Molecular genetic analysis of primary renal epithelial tumours with granular oncocytic cytoplasms / Análisis genético molecular de tumores epiteliales renales primarios con citoplasma oncocítico granular

SUMMARY: The group of primary renal tumours with granular-oncocytic cytoplasm is a very heterogeneous group, in its histological origin and biological behavior resulting in many diagnostic problems. In this study 57 renal epithelial tumours with granular oncocytic cells were analyzed using fluorescence in situ hybridisation (FISH), array comparative genomic hybridisation (aCGH) and polymerase chain reaction (PCR). The results of analysis in renal oncocytoma (RO) did not indicate the presence of the gene mutations or chromosomal abnormalities. Sporadic renal hybrid oncocytic/chromophobe tumours (HOCT) had multiple numerical aberrations of chromosomes 1, 2, 6, 9, 10, 13, 17, 20, 21 and 22. This type of tumour had no mutations in the VHL, c-kit, PDGFRA, and FLCN genes. Oncocytic papillary renal cell carcinoma (O-PRCC) had numerical abnormalities of chromosomes 7 and 17 and the loss of the Y chromosome. Cytogenetic analysis of 20 pigmented microcystic chromophobe renal cell carcinomas (PMChRCC) showed monosomy as the most frequent aberration in all analyzed chromosomes 1, 2, 5, 10, 13, 17 and 21. One case of chromophobe renal cell carcinoma (ChRCC) with hyaline globules had a mutation in the distal part of exon 3 of the VHL gene. Absence of genetic disorders in usual RO is common result, but we have established absence of genetic disorders even in rare variants. Variety of genetic alterations detected in sporadic renal HOCT proves it to be a separate entity, not a variant of ChRCC, while PMChRCC is an uncommon variant of ChRCC. O-PRCC is a subtype of papillary renal cell carcinoma.

RESUMEN: El grupo de tumores renales primarios con citoplasma granular-oncocítico es un grupo muy heterogéneo, en su origen histológico y comportamiento biológico, resultando en problemas de diagnóstico. En el estudio se analizaron 57 tumores epiteliales renales con citoplasma oncocítico granular mediante hibridación fluorescente in situ (FISH), hibridación genómica comparativa de matriz (aCGH) y reacción en cadena de la polimerasa (PCR). Los resultados del análisis en oncocitoma renal (RO) no indicaron la presencia de mutaciones genéticas ni anomalías cromosómicas. Los tumores oncocíticos / cromófobos híbridos renales esporádicos (HOCT) tenían múltiples aberraciones numéricas de los cromosomas 1, 2, 6, 9, 10, 13, 17, 20, 21 y 22. No se observaron mutaciones en este tipo de tumor en el VHL, c-kit, PDGFRA y genes FLCN. El carcinoma de células renales papilar oncocítico (O-PRCC) tenía anomalías numéricas de los cromosomas 7 y 17 y la pérdida del cromosoma Y. El análisis citogenético de 20 carcinomas de células renales cromófobos microquísticos pigmentados (PMChRCC) mostró que la monosomía era la aberración más frecuente en todos los cromosomas analizados 1, 2, 5, 10, 13, 17 y 21. Un caso de carcinoma de células renales cromófobo (CCRc) hialino tenía una mutación en la parte distal del exón 3 del gen VHL. La ausencia de trastornos genéticos en la OI habitual es un resultado común, pero hemos establecido la ausencia de trastornos genéticos incluso en variantes raras. Varias alteraciones genéticas detectadas en esporádica HOCT renal demuestran que es una entidad separada, no una variante de ChRCC, mientras que PMChRCC es una variante poco común de ChRCC. O-PRCC es un subtipo de carcinoma papilar de células renales.

Oncocitoma suprarrenal / Adrenal oncocytoma

Introducción: El oncocitoma suprarrenal es un tumor infrecuente e incidental y sin manifestaciones clínicas propias. Objetivo: Presentar un caso de oncocitoma suprarrenal y sus particularidades diagnósticas y terapéuticas. Caso clínico: Paciente masculino de 34 años de edad con antecedentes de salud, que se presentó por dolor lumbo-abdominal derecho, sin irradiación ni otros síntomas acompañantes. El examen físico fue normal. El ultrasonido informó un tumor de unos 7 cm de diámetro, localizado hacia el polo superior del riñón derecho. El origen suprarrenal se definió con la tomografía abdominal contrastada. Los valores sanguíneos de hormonas de la corteza suprarrenal fueron normales. Se hizo la exéresis total del tumor, mediante laparotomía convencional. El riñón estaba normal. El examen histopatológico notificó oncocitoma suprarrenal y lo ratificó la inmunohistoquímica. El paciente evolucionó satisfactoriamente. Conclusiones: El oncocitoma suprarrenal es un tumor infrecuente y de hallazgo fortuito. La tomografía abdominal contrastada no tiene alta especificidad para diferenciarlo de otros tumores suprarrenales. El perfil inmunohistoquímico del tumor es concluyente en el diagnóstico definitivo(AU)

