Postmortem findings in congenital generalized lipodystrophy.
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Biomedical subjects
Publications and source records attributed to F Vuitch.
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To evaluate the contribution of sarcoidosis to death, we reviewed 38 autopsy cases. The diagnosis of sarcoidosis was established or suspected antemortem in 17 (45%) of 38 cases. Sarcoid was fatal in 67% and incidental in 33%. Of 28 fatal cases, 14 (50%) resulted from cardiac involvement and 12 (43%) from pulmonary disease. Antemortem diagnosis was made in 4 (29%) of 14 fatal cardiac cases versus 9 (75%) of 12 fatal pulmonary cases. This study reveals that antemortem diagnosis of sarcoidosis is challenging and that heart and lung involvement are the most significant contributors to fatal outcome. Cardiac sarcoidosis is frequently first diagnosed postmortem and is a more common cause of death than previously reported. Unlike previous studies, we found that fatal cardiac sarcoidosis is commonly associated with significant extracardiac disease, implying that antemortem diagnosis may be suspected without endomyocardial biopsy in most patients.
BACKGROUND: Papillary (PC) and follicular (FC) thyroid carcinomas may have a focal or predominant insular component (IC). This study correlates histologic behavior with the extent of the IC of these tumors. METHODS: Forty-one thyroid carcinomas (17 follicular variants [FV] of PC, 24 FC) with focal or predominant IC were reviewed. The tumors were stained with carcinoembryonic antigen, thyroglobulin, and calcitonin. DNA ploidy analysis was done on 18 tumors on paraffin-embedded tissue. The IC was correlated with age, stage of disease, follow-up, and ploidy analysis by Fisher's exact two-tailed test. RESULTS: No tumor was purely insular. IC was minor (10-40% tumor area) in 16 and predominant (50-90%) in 25. Nuclear features in IC included typical FC nuclei in 14, FVPC nuclei in 16, and intermediate-type nuclei in 16 tumors. Vascular invasion was seen in 22, necrosis in 10, and sclerotic stroma in all. Fourteen tumors were confined to the thyroid, 13 showed regional spread, and 9 had distant metastases; five patients are dead of disease and six are alive with disease. Thirteen tumors were diploid, three tetraploid, and two hyperdiploid aneuploid. There was no correlation of quantity of IC with tumor stage, follow-up status, or ploidy. CONCLUSION: IC within PC and FC does not adversely affect prognosis.
The authors report two examples of the tubular variant of adenoid cystic carcinoma of the Bartholin's gland. Each occurred in young women (both aged 34 years) and presented with a painful indurated mass, clinically thought to be of inflammatory nature. On microscopic examination, the most distinctive feature was the haphazard proliferation of bland-appearing tubular structures often lined by two cell layers. A thin, periodic acid-Schiff-positive basement membrane immunoreactive for collagen IV surrounded the tubules, but hyaline globules were virtually absent. The inner cell layer was strongly and diffusely cytokeratin positive, whereas epithelial membrane antigen reactivity was seen only along the luminal borders. Focal positivity for S100 protein identified the peripheral myoepithelial cells, which were confirmed by electron microscopy. Focal perineural invasion was seen. The histologic, ultrastructural, and immunohistochemical features of these tumors are similar to those of the tubular variant of adenoid cystic carcinoma arising in major and minor salivary glands. In one of the cases, which was followed for 6 years, the tumor metastasized, despite the low histologic grade, and the patient died. Similarities and differences between the tubular, cribriform, and solid variants and other vulvar tumors are discussed.
The authors report a case of metastatic prostate cancer masquerading as a primary lung tumor. Histologically, the lung tumor displayed eosinophilic crystalloids in the malignant glands typical of those previously described in prostatic adenocarcinoma. Review of histologic material from 30 additional patients with metastatic prostate cancer failed to reveal crystalloids in the metastases. Seven patients with histologic material from locally advanced prostatic adenocarcinoma, defined as spread to the rectum, bladder, or nonnodal pelvic soft tissue, were also reviewed. One of these patients demonstrated crystalloids in the bladder extension of locally advanced prostatic adenocarcinoma. Although rare, the presence of crystalloids may be used as strong evidence for the prostatic origin of an adenocarcinoma of uncertain origin.
We present the fifth case of rhabdomyomatous mesenchymal hamartoma, the first reported in a female infant. The patient is an otherwise healthy 3-day-old Latin American infant, the product of an uneventful pregnancy and term delivery by a primigravida 23-year-old. Otolaryngic consultation was obtained when a 4-mm polypoid mass was noted within the left naris. Histopathology revealed the characteristic central core of mature skeletal muscle with extension peripherally into the glandular and follicular dermal structures. Although primarily of cosmetic significance, a possible association with other anomalies has been reported.
