Benign renal tumors are classified into renal cell tumors, metanephric tumors, mesenchymal tumors, and mixed epithelial and mesenchymal tumors. We present three cases of pecoma with a tfe3 gene abnormality detected by immunohistochemistry ihc and fluorescence in situ hybridization fish. Renomedullary interstitial tumors, also referred to as medullary fibromas, arise from the renal medulla and appear as nonenhancing hypoattenuating solid mass on ct. Most renomedullary interstitial cell tumors are small and typically measure less than 5 mm. Renomedullary interstitial cell tumor rmict, previously known as medullary fibroma, is a rare benign tumor of kidney that localizes in the medulla 1 it is almost an incidental finding at.
Urology, the gold journal, is planning a special edition on womens health in urology, with guest editor una j. Kidneys from 402 unselected autopsies were sectioned at 1 to 2 mm intervals, and all lesions were examined histologically. The 2012 isup vancouver and 2016 who classification of adult. Relating to, occurring in, or affecting interstices. Psoriasiform lichenoid dermatitis in the springer spaniel. Interstitialcell tumor article about interstitialcell. Desmoplastic small round cell tumor desmoplastic small round cell tumor. Mar 24, 20 renomedullary interstitial tumors, also referred to as medullary fibromas, arise from the renal medulla and appear as nonenhancing hypoattenuating solid mass on ct. Dermatopathology reference describes merkel cell tumor trabecular carcinoma histopathology including histologic features and provides links to additional medical references. Hepatic pecomas are quite rare and diagnosis involves positivity of melana and hmb45 on immunohistochemistry. Renomedullary interstitial tumor american urological. Renomedullary interstitial cell tumor rmict is a common benign tumor of the kidney that is usually discovered incidentally. Large medullary fibromas may protrude into the renal pelvis.
Progesterone receptor reactivity in renal oncocytoma and. Medullary fibroma renomedullary interstitial cell tumor of kidney, gross. We present the case of a young man, 24yearold, with ehe of the forearm with secondary involvement of the distal radius. A benign tumor of the testes composed of leydig cells. Histopathology images of desmoplastic small round cell tumor.
Small benign tumor composed of renomedullary interstitial cells. Contrastenhanced ct image shows an expansile homogeneous mass arrow with softtissue attenuation in the renal sinus region of the left kidney. Juxtaglomerular cell tumor renomedullary interstitial tumor schwannoma solitary. Differential diagnosis extrarenal rhabdoid tumor surgical.
The renal pyramid is the characteristic location of renomedullary interstitial cell tumors. Werner syndrome ar 8p1112 wrn bone and soft tissue sarcomas soft tissue tumors classification major types of soft tissue tumors cell type benign tumor malignant tumor myofibroblast fibroma, myxoma fibrosarcoma, mfh adipocyte lipoma liposarcoma smooth muscle cell leiomyoma leiomyosarcoma skeletal muscle cell rhabdomyoma rhabdomyosarcoma. Interstitial cell tumor definition of interstitial cell. This lesion is usually small and thus escapes clinical detection. The precipitates showed birefringence using a partially polaris ed light and were stained black in yasues silver nit raterubeanic acid method. Notably, 3 of our 4 cases had aids and were being treated with haart. Interdigitating dendritic cell sarcoma idcs is an extremely rare hematopoietic tumor representing neoplastic counterpart of interdigitating dendritic cells of lymph nodes and other hematopoietic tissues. Leydig cell tumor explanation of interstitial cell tumor.
The purpose of this special edition is to raise awareness and educate the urologic community and promote the urologists role in the patientcentered care of women. Pathology outlines perivascular epithelioid cell tumor pecoma. A critical assessment of the prognostic value of clear cell, papillary and chromophobe histological subtypes in renal cell carcinoma. The topic rmict renomedullary interstitial cell tumor you are seeking is a synonym, or alternative name, or is closely related to the medical condition renomedullary interstitial cell tumor.
