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We report herein one case of conventional renal cell carcinoma (RCC) producing extensive extracellular mucinous secretion in a 71-year-old man. To the best of our knowledge, the presence of mucinous secretion in this tumor has not been documented. Mucin production, despite its low frequency, can be considered an additional feature of conventional RCC. Therefore, clear cell RCC should be added to the list of parenchymal renal tumors that can show significant mucin secretion; and it should be included in the inventory of morphologic variations of this tumor, which may cause diagnostic difficulties. It is of primary importance to distinguish mucin-secreting clear cell RCC from the metastasis of a mucin-secreting tumor to conventional RCC. Presence of mucin in a clear cell carcinoma does not exclude a renal origin.

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Xanthogranulomatous orchitis (XGO) is a rare chronic inflammatory process characterized by destruction of tissue that is replaced by an outstanding cellular infiltrate of lipid-laden macrophages. To date, 20 cases of this process have been reported previously. We present herein the case of a 55-year-old man who had sustained complete tetraplegia at C-6 level and neuropathic bladder for 21 years. After repeated episodes of urinary tract infection, the patient developed a bilateral XGO and a right xanthogranulomatous epididymitis (XGE) that were treated with bilateral orchiepididymectomy. To our knowledge, a bilateral XGO has not yet been reported. Repeated episodes of high-pressure urinary reflux along the vas deferens during dyssynergic voiding possibly led to retrograde extension from the urinary tract by common urinary pathogens and development of bilateral XGO and right XGE. Since tissue destruction is a feature of this process, curative treatment required antibiotic therapy followed by bilateral excision of testes and epididymes.

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Oncocytosis (oncocytomatosis) is a rare condition characterized by the presence of innumerable oncocytic nodules in one or both kidneys, usually associated with the presence of a dominant nodule. An incidental detection of a renal tumor in a renal biopsy performed for the diagnosis of a medical disease of the kidney is exceptional. We report herein a case of a 58-year-old woman presenting with idiopathic nephrotic syndrome. Histological examination of the percutaneous renal core biopsy revealed a minimal-change glomerular lesion and oncocytosis. A contrast-enhanced abdominal CT scan showed two solid round masses, one in each kidney, measuring 1.5 and 2 cm. The nephrotic syndrome was responsive first to prednisone and finally to cyclosporine. Pathological study of both renal dominant masses confirmed that they were oncocytomas. As far as we are aware, renal oncocytosis incidentally discovered in a biopsy performed for the diagnosis of a medical disease of the kidney has not been reported.

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We report here one case of renal oncocytoma producing focal extracellular mucinous secretion in a 47-year-old woman. To the best of our knowledge, the presence of mucinous secretion in this tumor has not yet been reported. Mucin production, despite its low frequency, can be considered an additional feature of renal oncocytoma. Therefore, oncocytoma should be added to the list of parenchymal renal tumors that can show significant mucin secretion, and it should be included in the inventory of morphologic variations of oncocytoma which may cause diagnostic difficulties.

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Ventricular non-compaction is a rare cardiomyopathy characterized by numerous, excessively prominent ventricular trabeculations and deep intertrabecular recesses communicating with the ventricular cavity. The lesion is postulated to result from an intrauterine developmental arrest that stops compaction of the myocardial fiber meshwork. This cardiomyopathy affects the left ventricle, with or without concomitant right ventricular involvement. The disease is now seen with increasing frequency and it is clinically diagnosed by imaging techniques such as echocardiography or cardiac magnetic resonance. Current diagnostic criteria are considered too sensitive, particularly in black individuals. Therefore, this condition has generated considerable controversy and demands a new definition. Non-compaction cardiomyopathy shows variability of hereditary patterns, genetic heterogeneity, diversity in associated phenotypes and a wide spectrum of clinical presentation and pathophysiological findings. Non-compaction can be simply a variant of normal maturation of the ventricular myocardium with only the most severe forms producing a distinct clinical-pathological entity. Ventricular non-compaction most probably is a secondary consequence of an underlying molecular derangement produced by a pathogenetic mutation. It is likely that surgical pathologists will find this entity more frequently due to involvement in transplantation teams.

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BACKGROUND: Melanocytic nevi from the genitalia are uncommon. Nevi on the vulva are much better described than nevi on male genitalia. To our knowledge, a systematic study of preputial melanocytic nevi has not been reported. OBJECTIVE: To investigate the frequency of clinically unapparent melanocytic nevi on a series of preputial excisions. MATERIALS AND METHODS: We undertook a prospective histologic study of the prepuce obtained in a series of 372 consecutive circumcisions for phimosis performed during the period between January 2000 and December 2002. RESULTS: Incidental preputial melanocytic nevi were detected in four (1.1%) patients. Lesions were dermal in nature, most of them showed pigment in superficial dermal nests and had pseudovascular spaces. There were no cytologic atypia, mitotic figures, architectural disorder or inflammatory infiltrate. The mean (standard deviation, SD) of the maximum diameter was 1.08 (0.85) mm (range 0.34-1.79 mm). The mean age (SD) of the patients was 41.5 (4.95) years (range 29-58). CONCLUSIONS: Incidental melanocytic nevi, although uncommonly, can be observed in the prepuce. They are detected in adults and may pose a diagnostic challenge when they are detected in the setting of concurrent malignant melanoma.