Introduction: Adrenal oncocytoma is a rare and incidental tumor without its own clinical manifestations. Objective: To present a case of adrenal oncocytoma and its diagnostic and therapeutic characteristics. Clinical case: 34-year-old male patient with a medical history, who presented with right lumbo-abdominal pain, without radiation or other accompanying symptoms. The physical exam was normal. The ultrasound reported a tumor of about 7 cm in diameter, located towards the upper pole of the right kidney. The adrenal origin was defined with contrast abdominal tomography. The blood levels of hormones of the adrenal cortex were normal. Total excision of the tumor was performed by conventional laparotomy. The kidney was normal. Histopathological examination reported adrenal oncocytoma and immunohistochemistry confirmed it. The patient evolved satisfactorily. Conclusions: Adrenal oncocytoma is a rare and fortuitous tumor. Contrast abdominal tomography does not have high specificity to differentiate it from other adrenal tumors. The immunohistochemical profile of the tumor is conclusive in the definitive diagnosis(AU)

Oncocitoma em carúncula: relato de caso / Oncocytoma of the caruncle: a case report

RESUMO O oncocitoma é um tipo de neoplasia rara na prática clínica e descrito na literatura, principalmente quando localizado no olho. Quando localizado nos anexos oculares, é mais frequentemente na carúncula. Analisou-se o caso de uma paciente de 74 anos, caucasiana, que relatou desconforto visual no olho esquerdo, e cujo exame físico mostrou lesão tumoral na carúncula esquerda, com volume moderado, presença de neovascularização e secreção excessiva. A excisão cirúrgica da lesão foi realizada sob sedação, e a peça foi enviada para avaliação anatomopatológica. A lesão foi diagnosticada histologicamente como oncocitoma, sem malignidade, e a paciente não apresentou recidiva após o procedimento. Embora raro, esse tumor deve ser reconhecido pelos oftalmologistas, devido ao risco já relatado de desenvolvimento de adenocarcinoma.

ABSTRACT Oncocytoma is a neoplasm rarely observed in clinical practice and reported in the literature, especially when located in the eye. When described in the ocular adnexa, it is most often located in the caruncle. The case of a 74-year-old Caucasian female patient is reported. She complained of visual discomfort in the left eye, and physical examination showed a tumoral lesion in the left caruncle, of moderate volume, presence of neovascularization, and excessive secretion. Surgical excision of the lesion was performed under sedation, and the specimen was sent for pathological examination. The lesion was histologically diagnosed as oncocytoma with no malignancy, and the patient presented no recurrence after the procedure. Although rare, this tumor must be recognized by ophthalmologists due to the risk of developing adenocarcinoma, as already reported.

Synchronous eyelid oncocytoma and conjunctival intraepithelial neoplasia

Oncocytoma of the eyelid is a rare neoplasm. Oncocytoma associated with an ocular surface squamous neoplasm, namely conjunctival intraepithelial neoplasia, is very hard to find in the literature. Herein we report a case of a 53-year-old male who presented with a swelling in the right lower lid over the last 6 years, along with a growth in the conjunctiva of the same eye for the last 2 years and encroaching upon the cornea for the last 4 months. Excision biopsy of the lower lid mass showed histopathological features consistent with oncocytoma. The conjunctival tissue revealed conjunctival intraepithelial neoplasia 3 (severe dysplasia). This case documents a rare synchronous dual ocular neoplasia, a very unlikely coexistence of oncocytoma with conjunctival intraepithelial neoplasia.