Their clinical features, pathological morphology, and prognosis were investigated. In addition, common and established tumor entities have been further refined with regard to their pathological and genetic features. Interstitial cell tumors are of leydig cell origin. Find out information about interstitial cell tumor. Recently, a recurrent gene fusion nab2stat6 has been identified as molecular hallmark. Cytogenetic abnormalities of alveolar softpart sarcomas using interphase fluorescent in situ hybridization. A perivascular epithelioid cell tumor in the livera. Renomedullary interstitial cell tumors are common incidental findings that are present in 50% of adults in autopsy series. Calcium oxalate precipitates in a renomedullary interstitial. Some have suggested that these tumors may have developed as response to hypertension. Leydig cell tumour, also leydig cell tumor us spelling, testicular interstitial cell tumour and testicular interstitial cell tumor us spelling, is a member of the sex cordstromal tumour group of ovarian and testicular cancers. Usual treatment is surgery for benign tumors and chemotherapy including mtor inhibitors for malignant tumors. Who classification of tumours of the urinary system and male.
Giant renomedullary interstitial cell tumor sciencedirect. Jan 04, 2019 renomedullary interstitial cell tumor rmict is a common benign tumor of the kidney that is usually discovered incidentally. Atherosclerotic oxalosis in coronary arteries europe pmc. Epithelioid hemangioendothelioma of the forearm with radius. In the past decade, the histological classification of renal neoplasia has undergone significant changes. Leydigcell tumor explanation of interstitialcell tumor. Epithelioid hemangioendothelioma ehe is a rare, welldifferentiated endothelial tumor with intermediate malignancy which develops more frequently from the peripheral veins, generally in the lower limb. Perivascular epithelioid cell tumor pecoma with tfe3 gene. We report a case of calcium oxalate deposition in a renomedullary interstitial cell tumor rict in a patient dying of fullblown aids. Merkel cell tumor trabecular carcinoma histopathology. This website is intended for pathologists and laboratory personnel, who understand that medical information is imperfect and must be interpreted using reasonable medical judgment. Renomedullary interstitial tumor is a common tumor in the renal medulla, present in 2641% of consecutive autopsy specimens.
Benign mesenchymal renal tumors include angiomyolipoma, leiomyoma, hemangioma, lymphangioma, juxtaglomerular cell tumor, renomedullary interstitial cell tumor medullary fibroma, lipoma, solitary fibrous tumor, and schwannoma. Renomedullary interstitial cell tumour libre pathology. A broad spectrum of benign renal neoplasms in adults shows characteristic ontogeny, histology, and tumor biology. While the tumour can occur at any age, it occurs most often in young adults. The cell membranes were irregular and collagen fibrils filled the ex tracellular spaces. Kidney equivalent terms and definitions c649 excludes. Ovary tumor perivascular epithelioid cell tumor pecoma primary ovarian pec tumor. These were most frequently a papillary adenoma n 9, followed by papillary rcc n 5, clear cell rcc n 3, angiomyolipoma n 3, renomedullary interstitial cell tumour n 3, chromophobe rcc n 1, acquired cystic disease. There was no statistically significant difference in age, sex, hypertension, heart weight, tobacco smoking, diabetes mellitus, and renal function between patients with renomedullary interstitial cell tumors and those without. Immunohistochemistry in differential diagnosis see tables mlcrcccortical cyst, ccrccchromooncobhd, aberrant staining ck7 in. Pathology outlines renomedullary interstitial cell tumor. Interstitial cell tumor article about interstitial cell. Kidney equivalent terms and definitions c649 excludes lymphoma and leukemia m9590 m9992 and kaposi sarcoma m9140 jump to.
Interdigitating dendritic cell tumor sarcoma interdigitating dendritic cell sarcoma. Sep 20, 2014 perivascular epithelioid cell tumors pecomas have been increasingly associated with gene rearrangement of the transcription factor e3 tfe3. Immunohistochemical characteristics of renomedullary. Despite their frequency, little is known about their morphology and pathogenesis. Anatomy relating to or situated in the small, narrow spaces between tissues or parts of an. Renomedullary interstitial cell tumou, medullary fibroma renal medullary fibroma is a benign kidney tumour. Interstitial definition of interstitial by the free dictionary. Surgical resection is the treatment of choice for symptomatic renomedullary interstitial cell tumor. Renal interstitial cell tumors described in this report have gross and microscopic similarities to renomedullary inter stitial cell tumors fibromas described in.
