Bilateral ovarian malignant mixed Mullerian tumor in a dog.

Malignant mixed Müllerian tumor (MMMT) is a rare neoplasm of the female genital tract. We report a case of bilateral ovarian MMMT in a 10-year-old female dog. Ovaries were only moderately enlarged with a papillary surface and firm nodules. Multiple metastases were observed in the abdominal cavity and pulmonary parenchyma. Histologically, both ovaries had intermingled carcinomatous and sarcomatous components with cartilage and bone. Metastatic lesions were not mixed. The peritoneal metastases were carcinomatous; pulmonary metastases were sarcomatous. Carcinomatous elements of the MMMT were immunohistochemically positive for cytokeratin (CK) AE1/AE3, CK7, and vimentin and estrogen receptors. Conversely, the sarcomatous cells were positive for vimentin, but negative for CKs. Chondrocytes also expressed S-100 protein. On the basis of similarities to human ovarian MMMT, the diagnosis was heterologous malignant mixed Müllerian tumor of the ovary.

Rectal endometrial stromal sarcoma arising in endometriosis: report of a case.

PURPOSE: Endometriosis of the rectovaginal septum can harbor different types of secondary tumors that may involve the rectal wall and protrude into its lumen, thus making diagnosis difficult. Extrauterine low-grade endometrial stromal sarcoma may rarely arise in endometriosis. The purpose of this article was to present the third case of this association. METHOD: This was a clinicopathologic study. RESULTS: A 42-year-old female presented with abdominal pain and fever. Laparotomy revealed a large pelvic mass involving the rectovaginal septum and the colonic wall and which protruded into the lumen forming endoluminal polypoid masses. Concomitant peritoneal nodules and a metastatic paracolic lymph node were also found. Histopathologically, primary endometriotic foci were found in close relationship with an endometrial stromal sarcoma which invaded the rectal wall. The female genital tract had no endometriotic lesions. The patient was treated by surgery and subsequent chemotherapy and was alive and well 20 months later. CONCLUSIONS: Endometriosis and its possible malignant changes should be taken into account in the differential endoscopic diagnosis of rectal masses in females.

[Blue nevus of the uterine cervix: description of a case and review of the literature]. / Il nevo blu della cervice uterina: descrizione di un caso e revisione della letteratura.

Blue nevus is a pigmented lesion of dermal melanocytes; the extracutaneous locations are uncommon. We report a case of a blue nevus of the uterine cervix in a 53 years old woman, with histochemical and immunohistochemical investigations.

Pure cystic nephroblastoma of the ovary with a review of extrarenal Wilms' tumors.

A second case of pure ovarian extrarenal Wilms' tumor (EWT) is presented. A clinical stage Ic tumor occurred in the right ovary of a 21-year-old female and corresponded to a 19-cm multilocular mass which histologically was a cystic, partially differentiated Wilms' tumor, closely resembling the highly differentiated metanephric adenoma. This pattern is reported for the first time in an ectopic location. At the interface between epithelial nests and ovarian tissue, plaques of alpha-inhibin positive cells were detected that corresponded to foci of peripheral stromal luteinization. Differential diagnosis with entities such as retiform Sertoli-Leydig cell tumors and with adenosarcoma should be made. The literature on EWT is also reviewed.

Immunohistochemical expression of BerEP4, a new epithelial antigen, in endometrial carcinoma: correlation with clinical parameters.

OBJECTIVE: To assess the immunochemical expression of BerEP4, a new epithelial antigen in endometrial carcinoma. METHODS: We studied 45 cases of endometrial carcinoma in which the BerEP4, CEA and TAG-72 antigens were searched by an immunohistochemical method. We evaluated the correlations among the immunohistochemical positivity and the grading, histotype, stage and receptorial status of the neoplasia. RESULTS: CEA was positive in 29 out of 45 cases (64.4%), TAG-72 in 17 out of 45 cases (37.7%) and BerEP4 in 31 out of 45 cases (68.9%). Both TAG-72 and CEA were inversely related to the grading while, with regard to the histotype, CEA resulted as highly positive in the 5 cases of adenoacanthoma. CONCLUSION: BerEP4 did not show any correlation with grading, histotype, stage of disease or receptorial status of the carcinoma.