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Pagetoid dyskeratosis (PD) is considered a selective keratinocytic response in which a small part of the normal population of pale keratinocytes is induced to proliferate. PD has been found incidentally in the squamous epithelium of the skin and mucosas in various locations, but not in the nipple. In cases in which PD cells are conspicuous, there is the danger of overdiagnosis. In a retrospective study, we describe the location and incidence of PD and other pale cells in the nipple epidermis, in 288 mastectomy specimens from women operated on for breast carcinoma, in situ or infiltrating, selected consecutively from our histopathologic files. In addition to the conventional histologic methods an immunohistochemical study was performed in selected cases. PD was found in 184 (63.9%) cases and was a prominent finding in 37 (12.8%) cases. Toker cells (TCs) were identified by standard light microscopy in 24 (8.3%) nipples. Paget carcinoma cells (PCCs) were found in 12 (4.2%) cases, and in 9 (3.1%) they were an incidental finding. The immunohistochemical profile of each type of pale cells was as follows: PD cells, EMA-,LMWCK-,CK7-,HMWCK+, CEA-, HER2/neu protein-, HMB45-, HPV-; TCs, EMA+, LMWCK+, CK7+, HMWCK-, CEA-, HER2/neu protein-, HMB45-, HPV-; PCCs, EMA+, LMWCK+, CK7+, HMWCK-, CEA+, HER2/neu protein+, HMB45-, HPV-. In conclusion, friction may be the stimulus for the appearance of PD cells. PD cells must be distinguished from TCs, PCCs, clear cells of Bowen's disease, pagetoid melanoma cells, cells of clear cell papulosis, koilocytes, artifactual clear cells, and glycogen-rich squamous cells. A combination of immunohistochemical markers is useful for this distinction; however, routine histologic study is usually adequate for recognizing PD. Pathologists should be familiar with the histologic features of PD in the nipple epidermis to avoid misdiagnosis.

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Prichard's structures or minute endocardial deformities with lacunas of capillary size lined by plump endothelial cells located in the fossa ovalis are an age-related alteration of unknown origin. In this report the histogenesis, the proliferative potential and the incidence in the aged of these structures are investigated. We have undertaken a prospective histological study of the fossa ovalis in a series of 111 consecutive hearts of patients aged >/=70 years obtained at autopsy. Included in this study was immunohistochemical staining for vimentin, CD31, CD34, thrombomodulin, c-kit (CD117), Ki67 (MIB1) and vascular endothelial growth factor receptor 2 in six cases showing these structures. Prichard's structures were observed in 50 hearts (45%), and were more frequent in males. We confirmed that these structures are age-related phenomena. Subendothelial structures were more common than intracavitary structures. Most individuals had Prichard's structures located on the right side; however, the structures were more numerous on the left side of the fossa ovalis. The immunohistochemical study revealed that Prichard's structures consisted of adult, fully differentiated, postmitotic-type endothelial cells. We suggest that Prichard's structures are formed by infolding of the endothelial lining of the endocardium of the fossa ovalis as an irritational response to altered blood flow, eddies or turbulence. A parallel can be established between Prichard's structures and senile angiomas as both structures increase with age; they are not genuine neoplasms; and they are overgrowths made up of endothelial cells with terminal differentiation. We propose that Prichard's structures are the cardiac equivalent of cutaneous senile angioma.

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BACKGROUND: Lipomas can undergo a variety of changes, one of which is ossification. Ossifying lipoma (OL), independent of bone tissue, is rare. No case has been reported with this heading in the dermatopathological literature. CASE REPORT: A 73-year-old woman was referred for a subcutaneous mass on the left scapular region that had been present for more than 22 years. RESULTS: The lesion was an OL measuring 8 cm and weighing 75 g. Only 25 cases of OL have been described in soft tissues. Including the present report, locations in order of frequency are the head and neck area (11 cases), the limbs (9 cases) and the trunk (6 cases). Average patient age is 50.9 years (range, 17-81 years). History is measured in years (mean, more than 6 years). Average tumor size is 7.1 cm (range, 0.8-20 cm). The adipose component is usually predominant and the mature bone tissue is irregular in distribution. Bone spicules are surrounded by fibrous tissue bands. Chondrous structures are found in 46.2% of tumors. No recurrences have been reported. CONCLUSIONS: OL is rare but has a characteristic gross, radiological and histological appearance. It has the same prognosis as simple lipoma. Excision is the treatment to be chosen.

