Tumoral amyloidosis (amyloidoma) of bone is a rare condition characterized by the massive destructive deposition of AL amyloid in bones. We report three cases. The patients ranged in age from 45 to 78 years and had tumors located in the lumbar spine, scapula, and humeral head measuring 6.5 to 18 cm. The radiologic diagnosis was chondrosarcoma in two cases. Microscopically, there were large, rounded deposits of amorphous eosinophilic material surrounded by numerous giant cells and a sparse lymphoplasmacytic infiltrate. The deposits proved to be composed of AL amyloid showing potassium permanganate resistant congophilia. Immunohistochemistry showed immunoglobulin IgG lambda, IgG kappa, and IgM lambda monoclonality of the plasma cell and (in one case) lymphoid infiltrate. The tumors were classified by morphology and immunohistochemistry as solitary plasmacytomas of bone (two cases) and plasmacytoid lymphoma (one case). During the relatively short follow-up period, one patient progressed to symptomatic generalized amyloidosis and died, one patient died of recurrent tumor, and one patient is alive with no evidence of disease. An extensive review of the world literature showed 34 well-documented similar cases, occurring most often in the spine and skull, causing neurologic symptoms, tending to occur in middle-aged men and frequently progressing to generalized disease. Most if not all AL amyloidomas of bone represent solitary plasmacytomas of bone or plasmacytoid lymphomas.
BACKGROUND Basosquamous carcinoma is a rare malignancy, with features of both basal cell carcinoma and squamous cell carcinoma. Some authors believe that basosquamous carcinoma merely is a variant of basal cell carcinoma, whereas others have suggested that basosquamous carcinoma may behave more aggressively. To the authors' knowledge the largest published series to date, comprised of 35 cases, was reported > 20 years ago. The authors reviewed their recent experience with basosquamous carcinoma to identify prognostic factors influencing recurrence. METHODS The medical records of all patients with the diagnosis of basosquamous carcinoma treated at the University of Louisville‐affiliated hospitals between 1985–1988 were reviewed by a senior pathologist. Prognostic factors were analyzed using Cox regression analysis and the log rank test. RESULTS Thirty‐one cases of basosquamous carcinoma were identified in 28 patients. The median age at diagnosis was 68 years (range, 10–94 years). The median follow‐up was 60 months (range, 12–312 months). Seventy‐five percent of cases were located on the face, neck, and scalp. One patient had regional lymph node metastasis synchronous with the primary tumor. Patterns of recurrence were: local recurrence only (five patients), local recurrence plus regional lymph nodes (three patients), and pulmonary plus regional lymph nodes (one patient). One patient died of pulmonary metastasis. Significant factors predictive of recurrence (P < 0.01) were male gender, positive surgical resection margin, lymphatic invasion, and perineural invasion. Although tumor size was not a statistically significant factor overall (P = 0.076), the 3 patients with lymph node metastases had large tumors (measuring 2 cm, 5 cm, and 5 cm, respectively). CONCLUSIONS Basosquamous carcinoma is an aggressive epithelial neoplasm with a propensity for local recurrence and potential for distant metastatic spread. This behavior differs substantially from basal cell carcinoma. Complete resection with negative surgical margins is essential. Long term follow‐up for the detection of local recurrence and distant metastatic spread is recommended. Cancer 2000;88:1365–9. © 2000 American Cancer Society.
One malignancy arose in a background of enchondromatosis, with all the others being solitary lesions. A pathologic diagnosis of malignancy is often difficult in the absence of radiologic signs of malignancy (cortical destruction with or without soft tissue extension). However, three cases were unusual in that the initial radiograph demonstrated a benign appearance. Another group of three malignancies was surprisingly indolent biologically. The treatment of choice is ray resection (or more limited amputation in a lesion of the middle or distal phalanx).
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