Maria Sironi

Sarcomatoid carcinoma of the colon. Report of the second case with immunohistochemical study.

Sarcomatoid Carcinomas are rare tumors composed of mixed malignant epithelial and mesenchymal cells. Only one case is reported in the colon, up to date. We describe the second case in an 86-year-old woman. The tumor, replacing the intestinal wall, consisted of carcinomatous and sarcomatous areas. The former was composed of differentiated superficial adenocarcinoma and of infiltrating epithelioid undifferentiated solid portion. The latter was mostly composed of undifferentiated spindle cells with focal chondrosarcomatous and pleomorphic anaplastic areas. A strong Cytocheratin (CK) and Epithelial Membrane Antigen (EMA) immunoreactivity (IR) with focal CA 125 positivity was detected in differentiated adenocarcinomatous areas. Solid carcinoma-like areas coexpressed CK and Vimentin (VIM). The sarcomatous spindle-cell component of the tumor was largely VIM positive, with focal CK IR. Both polyclonal and monoclonal antibodies for Carcino-Embryonic Antigen (CEA) did not display any reaction.

Submandibular ectopic thyroid gland.

A 45-year-old euthyroid woman had symmetric submandlbular masses. Radionuclide imaging with Tc-99m pertechnetate will hide the presence of ectopic thyroid tissue because of the physiologic radionuclide uptake by the salivary glands, whereas a study using radioactive iodine allows a correct diagnosis. In this case, thyroid rests were situated In the submandlbular regions, with no thyroid gland in the neck, probably because of an arrest in the normal descent of thyroid tissue after the development of the two lobes.

Oncocytic Angiomyolipoma of the Kidney: A Case Report

We describe a case of oncocytic angiomyolipoma composed of polygonal eosinophilic cells, that shares many features with renal oncocytoma. It was composed of alveolar structures, with accumulation of foamy histiocyte-like cells surrounding large glob ules of acellular, nonfibrillary, densely eosinophilic basal membrane-like substance. Immunohistochemically, there was focal reactivity for HMB45 and negativity for the epithelial markers. Oncocytic angiomyolipoma lacks the atypical features of the re cently described epithelioid variant of angiomyolipoma. None of the reported cases have been associated with tuberous sclerosis

Melanotic schwannoma of the sympathetic ganglia : A histologic, immunohistochemical and ultrastructural study

We describe the case of a 46-year-old male patient who presented withpain in the left thigh, often accompanied by lumbar pain. These symptomswere sustained by a neoplasm, which was located in the sympathetic ganglia,at the level of the 3rd left lumbar spinal root and which was completelyexcised. Immunohistochemical positivity for S100, HMB45, and NSE antibodiessuggested that the lesion was a melanotic schwannoma (MS), with bothschwannian and melanocytic differentiations, the latter containingmelanosomes at ultrastructural examination. Non-recurrence after 16 monthsof follow-up further supports our diagnosis of MS.

A CASE OF MALIGNANT PHYLLODES TUMOR WITH MUSCULAR AND FATTY DIFFERENTIATIONS

A 50-year-old female underwent surgical removal of a mammary phyllodes tumor, whose peculiar histologic feature was the coexistence of areas of liposarcoma and leiomyosarcoma. The morphologic differential diagnosis is briefly discussed.

Fine‐needle aspiration cytology of low‐grade adenosquamous carcinoma of the breast

Low-grade adenosquamous carcinoma of the breast is a salivary gland-like carcinoma, traditionally considered of myoepithelial origin, characterized by dominant basal cell phenotype and related to the metaplastic carcinoma group. The presence of myoepithelial cells in the cytological smears from fine-needle aspiration causes many difficulties in the cytological diagnosis. We present a case in which immunocytochemical study of the lesion was performed on cytological smears also.

A cytologic, immunocytochemical and ultrastructural study of a malignant parotid gland myoepithelioma

A 38-year-old man presented with a mass in the superficial lobe of the left parotid gland. One year before he underwent a total thyroidectomy with cervical node dissection for a papillary carcinoma, pT2 N0. The fine needle aspiration (FNA) cytology of the parotid mass showed large sheets of cells, with irregular borders and nuclear overlapping, and single malignant cells, in a bloody background devoid of metachromatic substance. Two cell types were evident: epithelioid cells, mostly single, characterized by an eccentric, occasionally kidney-shaped or bilobated nucleus, with round or polygonal, abundant and homogeneous cytoplasm, basophilic with the May–Grünwald–Giemsa (MGG) stain (Figure 1a); and spindle-shaped cells, with bipolar form, aggregated in cohesive sheets, showing a centrally placed oval vesicular nucleus, with elongated fibrillary cytoplasm (Figure 1b). The nuclear texture was coarsely granular with a prominent nucleolus or multiple nucleoli and an irregular nuclear membrane. Scattered atypical mitotic figures were present. Occasionally normal serous acini were intermingled within the neoplastic proliferation. A diagnosis of malignant cells, not otherwise specified (NOS) was made. A superficial left parotidectomy was performed. The lobe contained a reddish, translucent, omogeneous mass, 3.5 cm in diameter, with ill defined borders (Figure 2). Histological examination of the specimen confirmed the absence of any capsule: the neoplastic proliferation infiltrated the fibrofatty tissue, the salivary gland (Figure 3) and the perineural spaces. The neoplastic cells, with a predominantly spindle shape, were haphazardly orientated with irregularly interwoven fascicles (Figure 4), delimiting nests of discohesive epithelioid cells, merging with a hyalinized or myxoid stroma (Figure 5a). The elongated processes of the spindle-shaped cells blended with hyalinized, fibrillary extracellular matrix (Figure 5b). The tumoural mass grew in a wide reticulin network. Both