We describe 11 patients with a distinctive salivary gland neoplasm. Most of the patients were adult women who presented with a painless mass. Nine tumors arose in minor salivary glands of the oral cavity (82%). Microscopically, they were characterized by the formation of trabeculae, cords, islands, and/or nests of monomorphic clear cells that were glycogen rich and mucin negative and were surrounded by hyalinized bands with foci of myxohyaline stroma. Cells with eosinophilic and granular cytoplasm were also noted. Both cell types showed minimal nuclear pleomorphism and a very low mitotic index. The neoplasms all had infiltrative borders. Immunohistochemically, the tumor cells expressed cytokeratins and epithelial membrane antigen, but not S-100 protein and smooth muscle actin. Ultrastructurally, the tumor cells contained abundant glycogen, desmosomes, peripheral tonofilaments, and prominent interdigitating microvilli without actin myofilaments or dense bodies. These immunohistochemical and ultrastructural findings provide evidence of epithelial differentiation without myoepithelial differentiation. For these tumors, we propose the name, hyalinizing clear cell carcinoma (HCCC). These are low-grade malignant neoplasms. Two patients had ipsilateral cervical lymph node metastases at presentation, but with surgical excision and either preoperative or post-operative radiotherapy in three cases, eight of 10 patients with clinical follow-up are alive and well without evidence of recurrence. The mean clinical follow-up is 3.6 years, with a range of 6 months to 11 years. One patient died as a result of surgery, another died of unrelated causes, and one patient was lost to follow-up.
Six men who had undergone hip replacements for degenerative joint disease or trauma subsequently had radical prostatectomies or cystoprostatectomies with bilateral pelvic lymph node dissections for adenocarcinoma of the prostate or transitional cell carcinoma of the urinary bladder. The hip prostheses implanted in three patients were known to contain cobalt-chromium alloy and titanium. The pelvic lymph nodes ipsilateral to the hip prosthesis in five patients and the bilateral pelvic nodes in the only patient with bilateral hip prosthesis had dark brown or black cut surfaces. These lymph nodes did not contain carcinoma but showed florid sinus histiocytosis characterized by large polygonal histiocytes filling and expanding sinuses and interfollicular regions. The foamy histiocytes contained cobalt-chromium and titanium microparticles by light microscopy, ultrastructure, and energy-dispersive x-ray microanalysis. The lymph nodes uninvolved by the histiocytic reaction lacked the heavy metal microparticles. Four cases were found to have a small number of polyethylene particles, which might have contributed to the histiocytic response. By immunohistochemistry, the foamy cells displayed immunoreactivity for lysozyme, alpha-1-antitrypsin, alpha-1-antichymotrypsin, and cathepsin D, providing additional support for their histiocytic derivation. To our knowledge, this is the first time that microparticles of cobalt-chromium and titanium that migrate from hip prostheses to pelvic lymph nodes have been shown to elicit a distinctive type of florid sinus histiocytosis. Pathologists should be aware of this characteristic foreign-body tissue response to avoid confusion with other types of sinus histiocytosis or with metastatic carcinoma.
Angiocentric T cell lymphoma may present with cutaneous inflammatory lesions masquerading as cutaneous vasculitis both clinically and pathologically. We describe a case of angiocentric T cell lymphoma. Although initial biopsies in this patient resembled polyarteritis nodosa, subsequent biopsies exhibited more characteristic changes and immunohistochemical stains confirmed the diagnosis of large cell angiocentric cutaneous T cell lymphoma. It is important to differentiate these diseases as treatment and prognostic implications are very different.
BACKGROUND: Salivary duct carcinoma (SDC) is a distinctive salivary gland neoplasm morphologically characterized by intraductal and infiltrating components. Most tumors occur in the major salivary glands and demonstrate a propensity for invasive growth with early regional and distant metastases. Therefore, SDC is regarded as a high-grade malignancy in the current classification of salivary gland neoplasms. METHODS: In an effort to identify clinically relevant prognostic features, we studied the clinicopathologic and immunohistochemical findings in 15 SDC, with ultrastructural evaluation of three tumors. RESULTS: Thirteen SDC occurred in the parotid gland, one in the Stensens duct, and one in the palate. Twelve patients were men (ratio of men to women = 4:1). Patients ranged in age from 39 to 81 years (mean = 59 years). Tumor size varied from 1.2 to 6.5 cm (mean = 3.1 cm). An intraductal component was identified in 10 of 14 primary SDC that made up 10% to 95% of the tumor. In three SDC a preexisting pleomorphic adenoma was identified. Immunohistochemical and electron microscopic evaluation showed the SDC to be composed entirely of ductal cells, and one tumor exhibited features of striated duct differentiation. CONCLUSIONS: SDC show a broader clinicopathologic spectrum than previously described. The tumor may arise in a pleomorphic adenoma. The proportion of intraductal and extraductal growth is variable and of prognostic significance. Although the majority of SDC behave in a high-grade fashion, those with a predominant intraductal component of greater than 90% (PID-SDC) and minimally invasive (< 8 mm) SDC in pleomorphic adenoma appear to have a more favorable prognosis.