Oncocitomas: hallazgos tomográficos y correlación anatomopatológica / Oncocytomas: Imaging Findings and Anatomopathology Correlation

Introducción: En imágenes, los hallazgos del oncocitoma generalmente coinciden con el carcinoma de células renales (CCR) por lo que resulta muy poco segura su discriminación mediante imágenes. Objetivos: Evaluar el comportamiento de una muestra de oncocitoma en tomografía (TC). Métodos: Se seleccionaron retrospectivamente los pacientes con cirugía de tumor renal y diagnóstico anatomopatológico de oncocitoma, que fueron tratados entre enero de 2015 y diciembre de 2017. Se incluyeron los pacientes a quienes se les realizó TC con medio de contraste endovenoso en nuestra institución. Resultados: Del total de pacientes (n = 44), 43 tenían una lesión única renal, mientras que uno tenía tres lesiones. Del total de las lesiones (n = 47), 20 (42,55 %) fueron diagnosticadas tras una nefrectomía radical y 24 (51,10 %) fueron diagnosticadas por una nefrectomía parcial. La media de diámetro máximo fue de 36,5 mm (RIQ 22-44,25), de los cuales se agruparon en menores y mayores a 4 cm; se encontraron 22 tumores en este último grupo (47 %). De estos, en 15 tumores (31,91 %) que tenían más de 4 cm se encontró la cicatriz central. Se evidenciaron calcificaciones en 3 pacientes (6,8 %). Se encontró 1 tumor (2,1 %) con inversión de realce segmentario luego de la administración medio de contraste endovenoso. En este caso, el oncocitoma era menor a 4 cm. Conclusión: El hallazgo de una masa sólida con realce más intenso que el parénquima circundante durante la fase nefrográfica obliga a considerar al oncocitoma entre los diagnósticos diferenciales

Introduction: The imaging findings of oncocytomas usually coincide with renal cell carcinoma (RCC), which makes it difficult to discriminate them in imaging. Objective: To evaluate the imaging findings of a sample of oncocytomas in tomography (CT). Methods: We retrospectively selected patients with renal tumor surgery and oncocytoma anatomopathological diagnosis, who were treated between January 2015 and December 2017. Patients who underwent CT with intravenous contrast at our institution were included. Results: Of the total number of patients (n = 44), 43 had a single renal lesion while one patient presented 3 lesions. Of the total lesions (n = 47), 20 (42.55%) were diagnosed after a radical nephrectomy and 24 (51.10%) were diagnosed by a partial nephrectomy. The mean maximum diameter was 36.5 mm (RIQ 22-44, 25), of which they were grouped by tumor length into smaller or larger than 4 cm, with 22 tumors in this last group (47%). Of these, 15 tumors (31.91 %) that were larger than 4 cm had a central scar. Calcifications were evident in 3 patients (6.8 %). One tumor (2.1%) was found with the presence of inversion of segmental enhancement after administration of intravenous contrast. In this case, the oncocytoma was less than 4 cm. Conclusion: The finding of a solid mass with more enhancement than the surrounding parenchyma during the nephrographic phase makes it necessary to consider oncocytoma among the differential diagnoses

Oncocitoma suprarrenal / Oncocytic adrenocortical carcinoma

El oncocitoma suprarrenal es un tumor extremadamente infrecuente, con apenas unos 150 casos descriptos. Paciente de sexo masculino, de 48 años, con adrenalectomía derecha por incidentaloma suprarrenal de 7 cm, no funcionante. El oncocitoma suprarrenal no puede distinguirse desde el punto de vista clínico-imagenológico de otros tumores adrenales más frecuentes, por lo que su confirmación es histológica. Rara vez son malignos, y el tratamiento aceptado es la suprarrenalectomía, idealmente por vía laparoscópica. En nuestro caso, la presencia de dos criterios histológicos menores lo clasifican como un tumor con potencial maligno.

Oncocytic adrenocortical carcinoma is an extremely rare tumor, with only 150 cases reported in the literature. A 48-year-old male patient, with right adrenalectomy due to a 7-cm non-functional adrenal incidentaloma. From a clinical imaging point of view, oncocytic adrenocortical carcinoma cannot be distinguished from other more common adrenal tumors; therefore, the diagnostic confirmation is histological. It is rarely malignant, and the accepted treatment is laparoscopic adrenalectomy. In our case, two minor histological criteria classify it as a potentially malignant tumor.

Is thyroid nodule volume predictive for malignancy?