Congenital mesoblastic nephroma (CMN) with an unusual immunohistochemical feature.

OBJECTIVES: To describe a case of congenital mesoblastic nephroma (CMN) treated by radical nephrectomy with no evidence of relapses after five years in spite of an unusual positivity for proliferating cellular nuclear antigen (PCNA). METHODS: A three-month-old child presented a right renal mass with compression of the inferior vena cava. Excretory urography showed an intrarenal mass with distortion of the calyceal system. There was no evidence of metastasis. Radical nephrectomy was carried out; no adjuvant therapy was given. Histological and immunohistochemical studies were performed. RESULTS: The tumor was a 6 x 6 x 5 cm solitary mass extending into perirenal tissue, involving the hilar vessels but not the ureter. Histologically, it has been classified as a congenital mesoblastic nephroma of the classic variant. Positive reaction for vimentin and actin was observed. Strong positivity for PCNA and negativity for P53 were revealed. CONCLUSIONS: PCNA is considered to be a reliable marker of potential malignancy. This, however, contrasts with the biological behavior of our case. Further evaluation is required for correct interpretation of this additional information and to avoid inappropriate aggressive therapy.

Do acute lesions of Wernicke's encephalopathy show contrast enhancement? Report of three cases and review of the literature.

Contrast medium was given intravenously to three nonalcoholic patients who underwent MRI or CT in the acute stage of Wernicke's encephalopathy. Lesions enhancement was not seen in one patient examined within 4 days of clinical onset, was mild in a another 3 days after clinical deterioration and marked in a patient examined 12 days after admission. Contrast enhancement of lesions was present in half of 12 cases of acute disease reported previously. There was a substantial overlap in the time interval between clinical onset and contrast-enhanced CT or MRI in the groups of enhancing and nonenhancing lesions. Since contrast enhancement may be absent in acute WE, proton-density and T2-weighted images are more useful for diagnosis of this reversible but potentially fatal condition.

Aggressive congenital fibromatosis of the small intestine in the newborn. Report of a case and review of the literature.

A case of so-called congenital fibro(leio)myosarcoma of the small intestine in a 18-day-old female baby, treated only with surgical resection, was studied by immunohistochemistry and electron microscopy in order to investigate the proliferating cell type. The tumour cells showed positivity only for vimentin and CD 34 and were negative for smooth muscle actin, desmin, alpha-sarcomeric actin, factor VIIIR: Ag and S-100 protein. Ultrastructural findings showed oval nuclei with prominent nucleoli, rare intracytoplasmic mitochondria and well developed rough endoplasmic reticulum. According to histoimmunological and electron microscopy findings the proliferating cells were likely to be of fibroblastic origin. A 7-year follow-up showed a favourable clinical evolution thus confirming that surgical resection can be a sufficient therapeutic approach. The morphological findings and clinical behaviour suggest that more appropriate terminology for this tumour would be "aggressive congenital fibromatosis" which better highlights its local progressive invasion without metastases.

Rhabdomyoma of the tunica vaginalis of the testis: a histologic, immunohistochemical, and ultrastructural study.

A case of an unusual tumor of skeletal muscle origin is described. The tumor was located in the tunica vaginalis of the testis in a 19-year-old man. Histologic examination showed a proliferation of elongated or round cells, with clearly discernible cross striations, surrounded by abundant mature connective tissue, consistent with genital rhabdomyoma. Immunohistochemical and electron microscopic features supported this diagnosis. Rhabdomyoma must be considered in the differential diagnosis of paratesticular tumors.

Regression of Henoch-Schönlein disease with intensive immunosuppressive treatment.