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Cellular angiofibroma (CAF) is a rare distinctive mesenchymal neoplasm that occurs almost exclusively in the genital area. We report the case of a 38-year-old woman who presented with an asymptomatic subcutaneous mass, 3.5 cm in diameter, located in the left hypochondrium, which had progressively enlarged during the previous 6 months. The lesion was completely excised. No recurrence was observed 3 months after the excision. A review of the literature--and including the present report--revealed five cases (three men and two women) with location outside the urogenital tract. Mean age was 57 (range 38-78) years; mean 41 years for women and 68 years for men. Average tumor size was 9 (range 3.5-25) cm; mean 5 cm for women and 12 cm for men. Tumors were located in the superficial soft tissue of the trunk, except for one case in the retroperitoneum. Mean follow-up was 29 (range 3-102) months, and no patient developed recurrence or metastasis. Extragenital CAF, except for location, shows similar clinicopathological features to genital CAF. Simple tumorectomy appears to be adequate treatment. Morphologically, CAF is closely related to spindle cell lipoma.

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Pseudoepitheliomatous hyperplasia is a reactive proliferation of benign squamous epithelium that can mimic squamous cell carcinoma. Herein is a report on this type of reaction on two subgemmal neurogenous plaques in a lingual circumvallate papilla. To the authors' knowledge this is the first report on this combination of lesions. A 66-year-old man died with disseminated squamous cell carcinoma of the lung. At autopsy the tongue was found to have an incidental pseudoepitheliomatous hyperplasia on two subgemmal neurogenous plaques with ganglion cells. Subgemmal neurogenous plaque should be included in the differential diagnosis of causes of lingual pseudoepitheliomatous hyperplasia. Awareness of the morphological spectrum of subgemmal neurogenous plaques can avoid the overdiagnosis of these lesions as neural tumors or squamous cell carcinoma.

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Aberrant epididymal tissue is uncommon and may be associated with an undescended testis. To the best of our knowledge, aberrant epididymal tissue has not been described in a testicular appendix. We report the case of a 7-year-old boy with left cryptorchidism who underwent a homolateral orchidopexy. At operation, the surgeon removed a pedunculated appendix testis containing an epididymal heterotopia. This Wolffian-derivative tissue was confirmed by the diffuse strong CD10 reactivity of the luminal border of the epithelial cells. Awareness of this aberrant tissue avoids misinterpretation as a transected functional reproductive structure.

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Hemangiomas of the cardiac valves are exceptional. To our knowledge, only ten cases of valve hemangiomas, six in the mitral and four in the tricuspid valve, have been reported in the English literature. We describe an incidentally detected aortic valve hemangioma of a 62-year-old man with chronic, degenerative aortic valve stenosis, who underwent renal transplantation 7 years before. We believe that this is the first report of a hemangioma in this localization and the first one in association with solid organ transplantation. The review of the literature of the adult cases of valve hemangioma, including this report, revealed that the average age was 47.2 years (range, 24 to 68 years). No clear sex predominance has been noted. Patients can be asymptomatic or experience sudden death. Symptomatic patients have complaints of palpitations, dyspnea, or syncopal episodes. Histologically, these valve tumors are classified as capillary, cavernous, and mixed. Mean tumor size is 1.1 cm (range, 0.6 to 2 cm). In 50% of cases the hemangioma is an incidental finding at autopsy or in a removed valve. Valve aortic hemangioma, despite its rarity, should be considered in the differential diagnosis of vascular lesions of this cardiac valve.

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Isolated left ventricular non-compaction is a rare unclassified cardiomyopathy characterized by arrest of normal embryogenesis that results in the persistence of intertrabecular recesses and the development of a spongy myocardium. The pathological diagnosis is often first established at autopsy. Described herein is the case of a 57-year-old man with isolated non-compaction of the left ventricle who had a 17 year history of worsening heart failure and was successfully treated with heart transplantation. To the authors' knowledge only seven adult patients with non-compaction cardiomyopathy have been reported to have undergone heart transplantation. Including the present case the mean age of the adult patients at transplantation was 39.5 years (range, 18-60 years). The male : female ratio was 3:1. Both ventricles were involved in 37.5% of cases. The mean follow up was 6.3 years (range, 2 months-17 years). One patient died because of a malignant tumor 9 years after transplantation. The morphological pattern of the isolated ventricular non-compaction represents a pathological entity discernible from other cardiomyopathies and should be classified as a specific cardiomyopathy. It is likely that surgical pathologists will encounter this entity more frequently due to involvement in transplantation teams.

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BACKGROUND: Papillary fibroelastoma (PFE) of the right ventricle is a rare lesion. This tumor has not been described in the heart of a cardiac transplant recipient. METHODS AND RESULTS: We report a 42-year-old woman suffering from chronic ischemic cardiopathy who underwent an orthotopic cardiac transplantation. Five years after surgery, the control two-dimensional echocardiography showed no abnormalities. At this time, the programmed endomyocardial biopsies of the right ventricle included a 2-mm lesion that was diagnosed as PFE. To our knowledge, only a previous case of this tumor has been detected at random in a control cardiac biopsy. Differential diagnosis includes chordae tendinae removed at the time of cardiac sampling, Lambl excrescence, and myxoma. CONCLUSION: PFE can arise in a cardiac transplant recipient and may be detected on a random programmed biopsy.

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