ABSTRACT Objective: We aimed to determine the roles of preoperative thyroid nodule diameter and volume in the prediction of malignancy. Subjects and methods: The medical records of patients who underwent thyroidectomy between January 2007 and December 2014 were reviewed. The nodule diameters were grouped as < 1 cm, 1-1.9 cm, 2-3.9 cm and ≥ 4 cm, and volume was grouped as > 5 cm3, 5-9.9 cm3 and > 10 cm3. ROC (Receiver Operating Characteristic) curve analysis was performed to find the optimal cutoff value of diameter and volume that can predict malignancy. Results: There were 5561 thyroid nodules in 2463 patients. Five hundred and forty (9.7%) nodules were < 1 cm, 2,413 (43.4%) were 1-1.9 cm, 1,600 (28.8%) were 2-3.9 cm and 1,008 (18.1%) were ≥ 4 cm. Malignancy rates were 25.6%,10.6%, 9.7% and 8.5% in nodules < 1 cm, 1-1.9 cm, 2-3.9 cm and ≥ 4 cm, respectively. When classified according to volume, 3,664 (65.9%) nodules were < 5 cm3, 594 (10.7%) were 5-9.9 cm3 and 1,303 (23.4%) were ≥ 10 cm3. The malignancy rates were 12.7%, 11.4% and 7.8% for the nodules < 5 cm3, 5-9.9 cm3 and ≥ 10 cm3, respectively (p < 0.001). In ROC curve analysis, an optimal cutoff value for diameter or volume that can predict malignancy in all thyroid nodules or nodules ≥ 4 cm could not be determined. Conclusion: In this surgical series, malignancy risk did not increase with increasing nodule diameter or volume. Although the volume of malignant nodules ≥ 4 cm was higher than that of benign nodules ≥ 4 cm, there was no optimal cutoff value. The diameter or volume of the nodule cannot be used to predict malignancy or decide on surgical resection.

Proliferaciones Oncocíticas de Glándulas Salivales: Estudio Estructural e Inmunohistoquímico de 7 Casos / Oncocytic Proliferations of Salivary Glands: Structural and Immunohistochemical Study of 7 Cases

RESUMEN: Los oncocitos son células originadas probablemente por transformación metaplásica del epitelio ductal o acinar de parótida y submandibular. Su proliferación puede originar condiciones patológicas que incluyen hiperplasias oncocíticas adenomatosas multinodulares (HOAM), oncocitomas y carcinomas oncocíticos. Los tumores oncocíticos constituyen el 1 % de todos los tumores salivales y entre el 82 y 90 % se desarrollan en la parótida; el resto se divide entre la glándula submandibular y las glándulas salivales menores. Las hiperplasias oncocíticas multinodulares son extremadamente raras. En el presente trabajo se analizaron 5 casos de oncocitomas de parótida y dos casos de HOAM, uno de parótida y otro de submandibular y se describieron las características estructurales e inmunohistoquímicas de los oncocitos. Cortes seriados de las biopsias incluidas en parafina se colorearon con Hematoxilina - Eosina, Hematoxilina/ácido fosfotúngstico (PTA/H), PAS y se marcaron con AC antimitocondrial, CK 5/6, CK 20 y EMA. Los tumores mostraron un crecimiento nodular encapsulado por tejido conectivo denso. En los cortes histológicos se identificaron oncocitos eosinófilos (oscuros) y granulaciones violáceas con PTA/H. En dos casos de parótida y el caso de HOAM de submandibular presentaron además oncocitos claros PAS positivos. La inmunomarcación fue positiva en todas las células siendo la marcación para mitocondrias periférica en los oncocitos claros. Las células eosinofílicas PTA/H positivas y con fuerte marcación con AC antimitocondrial, CKs y EMA confirman el diagnóstico de patología oncocítica. En tres casos coexisten oncocitos claros y oscuros. Las células claras son oncocitos que acumulan glucógeno en su citoplasma desplazando a las mitocondrias hacia la periferia. En el diagnóstico diferencial de este tumor debemos considerar los tumores salivales con células claras, el carcinoma renal metastásico, el tumor de Whartin, la variante de células claras del carcinoma epitelial/mioepitelial y el carcinoma mucoepidermoide con metaplasia oncocítica.

ABSTRACT: Oncocytes are cells probably originated by metaplastic transformation of the ductal or acinar epithelium of parotid and submandibular. Its proliferation can cause pathological conditions that include multinodular adenomatous oncocytic hiperplasia (HOAM), oncocytomas and oncocytic carcinomas. Oncocytic tumors make up 1 % of all salivary tumors and between 82 and 90 % develop in the parotid; the rest of the tumors are divided between the submandibular gland and the minor salivary glands. Multinodular oncocytic hyperplasias are extremely rare. In the present work we analyzed five cases of parotid oncocytomas and two cases of HOAM, one of parotid and the other of submandibular; structural and immunohistochemical characteristics of the oncocytes were described. Biopsies were included in paraffin, serial cuts were stained with H&E, Hematoxylin / phosphotungstic acid (PTA / H), PAS and were marked with antimitochondrial AC, CK 5/6, CKs 20 and EMA. The tumors showed a nodular growth encapsulated by dense connective tissue. The histological cuts showed dark eosinophilic oncocytes and violaceous granulations with PTA / H. In two cases of parotid and the case of submandibular HOAM, PAS positive clear oncocytes were also present. The immunostaining was positive in all the cells, being the labeling for peripheral mitochondria in the clear oncocytes. Eosinophilic cells PTA / H positive with strongly marked with antimitochondrial AC, CKs and EMA confirm the diagnosis of oncocytic pathology. In three cases, light and dark oncocytes coexist. Clear cells are oncocytes that accumulate glycogen in their cytoplasm, displacing the mitochondria to the periphery. In the differential diagnosis we should consider salivary tumors with clear cells, metastatic renal carcinoma, Whartin's tumor, the clear cell variant of epithelial / myoepithelial carcinoma and mucoepidermoid carcinoma with oncocytic metaplasia.