OBJECTIVE: To assess the results of a new immunosuppressive cycle, which had given favorable results in other immune-mediated glomerulonephritides, in the treatment of Henoch-Schönlein disease. METHODS: Eight patients (seven male and one female; age range, 13 to 61 years) with biopsy-proved Henoch-Schönlein were treated with the following protocol: (1) induction with 250 to 750 mg intravenous methylprednisolone every day for 3 to 7 days plus 100 to 200 mg oral cyclophosphamide every day, (2) maintenance with 100 to 200 mg oral prednisone on alternate days plus cyclophosphamide, as before, for 30 to 75 days; (3) tapering, with prednisone reduced on average by 25 mg every month while the cyclophosphamide dose remained the same, and (4) discontinuation, after at least 6 months, with abrupt interruption of cyclophosphamide and slow tapering of prednisone. The results were assessed in terms of remission, improvement, progression of disease, kidney failure, and death, unambiguously defined. The follow-up extended up to 12 years. RESULTS: Seven of eight patients had a complete remission that was maintained indefinitely thereafter. Plasma creatinine levels decreased on average from 211 +/- 81 to 92 +/- 27 mumol/L (p < 0.01) and urine protein excretion decreased from 1.9 +/- 0.8 to 0.3 +/- 0.1 gm/day (p < 0.01). One patient died of intestinal infarction caused by atherosclerotic mesenteric artery thrombosis. CONCLUSIONS: Our data suggest that an intensive immunosuppressive regimen that combines prednisone and cyclophosphamide at high doses can be effective in healing Henoch-Schönlein disease.

Immunosuppressive treatment of Berger's disease.

BACKGROUND: The aim of our study was to determine whether immunosuppressive treatment is effective in preventing and reversing the evolution of Berger's disease toward chronic renal failure. METHODS: We studied 20 unselected, consecutive patients with biopsy-proven Berger's disease who met the criteria for disease progression. They had proteinuria, significant histologic changes, persistent hematuria, and red cell casts. The treatment consisted of prednisone in an alternate-day regimen and cyclophosphamide, either in a daily oral administration or in a monthly intravenous pulse injection, both given for a 6-month cycle. Five patients had chronic renal failure (as disclosed by plasma creatinine of 230 +/- 71 mumol/L), hypertension, and proteinuria (2.7 +/- 0.8 gm/day), whereas the remaining 15 patients had normal renal function (plasma creatinine, 97 +/- 18 mumol/L) and less severe proteinuria (1.9 +/- 1.1 gm/day). However, even these 15 patients had a significant number of risk factors heralding progression to chronic renal failure. RESULTS: Over an average follow-up of 8.7 +/- 3.7 years (range, 5 to 15 years), all patients but one had complete disease remission, including five patients with incipient chronic renal failure. Relapse occurred in two patients who were healed after a repeat treatment cycle. Over the entire follow-up period, no patient progressed to chronic renal failure and plasma creatinine concentration remained stable, even in subjects in whom it was high before treatment (257 +/- 79 versus 230 +/- 71 mumol/L; p > 0.05). CONCLUSION: The immunosuppressive treatment of patients with Berger's disease with high probability of progression appears to be effective in the prevention of end-stage renal disease.

Preliminary reports on morphological and ultrastructural changes in the corpora cavernosa of the rat after chronic arterial obstruction.

OBJECTIVES: Various authors have investigated the pathological findings of penile tissue after chronic arterial obstruction, but the significance of these changes in the corpora cavernosa remains unclear. In order to evaluate the possible damages in the erectile tissue after chronic arterial obstruction at different levels, we utilized an experimental model in rats to determine the changes in smooth muscle cells and elastic fibers of the erectile tissue by microscopic immunohistochemical and ultrastructural studies. METHODS: Twelve adult male Wistar rats were employed in this study. Ten were used for the experiments and two as control. The rats were anaesthetized with ether and intraperitoneal 10% chloral hydrate. Microsurgical isolation of the involved vessels was performed with the aid of a Zeiss dissecting microscope. Partial stenosis was achieved with 11/0 nylon and amputation of the penis was performed at previously established time periods. The partial stenosis was carried out at the level of the aortic below the renal vessels (Group A), the common iliac artery on both sides (Group B) and the hypogastric artery on the left side (Group C). Arterial obstruction was maintained in each group for 50, 40 and 25 days, respectively. RESULTS/CONCLUSIONS: In comparison with normal controls, the histological findings revealed discrete and diffuse interstitial hyalinosis with reducted elastic fibers, while dilated vascular lacunae were also present on TEM (Transmission Electron Microscope) and SEM (Scanning Electron Microscope) examination. The greatest ultrastructural damages were observed in the group with obstruction of the common iliac artery on both sides. The foregoing confirms that an eventual chronic obstruction of these arteries does not allow the formation of collateral vessels for the corpora cavernosa.

