Primary pure squamous cell carcinoma of the breast diagnosed by fine-needle aspiration cytology: a case study using liquid-based cytology.

Pure primary squamous cell carcinoma (SCC) is an extremely rare type of breast tumor. We report one of such cases in a 32-year-old woman, diagnosed by fine-needle aspiration cytology (FNAC). Aspiration smears were characterized by squamous cells, both isolated and in aggregates, at various stages of maturation. The tumor was excised, and the histologic sections confirmed the cytologic diagnosis. Pure primary SCC of the breast has a distinctive cytomorphologic appearance, and diagnosis of this tumor by FNAC is possible. For its diagnosis, the exclusion of SCC of local cutaneous structures and metastasis of distant squamous carcinoma are mandatory.

[Leiomyosarcoma of the urinary bladder]. / Leiomiosarcoma de vejiga urinaria.

We report a case of bladder leiomyosarcoma treated at our department. We review the published papers about this entity. We emphasize the low incidence of that pathology and the lack of consensus about treatment.

Combined use of novel epithelial (MOC-31) and mesothelial (HBME-1) immunohistochemical markers for optimal first line diagnostic distinction between mesothelioma and metastatic carcinoma in pleura.

AIMS: To determine the value of immunohistochemistry in differentiation of malignant pleural mesothelioma from carcinoma in a pleural biopsy we optimized a double panel of MOC-31 and HBME-1 and compared the results with others from the literature. METHODS AND RESULTS: A multi-antibody panel was applied to biopsy samples from 44 cases of malignant pleural mesothelioma and 23 cases of carcinoma metastatic to the pleura. We used monoclonal antibodies against keratins, epithelial membrane antigen (EMA), epithelial antigen Ber-EP4, carcinoembryonic antigen (CEA), tumour-associated glycoprotein (B72.3), LeuM1, vimentin, desmin, epithelial related antigen (MOC-31) and mesothelial cell (HBME-1). Positivity for MOC-31 and Ber-EP4 was found to have the highest nosologic sensitivity (94.1% and 84.6%, respectively) and specificity (86.3% both antibodies) for carcinoma. Positive staining for HBME-1 and vimentin had the highest sensitivity (90.9% and 100%, respectively) and specificity (91.3% and 60%, respectively) for mesothelioma. A two-marker antibody panel with HBME-1 and MOC-31 was the most efficient for the distinction between carcinoma and malignant pleural mesothelioma. CONCLUSION: A combination of MOC-31 (an anti- epithelial marker) and HBME-1 (an anti-mesothelial marker) has a diagnostic efficiency of 76.1% for the distinction between carcinoma and mesothelioma in pleura.

Leiomiosarcoma de vejiga urinaria / Leimyosarcoma of bladder urinary

Se presenta un caso de leiomiosarcoma vesical tratado en nuestro Servicio. Se revisa la literatura sobre el tema, remarcando su baja incidencia y la falta de criterio unánime desde el punto de vista terapéutico (AU)

[Intravesical hemangiopericytoma]. / Hemangiopericitoma intravesical.

OBJECTIVE: To report on a case of hemangiopericytoma of the urinary bladder mimicking a urothelial tumor. METHODS: A case of hemangiopericytoma arising from the urinary bladder in a 78-year-old man is described. The initial symptoms included gross hematuria, and it was confused with a urothelial tumor. The difficulty in making the diagnosis in regard to the site of origin and histological findings are discussed. The literature is briefly reviewed. RESULTS/CONCLUSIONS: Ultrasound and urographic evaluation showed an intravesical mass which was suspected to be a urothelial tumor. The patient was submitted to transurethral surgery. Histological analysis and immunohistochemical techniques demonstrated a hemangiopericytoma.

Myofibroblastoma of the breast with diverse histological features.

