Unifocal bone paracoccidioidomycosis, Brazil.

We report a 47 year-old man from the State of Mato Grosso do Sul, Brazil, with unifocal tibial paracoccidioidomycosis. A radiograph showed an osteolytic lesion on the tibial middle third diaphysis. The diagnosis was confirmed by histopathologic analysis, and treatment with sulfamethoxazole and trimethoprim was started. After three months, the patient showed significant clinical improvement. Ten months after treatment, the patient showed lesion bone healing. This case describes the rare occurrence of an osteolytic lesion caused by endemic Paracoccidioides brasiliensis in Latin America.

Familial primary localized cutaneous amyloidosis in Brazil.

BACKGROUND: Macular and lichen amyloidosis are clinical variants of primary localized cutaneous amyloidosis (PLCA). Most cases are sporadic, but approximately 10% of cases may be familial. To our knowledge, the clinicopathologic and molecular features of such pedigrees, however, have not been studied in detail. OBSERVATIONS: We assessed 2 Brazilian families with either lichen-type (family 1 had 14 affected subjects) or macular-type (family 2 had 7 affected subjects) PLCA. Typically, in both pedigrees, the onset of symptoms was around puberty, and pruritus usually began on the lower legs. Findings from lesional skin biopsy samples from both families showed thioflavin T-positive material in the papillary dermis, which was more prominent in the lichen phenotype in family 1. Spontaneous improvement occurred in 3 subjects (from both families) after age 25 years. All affected individuals in family 1 had a heterozygous missense mutation in the OSMR gene (p.I691T), but no pathogenic mutation in OSMR was found in family 2. CONCLUSIONS: Familial PLCA shows autosomal dominant inheritance, but there is clinical and genetic heterogeneity and variable clinical penetrance. Demonstration of mutations in the OSMR gene provides new insight into mechanisms of itch and apoptosis in human skin.

Infiltrating syringomatous adenoma of the nipple.

Infiltrating syringomatous adenoma of the nipple is a rare, benign, locally invasive tumor with recurrence potential, showing sweat duct differentiation. It can clinically, radiologically and pathologically mimic cancer. Histopathologically, it must be distinguished from florid papillomatosis, adenosquamous carcinoma, adenoid cystic carcinoma and sclerosing syringomatous carcinoma. A 44-year-old woman presented with pain on the right nipple for 7 days. On physical exam there was an irregular nodule on nipple area with edema. The skin was intact. The ultrasound showed a hypoechoic irregular nodule measuring 7.5 mm in the nipple area. The mammography was unspecific. The lesion was surgically removed and histopathologically, the tumor was composed of ducts and tubules lined with a double-layered epithelial cells. The lining cells were small, cuboidal with a central nuclei and eosinophilic nuclei. The stroma was dense with lymphocytes and plasma cells, and compressed many of the ducts that contained a comma or tadpole-shape, giving an impression of a syringoma. Some ducts were slightly dilated with squamous metaplasia. Some of these cysts were connected with the overlying epidermis. Mitotic figures were rare and no pleomorfism or hyperchromasia was observed. At the periphery, the ducts invaded muscular fibers of the nipple. The surgical margins were free of neoplastic involvement. Patient has no signs of progression of disease in 1 year of follow-up.

Immunohistochemical panel of undifferentiated orbital metastatic carcinomas.

PURPOSE: To examine the applicability of an immunohistochemical panel of seven monoclonal antibodies to identify the primary site of poorly differentiated orbital metastatic carcinomas. MATERIAL AND METHODS: Immunohistochemistry was performed to detect cytokeratin (CK) 7, CK20, thyroid transcription factor-1 (TTF-1), BRST1, BRST2, carcinoembryonic antigen (CEA) and prostate-specific antigen (PSA) in seven cases of poorly differentiated orbital metastases. Of the seven cases, four were female and three male. The youngest patient was thirty-six while the oldest was eighty-eight years of age. RESULTS: The immunohistochemical panel alone was helpful to identify the primary source of the metastatic lesion in three out of the seven cases. Two of them were metastatic breast carcinomas (BRST1, BRST2 positive) and one was a prostate carcinoma (PSA positive). By correlating the immunohistochemical results with the previous clinical history, the primary site could be identified in two more cases. In those metastatic lesions, the positive staining for CK7, CK20, and CEA, associated with negative staining for BRST1, BRST2, PSA and TTF-1, indicated bladder as the probable primary site. In two out of seven cases, the metastatic tumor was only positive for CEA, therefore a primary site could not be identified. CONCLUSIONS: An immunohistochemical panel of poorly differentiated orbital metastases is helpful in the identification of the primary tumor site. The association of seven markers with the patient's clinical history allowed for the positive identification of the primary tumor in the majority of these cases.

