, 30% surviving 5 years compared to 58% for biphasic tumors. At the ultrastructural level, monophasic tumors and the spindle-cell components of biphasic tumors were identical. Both were composed of spindle or polygonal cells attached by numerous desmosomes. Prominent Golgi, abundant RER, perinuclear microfilaments, and glycogen aggregates were characteristic. Intercellular spaces containing elongated cytoplasmic filopodia were observed consistently, as were fragments of basement membrane-like material. The EM findings concur with those described previously in normal and pathologic synovium, and support a synovioblastic origin for the monophasic variant of synovial sarcoma.

Low-grade mucoepidermoid carcinoma of the breast.

Two cases of primary breast carcinoma morphologically identical to low-grade mucoepidermoid carcinoma of salivary gland origin occurred. To our knowledge, this article represents the first description of this tumor type in the breast. Both lesions were well circumscribed, wholly or partially cystic, and composed of intimately associated, well-differentiated, squamous and mucinous glandular epithelium. It is suggested that these may represent an indolent tumor type in the breast analogous to their behavior in the salivary gland.

Tubular carcinoma of the breast.

Retrospective pathologic review of 636 breast carcinomas from 611 patients revealed twelve tumors which were pure low grade tubular carcinoma (TC) and nineteen tumors with features combining both low grade tubular carcinoma and invasive duct carcinoma (T&D). A control group of 23 cases consisted of invasive duct carcinoma with at least a third of the tumor surface area showing tubular formation, but without the low grade features of tubular carcinoma. Life table analysis at 15 years showed a 100%, 72%, and 33% survival for TC, T&D, and controls, respectively. Eight percent of TC and 21% of T&D had axillary metastases compared to 67% for controls. Axillary metastases had no detrimental effect on TC or T&D survival. There were no recurrences in the TC group. Patients with T&D with tumor diameter 1.0 cm or less with 50% or greater low grade tubular carcinoma component are alive and well. The mean age of T&D was 7 years greater than TC. The combined TC and T&D group showed a significant incidence of multiple cancers in the ipsilateral breast and a significant trend toward bilateral cancers when compared to controls. Tubular carcinoma has an inherently low malignant potential with a histological and biological spectrum.