We report two cases of myofibroblastoma with unusual pathological features, in a 66-year-old woman and a 49-year-old man. Both tumours were unilateral, grossly nodular and well circumscribed, but not encapsulated. The lesions were made up of bipolar spindle cells arranged in fascicular clusters separated by bands of hialinized collagen; one included several islands of mature cartilage next to fat cells. The other contained atypical mononucleated and multinucleated giant cells. No mitotic figures were observed. Immunohistochemically, both tumours showed strong and diffuse cytoplasmic staining for vimentin and CD 34 and focal positivity for alpha-smooth muscle actin, and both were negative for cytokeratins, CD 68, Ham 5, 6, Mac 387, and S-100 protein. Desmin was positive in one case. Ultrastructural study revealed populations composed of fibroblastic cells without signs of myofibroblastic differentiation in one case; the second featured abundant undifferentiated mesenchymal cells with myofibroblastic differentiation. Both patients remain disease-free 38 and 36 months after lumpectomy.

A comparative evaluation of immunohistochemical markers for the differential diagnosis of malignant pleural tumours.

AIMS: To determine the value of immunocytochemistry in differentiation of malignant pleural mesothelioma from carcinoma in a pleural biopsy using commercially available monoclonal antibodies. METHODS AND RESULTS: A panel of monoclonal antibodies against keratins, epithelial membrane antigen (EMA), epithelial antigen Ber-EP4, carcinoembryonic antigen (CEA), tumour-associated glycoprotein (B72.3), Leu-M1, CD30 (Ber-H2), vimentin and desmin, was applied to 40 cases of malignant pleural mesothelioma and 23 cases of carcinoma metastatic to the pleura (16 pulmonary and seven extrapulmonary). Positivities for Ber-EP4, CEA, B72.3 and Leu-M1 were found to have the highest nosologic sensitivities (87.0%, 65.2%, 52.5% and 43.5%, respectively) and specificities (97.5%, 97.5%, 100% and 95%, respectively) for carcinoma. Positive staining for vimentin had the highest sensitivity (87.5%) with 95.7% specificity for mesothelioma. Positive staining for desmin was found in 45% of mesotheliomas and 0% of carcinomas. Diagnostic sensitivity and diagnostic specificity (P-values) were calculated for these markers. In respect to the diagnostic power defined by the clinically relevant predictive values of positive and negative tests, we found that a two-marker panel of antibodies including vimentin and Ber-EP4 is most useful for the histopathological distinction between carcinoma (pulmonary or extrapulmonary) and malignant pleural mesothelioma. CONCLUSIONS: A combination of Ber-EP4 and vimentin provides the most sensitive and specific pair of markers for distinguishing between malignant pleural mesothelioma and carcinoma metastatic to the pleura. The prevalence of the tested tumours should be taken into account when evaluating the clinical value of ancillary techniques in pathology.

Bone marrow-derived dendritic cells serve as potent adjuvants for peptide-based antitumor vaccines.

Dendritic cells (DCs) are considered the most effective antigen-presenting cells (APCs) for primary immune responses. Since presentation of antigens to the immune system by appropriate professional APCs is critical to elicit a strong immune reaction and DCs seem to be quantitatively and functionally defective in the tumor host, DCs hold great promise to improve cancer vaccines. Even though they are found in lymphoid organs, skin and mucosa, the difficulty of generating large numbers of DCs has been a major limitation for their use in vaccine studies. A simple method for obtaining DCs from mouse bone marrow cells cultured in the presence of GM-CSF + interleukin 4 is now available. In four different tumor models, mice injected with DCs grown in GM-CSF plus interleukin 4 and prepulsed with a cytotoxic T lymphocyte-recognized tumor peptide epitope developed a specific cytotoxic T lymphocyte response and were protected against a subsequent tumor challenge with tumor cells expressing the relevant tumor antigen. Moreover, treatment of day 5-14 tumors with peptide-pulsed DCs resulted in sustained tumor regression in five different tumor models. These results suggest that presentation of tumor antigens to the immune system by professional APCs is a promising method to circumvent tumor-mediated immunosuppression and is the basis for ongoing clinical trials of cancer immunotherapy with tumor peptide-pulsed DCs.

Leiomyosarcoma of the soft tissues in a patient with nevoid basal-cell carcinoma syndrome.