Intercellular adhesion molecule-1 (ICAM-1) immunoreactivity in well-differentiated thyroid papillary carcinomas.

Fifty-nine cases of tumors, 51 taken from the thyroid gland including 17 well-differentiated papillary, 1 medullary and 3 follicular carcinomas, 14 follicular adenomas, 16 nodular goiters and 8 cases selected from other sites; 5 breast carcinomas; 1 melanoma; 1 Merkel cell tumor; and 1 squamous cell carcinoma of the uterine cervix, were investigated for intercellular adhesion molecule-1 (ICAM-1) expression. All cases of well-differentiated papillary thyroid carcinoma showed positivity for ICAM-1, whereas the follicular carcinomas, follicular adenomas, and all but one nodular goiters were negative. It is suggested that the occurrence of ICAM-1 on the thyroid cell surfaces in well-differentiated papillary thyroid carcinomas may contribute to the understanding of their biology and could be of potential significance for diagnostic purposes.

Primary spindle cell tumor of lymph node with "amianthoid" fibers: a histological, immunohistochemical and ultrastructural study.

A primary mesenchymal spindle cell tumor with amianthoid fibers was observed in an inguinal lymph node of a 70-year-old woman. With immunohistochemical stains the cells were positive for muscle actin and negative for desmin. Transmission electron microscopy (TEM) showed intracytoplasmic bundles of microfilaments with focal densities, profiles of rough endoplasmic reticulum, and basementlike material indicative of myofibroblastic and/or smooth-muscle cell differentiation. At scanning electron microscopy (SEM) the tumor was organized into slitlike spaces containing red blood cells. Amianthoid fibers showed a strong positivity for actin at their edge with a central negative core; by TEM they appeared to be formed of 200 nm thick crystallized collagen fibers surrounded by actin microfilaments arranged in a palisading fashion. The immunohistochemical and ultrastructural findings of the proliferating myoid cells suggest a possible derivation from smooth-muscle vascular cells. Collagen fiber aggregation and actin filament dismission might be secondary to anoxia due to vascular obliteration or disruption.

Rhabdoid tumours of the central nervous system. Report of three cases with immunocytochemical and ultrastructural findings.

Three cases of rhabdoid tumour of the central nervous system arising in a supratentorial location are reported. The patients were 18, 14, and 7 years old. All three tumours showed a common morphology. The neoplastic cells were usually globoid with round nuclei and prominent nucleoli and large acidophilic, cytoplasmic inclusions were present in many of them. These inclusions showed strong immunoreactivity for vimentin, weak immunoreactivity for epithelial membrane antigen and focal immunoreactivity for cytokeratins. Ultrastructurally they were made up of whorls of intermediate filaments, 8-10 nm in thickness. Rhabdoid tumours of the central nervous system, whatever the cell of origin, appear to be an independent entity with identifiable histology and aggressive behaviour.

Argyrophilic neuroendocrine carcinoma of the male breast.

A case of breast carcinoma with neuroendocrine features occurring in an elderly male patient is reported. Histologically, the tumor was characterized by solid growth with nests and ribbons of small to medium-size uniform neoplastic cells. Argyrophilia, expression of chromogranins at both protein and gene level, and the presence of dense granules of the neurosecretory type by electron microscopy were demonstrated.

Squamous cell carcinoma and lichen sclerosus et atrophicus of the prepuce.

Two cases of squamous cell carcinoma of the prepuce arising on balanitis xerotica obliterans are described. This event is unusual and not well known. The surgical treatment was a wide circumcision in which the prepuce and part of the shaft skin were removed, performing as well a decortication of the glans base. This technique seemed to be satisfactory in removing the carcinoma and obtaining a definite improvement in the clinical picture of balanitis xerotica obliterans as well.

Melanotic neuroectodermal tumor of the epididymis in infancy. A case report and review of the literature.

A melanotic neuroectodermal tumor of the epididymis in an 8-month-old child is reported. The patient was treated with radical orchiectomy. Primary involvement of the epididymis has been reported in only 10 cases previously. The therapeutic management is discussed.