Solid Variant of Adenoid Cystic Carcinoma of the Breast: A Case Series With Proposal of a New Grading System.

Adenoid cystic carcinoma (ACC) of the breast is a low-grade malignancy “triple negative” breast tumor. ACC of the breast can present a great variety of morphological features having a prognostic impact. Recently, cases of ACC having solid-basaloid features (SBACC) have been described. In the present study, 6 cases of SBACC have been reported. All the cases affected female patients aged 47 to 69 years (mean = 54 years). Two patients had metastases to the axillary lymph nodes, and 2 patients experienced local recurrences. No deaths due to the tumor were observed. A review of the literature on breast SBACC showed that local recurrences and lymph node metastases are more frequent than in the ACC conventional type; nevertheless, no deaths due to the tumor are registered at the moment. On the contrary, cases of ACC with features of malignant transformation are on record. Therefore, a new grading system on breast ACC is proposed.

Transition of Essential Thrombocythemia to Megakaryoblastic Leukemia after Long-Term Therapy with Sequential Busulfan, Pipobroman and Hydroxyurea

Accessible online at: www.karger.com/aha After reading the interesting observations of Quaglino et al. [1] concerning leukemic transformation of essential thrombocythemia (ET), we would like to report a case of a megakaryoblastic leukemia in a case with long-standing (17 years) ET. In January 1984, a 54-year-old man was admitted to our hospital for persistent microvascular thrombotic episodes of the toes, with rapid progression to ulcerative and necrotic lesions. At that time, his blood picture was as follows: Hb 14.3 g/dl, RBC 4.3 ! 1012/l, WBC 9 ! 109/l and platelet count ranging from 2.4 to 1.6 ! 1012/l. The clinical examination did not show splenomegaly or malignancies. Leukocyte alkaline phosphatase and serum ferritin levels and erythrocyte sedimentation rate were within normal limits. A bone marrow biopsy showed marked proliferation of large to giant megakaryocytes in small clusters, with deep lobated staghorn-like nuclei, but without atypical forms (fig. 1). The bone marrow erythroid and myeloid populations were within normal limits and there was neither increased reticulin network, nor neoangiogenesis. The morphological features of bone marrow, as stated by Thiele et al. [2], confirmed the diagnosis of ET. The patient treated with busulfan (Mileran) from September 1984 to July 1985 (10 months), replaced with pipobroman (Vercite), because of the appearance of lung fibrosis. Pipobroman achieved a good control of the platelet count until April 2000, when the platelet count increased; the patient then received hydroxyurea. Six months after the change of therapy, the patient’s peripheral blood picture suddenly deteriorated, with a progressive decrease in hemoglobin and leukocyte and platelet counts, so all therapy was stopped and he underwent clinico-hematological reevaluation. A bone marrow biopsy, performed to explain three-lineage cytopenia, showed severe dysplastic features of megakaryocytes, with micromegakaryocytes and undifferentiated blast, dyserythropoiesis and irregularly scattered foci of marrow hypoplasia, but, above all, a dense reticulin network. A contemporary dry tap did not allow to perform a cytogenetic study of marrow aspiration. The patient experienced rapid, progressive cytopenia, with a fall in his hemoglobin level (Hb 7.2 g/dl), platelets (18 ! 109/l), leukocytes (2.5 ! 109/l) and with a differential count as follows: neutrophils 64%, eosinophils 0%, lymphocytes 25%, monocytes 1%, blast cells 10%. A new bone marrow biopsy, performed 30 days later, showed 30% of blasts CD34+ and factor VIII+ (fig. 2 and inset), interspersed with abundant reticulin network (malignant fibrosis) and extensive Fig. 1. Marked proliferation of large to giant megakaryocytes, in small clusters, with deep lobated staghorn-like nuclei, without atypical forms; erythroid and myeloid populations within limits.

Atrio-Ventricular Nodal Tumor Associated with Polyendocrine Anomalies

We report the clinical and autopsy findings of a young female suffering of complete heart block since the age of 11 and who suddenly died at the age of 32 because of a polycystic atrio-ventricular node tumor. In association with this tumor, we observed a hyperplasia of the islets of Langherans, a true thymic hyperplasia, bilateral multicystic ovaries, adrenal tissue heterotopia and clear cell adenomatosis in the right kidney. We suggest that the polycystic atrio-ventricular node tumor may be part of a complex syndrome with endocrine disorders and heterotopias.