A 57-year-old man was admitted with complaints of progressive anorexia, weight loss and right flank pain. He had been treated for basal-cell carcinoma of the skin 19 years before. On physical examination, eight moles in the face, back and left thigh were found along with palmar pits. In addition, a painful induration in his right thigh was evident. Biopsy proved that six moles were basal-cell carcinomas and the thigh mass a high-grade leiomyosarcoma. Computed tomographs revealed multiple metastases in the lungs and the liver. The patient was treated with epirubicin, with partial response, and subsequently with ifosfamide. He died 17 months after diagnosis. Whereas the world literature records several cases of soft tissue tumors in patients with nevoid basal-cell carcinoma syndrome, this is the first report of a simultaneous occurrence of leiomyosarcoma and nevoid basal-cell carcinoma syndrome.

Amputation neuroma of the hepatic hilum after orthotopic liver transplantation.

Amputation neuromas following biliary surgery have been previously reported. There are no descriptions, however, of amputation neuroma following liver transplantation. Serial hilum sections taken from 93 hepatectomy specimens obtained during the clinical course of 262 consecutive orthotopic liver transplantations revealed 26 amputation neuromas (27.9% of the specimens examined). The finding was confirmed by immunohistochemistry with numerous S-100 protein positive cells intermingled with neurofilaments interrupting the perineurial layer of cells testing positive for epithelial membrane antigen. Neuromas were found in liver specimens obtained between 89 and 775 days post-transplant (mean time, 211 days). The incidence of neuroma was higher in specimens resected more than 3 months post-transplant. There was only one symptomatic patient, who died from extrahepatic cholestasis demonstrated at autopsy to be caused by a hilar neuroma obstructing the main bile duct.

Ovarian and extragonadal malignant germ-cell tumors in females: a single-institution experience with 43 patients.

From 1978 to 1992, 276 patients (pts) with MGCT were treated in our institution. Forty-three of the pts were female (15.5%). Median age at diagnosis was 20 years (newborn-70). Histology was dysgerminoma (D) in 14 pts (including 2 anaplastic D), endodermal sinus tumor (EST) in 9 pts, immature teratoma in 10 pts and mixed tumors in 10 pts. Primary locations were as follows: ovary (O) 33 pts and extragonadal (EG) 10 pts (pineal in 4 cases, mediastinum in 3, sacrum in 2 and pharynx in 1). Stage: I in 20 (16 O, 4 EG), II in 7 (5 O, 2 EG), III in 12 (10 O, 2 EG) and IV in 4 (2 O, 2 EG). Serum AFP was elevated in 20/22 non-dysgerminoma pts, HCG in only 5 pts and LDH in 15/36 pts. TREATMENT RESULTS: Ovarian tumors: all but one pt (biopsy only) underwent surgery: unilateral oophorectomy was performed in 15 pts and bilateral oophorectomy (+/- hysterectomy, +/- others) in 17 pts. Fourteen pts were rendered disease-free, 8 pts had residual tumor (RT) < 2 cm and 11 RT > 2 cm. Chemotherapy (PVB or BEP) was given to 28 pts, radiotherapy to 2 pts and no additional treatment to 3. Finally, 30 pts achieved complete response (CR) and none have relapsed at a median follow-up of 43 months. EG tumors: None of the pts underwent radical surgery. Radiotherapy was applied to 4 pineal tumors and BEP or PVB were given to all 10 pts. To date 6 pts are disease-free, 1 is alive with mature teratoma, 2 are alive with disease and 1 died of toxic effects. The projected overall survival of the series as a whole is 89% at 10 years, and it is significantly higher for pts without EST (p < 0.02) and for pts with AFP < 1000 (P < 0.01) and age < 22 years at diagnosis (p < 0.01). The projected event-free survival at 10 years is 80.4% (87.7% for ovarian tumors vs. 54% for extragonadal, p = 0.05). No events were recorded after 28 months. CONCLUSIONS: The present results reflect the dramatic effectiveness of cisplatin-based chemotherapy for ovarian MGCT and confirm that unilateral oophorectomy can preserve fertility without compromising cure. Age > 22 years, histology (EST) and serum AFP > 1000 ng/ml are possible prognostic factors (univariate analysis) to be tested in an independent body of data on cisplatin-treated patients.