Expression of EpCAM in uveal melanoma.

BACKGROUND: Uveal melanoma (UM) is the most common primary intraocular malignant tumor in adults, and nearly 40% of UM will develop metastasis that will ultimately lead to death. The Epithelial Cell Adhesion Molecule (EpCAM) is a type I transmembrane glycoprotein expressed by carcinomas of head and neck, ovary, colon, breast, kidney and lung. Recently, antibodies against EpCAM such as Edrecolomab and Catumaxomab were developed, and clinical trials with these antibodies have been used in several types of neoplasia. We studied the expression of EpCAM in UM. METHODS: 25 enucleated formalin-fixed, paraffin-embedded UM specimens were immunostained for EpCAM. Histopathological analysis of the specimens with regards to prognostic factors such as cell type, largest (linear) tumor dimension, number of mitotic figures, scleral invasion and tumor infiltrating lymphocytes were done. RESULTS: None of them was positive for this EpCAM. CONCLUSION: In our report, UM did not express EpCAM. Therefore, it is not a helpful immunohistochemical marker to predict the behavior of UM. Further studies are needed to verify if EpCAM could also be related with prognosis and treatment of UM.

Minimal deviation endometrioid adenocarcinoma of the cervix: report of three cases with exfoliative cytology.

Three histologically confirmed minimal deviation endometrioid adenocarcinomas (MDEA) of the uterine cervix with cytologic evaluation by cervical scraping were reviewed. The smears were cellular and showed tall columnar tumor cells arranged in monolayered sheets with nuclei in palisade at free borders, rosettes, and irregular clusters. Cellular strips with palisading nuclei was noted in one case. The individual tumor cells showed monomorphic, round or oval, hyperchromatic nuclei with chromatin clumping, small nucleoli, and granular, nonvacuolated cytoplasm with cytoplasmic extensions or tails. The smear background showed a variable amount of necrotic debris admixed with polymorphonuclear leukocytes in two cases. The cytologic manifestations of those three cervical MDEAs overlapped, to some extents, with those of a cervical adenocarcinoma in situ and with those of a well-differentiated endometrial adenocarcinoma invading the cervix.

Mammary myofibroblastoma: report of two cases with fine-needle aspiration cytology and review of the cytology literature.

Fine-needle aspirates from two histologically, immunohistochemically, and ultrastructurally confirmed mammary myofibroblastomas (MM) of two elderly women revealed abundant, randomly arranged single and clustered benign spindle-shaped mesenchymal cells with scant cytoplasm and elongated or oval nuclei displaying a finely granular chromatin pattern and inconspicuous nucleoli. In one case a few cells showed inconspicuous nuclear grooves. The aspirated tumor cells from the other case stained positively with desmin and CD34 antibodies and negatively with cytokeratin and S-100 protein antibodies, in keeping with an MM. A review of the literature was briefly presented.

Consideraçöes a respeito da cistite eosinofílica / Eosinophilic cystitis

Eosinophilic cystitis is a rare bladder disorder that was first reported in 1960 and can be diagnosed in children or adults. Its cause is unknown and usually presents with hematuria, supra pubic pain, sysuria and frequency. We have made an extensive literature review and we reported on 2 illustrative cases. The first case was a man presented with hematuria; a computed tomography, shown expansive process with a mass suggesting infiltration of the perivesical tissues. A biopsy was done and the pathological diagnosis was eosinophilic cystitis. The patient was treated with steroids. Later on, he had an episode of hematuria on the left ureteral orifice and a nephrouretectomy showed grade 2 urothelial carcinoma of the ureter. The second case is a child with neurogenic bladder due to myelomeningocele who had a cystostomy since the age of 5 months. Surgery was performed, including partial cystectomy with an ileal neobladder. The anatomopathological diagnosis was eosinophilic cystitis. The different classifications of eosinophilic cystitis were presented and discussed. The diagnosis of this entity can only be made by microscopic examination which shows mucosal edema or fibrosis, a polymorphous infiltrate rich in eosinophils and sometimes replacement of the bladder muscle by fibrotic tissue