The nab2stat6 fusion leads to egr1 activation and transcriptional deregulation of egr1dependent target genes and is a driving event in initiation of sft. Kidney tumor renomedullary interstitial cell tumor. The combination of calcium oxalosis and rict has not been reported before and might possibly be due to systemic. To investigate the reactivity for oestrogen and progesterone receptors er and pr in renal oncocytoma ro and chromophobe renal cell carcinoma chrcc. Pdf adolescent renomedullary interstitial cell tumor. Oct 06, 2010 benign mesenchymal renal tumors include angiomyolipoma, leiomyoma, hemangioma, lymphangioma, juxtaglomerular cell tumor, renomedullary interstitial cell tumor medullary fibroma, lipoma, solitary fibrous tumor, and schwannoma. Many new entities with distinct clinical, pathological and genetic features have been identified.
Perivascular epithelioid cell tumors are soft tissue tumors that can occur in various locations in the body whose incidence is rising. However clinically evident case is infrequent because this lesion is. Almost all are incidental finding, found in up to 40% of adult autopsies. The renal medulla appears to exert an endocrinelike antihypertensive action. Histopathology images of interdigitating dendritic cell tumor. Renomedullary interstitial cell tumor formerly fibroma of renal medulla. Hemangioendothelioma or epitheliod hemangioendothelioma in soft tissue is considered a rare sarcoma showing endothelial differentiation. Renomedullary interstitial cell tumor in a young lady. Histology coding rules two distinctly different histologiesdifferent rows, code the histology from the most representative specimen the greater amount of tumor. It primarily occurs in the abdominal cavity associated with mesentery and peritoneum and presents as large abdominal mass with peritoneal effusion. Mar 16, 2020 webpathology is a free educational resource with 10328 high quality pathology images of benign and malignant neoplasms and related entities. Perivascular epithelioid cell tumors pecomas have been increasingly associated with gene rearrangement of the transcription factor e3 tfe3.
The tumor is seen in a wide age range of adult individuals the kidney consists of 2 zones. The main candidate for exertion of this action is the renomedullary interstitial cell. Renomedullary interstitial cell tumors are common incidental findings in kidney specimens. Bone ehe comprises less than 1% of the bone neoplasms. Printable alveolar soft part sarcoma surgical pathology. Webpathology is a free educational resource with 10328 high quality pathology images of benign and malignant neoplasms and related entities. Who classification of tumours of the urinary system and male genital organs is the eighth volume in the 4th edition of the who series on histological and genetic typing of human tumours. Anaplastic large cell lymphoma extrarenal rhabdoid tumor.
Renomedullary interstitial cell tumor rmict, previously known as medullary fibroma, is a rare benign tumor of kidney that localizes in the medulla it is almost an incidental finding at autopsy. Renomedullary interstitial cell tumor or medullary fibroma, is a small tumor that commonly presents as an incidental finding, but in rare cases maybe large and symptomatic. Grossly, tumors are circumscribed, soft, lobulated, light yellow to hemorrhagic, single or multiple masses involving one or both testes. Renomedullary interstitial cell tumors are almost always incidentally identified either at autopsy or upon resection of the kidney for other reasons. Originally referred to as medullary fibroma, the terminology renomedullary interstitial cell tumor was proposed based on ultrastructural characteristics resembling renomedullary interstitial cells. Solitary fibrous tumor is a mesenchymal tumor of fibroblastic type, which can affect any region of the body. Dystrophic oxalosis has been reported in aids patients in the globe of the eye 8, the eyelids 9, and a renomedullary insterstitial cell tumor 10. B, this low power view of a renomedullary interstitial cell tumor shows tile rounded nature of the tumor and the compression by the adjoi,fing tissue. Renal interstitial cell tumors described in this report have gross and microscopic similarities to renomedullary inter stitial cell tumors fibromas described in man. A, the arrow points to a renonacdullar interstitial cell tumor in its most common location.
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