Neoplasms of unknown primary site: a clinicopathological study of autopsied patients.

BACKGROUND: Malignant neoplasms of an unknown primary site (NUPS) remain a diagnostic and therapeutic challenge in clinical practice. With this in mind, we have reviewed all autopsies performed in patients with NUPS in a single institution. PATIENTS AND METHODS: By reviewing 1656 autopsies performed on adults in our institution (1974-1990), 43 cases of NUPS were found. (NUPS was defined as histologically proven malignant tumor for which a primary site could not be found after anamnesis, complete physical examination, chest X-ray and routine chemistries.) RESULTS: There were 24 men (56%) and 19 women. Mean age was 62 years (76% of patients were aged 40-75). Clinical presentation included general deterioration (73%), digestive symptoms (58%), liver enlargement (58%) abdominal pain (56%), respiratory symptoms (45%), ascites (26%) and node enlargement (16%). Abnormalities in analysis and image tests were frequent but nonspecific. Median time from admission to death was 42 days (range, 4-135). Pathologic diagnoses at autopsy were: 23 adenocarcinomas (53%), arising from pancreas (6), biliary tree (6), lung (3), prostate (2), stomach (1), kidney (1) and unknown (4); 3 squamous carcinomas (5%) (1 renal pelvis, 1 biliary tree, 1 stomach); 5 undifferentiated carcinomas (1 lung, 4 unknown); and 12 miscellaneous tumors (including 3 lymphomas, 3 neuroendocrine tumors, 3 hepatocarcinomas, 2 mesotheliomas and 1 melanoma). There was a tendency towards a metastatic pattern different from that expected from the primary tumor. Image tests were of little usefulness in the search for the primary tumor. CONCLUSIONS: 1) Adenocarcinomas were the most frequent tumor presenting as NUPS, especially from the pancreas and biliary tree. 2) In this series, at least 11% of patients were amenable to standard systemic therapies (3 lymphomas and 2 prostatic adenocarcinomas) if a correct pathologic diagnosis could have been established when alive. 3) Presenting symptoms and metastatic pattern differed from those expected for the primary neoplasm eventually found. 4) Image tests were often misleading as regards the primary site, although they were useful to quantify the dissemination of the tumor.

Granular cell basal cell carcinoma. Light microscopy, immunohistochemical and ultrastructural study.

Granular cell basal cell carcinoma (BCC) is a rare histological variant of BCC. In this, the fifth reported case, a 67-year-old male with BCC located on the nose, light microscopy examination showed a tumour with the classical configuration of nodular BCC, in which most cells had finely granular eosinophilic cytoplasm. Ultrastructural observation showed numerous lysosome-like granules filling the cytoplasm of tumour cells, along with numerous well-formed pentalaminate desmosomes. Immunohistochemical profile (including positivity for keratins C 5.2 and AE 1 and for Leu-M1), together with the presence of cytoplasmic tonofilament bundles and desmosomes, are consistent with the proposed epithelial origin of granular cells in this tumour.

Glomus tumour of the trachea: report of a case with microscopic, ultrastructural and immunohistochemical examination and review of the literature.

A case of glomus tumour of the trachea is reported. The patient, a 58-year-old man, complained of dyspnoea, cough and occasional haemoptysis for many years and had been misdiagnosed as having chronic bronchitis. The diagnosis of glomus tumour in a tissue sample taken by bronchoscopy was useful in planning adequate surgery. Light and electromicroscopy of the excised tumour confirmed the preoperative diagnosis. Immunohistochemical examination showed vimentin and actin in the tumour cells, and negativity for high and low molecular weight keratins, desmin, neurofilaments, and factor VIII-related antigen, findings similar to glomus tumours of other sites.