Fine-needle aspiration has assumed a large role in the evaluation of head and neck masses. It has been found to be simple, quick, accurate, and cost-effective in the workup of patients with unknown head and neck masses. This article reviews these advantages and the role of fine-needle aspiration in the evaluation of head and neck masses.
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A histopathologic analysis of regional lymph node specimens in 48 patients with previously untreated squamous cell carcinoma of the larynx was performed to determine which parameters, if any, correlated with 3-year patient survival. The presence of sinus histiocytosis in lymph nodes containing metastatic squamous cell carcinoma was statistically related to survival (P < .007). Multivariate analysis confirmed the independent significance of this variable compared to tumor stage, nodal status, and extracapsular spread from lymph nodes. Previous studies have linked improved survival with an active immunologic response in head and neck cancer patients. However, few studies have examined a homogeneous group of patients who all underwent the same type of treatment. This study's findings support the concept that an active immunologic response in lymph nodes has prognostic significance in squamous cell carcinoma of the larynx.
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We report a previously undescribed virilizing malignant adrenocortical tumor in an 21-month-old infant with elevated serum alphafetoprotein. The tumor consists of a peculiar mixture of immature epithelial and mesenchymal elements as well as slit-like spaces partially lined by primitive epithelial cells. Focally, the tumor has features reminiscent of the normal embryologic development of the adrenal cortex. A panel of immunohistochemical stains revealed only vimentin reactivity. We propose the term "adrenocortical blastoma" for this unusual neoplasm.
The magnetic resonance imaging (MRI) features of two cases of malignant lymphoproliferative disease involving skeletal muscle are presented. In both cases involved muscles were quantitatively and subjectively hypointense to fat on T1-weighted spin echo images, hypointense or isointense on T2-weighted spin echo images, and hyperintense on short tau inversion recovery (STIR) images. The findings suggest that lymphoproliferative disease should be considered as an etiology of a skeletal muscle lesion that is hypointense or isointense to fat on T2-weighted spin echo magnetic resonance images.
During a 2-year period 5 men positive for the human immunodeficiency virus (HIV) presented with 6 testis tumors among a total of 3,015 men seen at our hospital acquired immunodeficiency syndrome (AIDS) clinic. This testis tumor incidence of 0.2% is 57 times that of the United States average of 3.5 cases per 100,000 men. Two patients were only HIV positive and 3 others already had AIDS-related complex for 2 to 15 months at the time of tumor diagnosis. Tumor histology was mixed germ cell tumor in 4 patients, pure seminoma in 1 and Burkitt's lymphoma in 1. Patients underwent routine staging evaluations. Three patients had low stage mixed germ cell tumor (clinical stage 1 or 2A) and underwent retroperitoneal lymphadenectomy, which revealed pathological stage 1 or 2A disease in 1 and 2, respectively. These patients did not receive adjuvant chemotherapy. Two patients had advanced mixed germ cell tumor (clinical stage 2C) or Burkitt's lymphoma (clinical stage 4) and received combination chemotherapy from the onset. Outcome was evaluated with regard to progression of HIV disease and tumor status. The 2 patients who were only HIV positive remained so for 9 and 48 months. The 3 patients with AIDS-related complex had progression to AIDS within 1 to 9 months and 2 of these patients died 1 1/2 and 7 months after tumor diagnosis. All 3 patients with resected low stage disease had tumor recurrence within 1 to 9 months and were begun on platinum-based combination chemotherapy. The risk of false low clinical staging and early tumor progression may be higher in HIV positive men than in other testis tumor patients. Patient ability to tolerate chemotherapy and to obtain a satisfactory tumor response appeared to be primarily related to lack of progression of HIV disease to frank AIDS.
Monoclonal 3F6 anti-Pneumocystis carinii antibody (MAB-3F6) was used to stain cell blocks from 164 bronchial lavage specimens from patients with the acquired immune deficiency syndrome (AIDS) and AIDS-related complex and compared with slides stained with Grocott's modification of the Gomori methenamine silver stain. Pneumocystis organisms were present in 83 of 164 cases using MAB-3F6 stain, whereas Grocott's modified silver stain demonstrated Pneumocystis organisms in 48. MAB-3F6 demonstrated Pneumocystis organisms in 38 cases with negative silver stains, whereas silver stain identified Pneumocystis organisms in only three MAB-3F6-negative cases. Of 70 patients with clinical Pneumocystis pneumonia at the time of the specimen was obtained, 59 had MAB-3F6-positive specimens, whereas 39 had organisms detected using Grocott's modified silver stain. Of 37 patients without clinically apparent Pneumocystis pneumonia any time in their course, 4 had abundant organisms and 33 had negative stains with MAB-3F6. MAB-3F6 detected Pneumocystis organisms in 22 of 31 cases of Pneumocystis pneumonia that had no organisms identified using Grocott's silver stain (X2 = 5.76, P = 0.016). MAB-3F6 immunochemical staining is a more sensitive method than Grocott's modified silver stain to detect Pneumocystis organisms.