Carcinoma adrenocortical oncocítico virilizante / Adrenal oncocytic virilizing carcinoma

Las neoplasias oncocíticas se encuentran raramente en la glándula suprarrenal. Suelen ser benignos y no funcionales. Existen informes extremadamente limitados de neoplasias oncocíticas suprarrenales. Alrededor del 20% de las neoplasias oncocíticas adrenocorticales muestran componentes malignos y el 10-20% produce hormonas que pueden causar desbalances hormonales. Se presenta un carcinoma adrenocortical oncocítico virilizante en mujer de 35 años de edad quien acudió a consulta por presentar hipomenorrea secundaria de seis meses de duración acompañada de hirsutismo y agravamiento del acné que afectaba principalmente la cara. El examen de laboratorio revela niveles elevados de testosterona libre. La tomografía computada mostró tumor multinodular en la glándula suprarrenal derecha con necrosis central y sin evidencia de linfadenopatía o invasión de estructuras que la rodeaban. Se programó la adrenalectomía derecha lográndose resección completa del tumor. Después de la cirugía, se normalizaron los ciclos menstruales, el hirsutismo, el acné y el resto de las pruebas. El examen histopatológico del tumor fue compatible con carcinoma adrenocortical oncocítico(AU)

Oncocytic neoplasms are found more rarely in the adrenal gland. They are usually benign and nonfunctional. There are limited reports of adrenal oncocytic neoplasms. About 20% of adrenocortical oncocytic neoplasms show malignant components and 10-20% produce hormones that can cause hormonal imbalances. We present a virilizing oncocytic adrenocortical carcinoma in a 35-year-old woman who consulted for hypomenorrhea accompanied by hirsutism and face acne worsening. Laboratory test revealed high levels of free testosterone. Computed tomography showed multinodular tumor of the right adrenal gland with central necrosis and without evidence of lymphadenopathy or invasion of surrounding structures. A right adrenalectomy was performed. After surgery, menstrual cycle, hirsutism and acne, as well as hormonal tests were normal. Histopathological examination of tumor showed an oncocytic adrenocortical carcinoma(AU)

Cistoadenoma papilar oncocítico de glándula parótida: caso clínico / Papillary oncocytic cystoadenoma of the parotid gland: case report

RESUMEN El cistoadenoma es un tumor epitelial benigno raro de glándulas salivales, de crecimiento lento y asintomático, que forma grandes masas uni o multiloculares con desarrollo papilar intraluminal. Si bien es más frecuente en glándulas salivales menores, se ha destacado lo inusual de la lesión en glándulas salivales mayores. Se presenta el caso de una mujer de 60 años, quien consultó por una masa indolora, blanda y de consistencia quistica en glándula parótida derecha, de crecimiento lento y progresivo. Su evolución fue de varios meses con piel de la zona intacta. Se realizó parotidectomia superficial. El examen histopatológico demostró cistoadenoma papilar oncocitico, tumor que ha sido destacado por su infrecuencia, y con una manifestación en parótida sumamente rara y de variados patrones histológicos. Su reconocimiento es trascendente para el profesional en casos como el que se presenta, pues su diagnóstico diferencial incluye lesiones con aspecto clínico similar y en algunos casos comportamiento agresivo.

ABSTRACT The cystadenoma is a rare benign epithelial tumor of salivary glands, characterized by slow growing and asymptomatic presentation, forming large uni or multilocular masses with intraluminal papillary development. Although it is more frequent in minor salivary glands, it has been emphasized the unusual of the major salivary glands affection. We present the case of a 60-year-old woman consulting for a painless, soft mass with cystic consistency in the right parotid gland, slow and progressive growing. The tumor evolved for several months keeping the skin of the area intact. A superficial parotidectomy was performed. Histopathological examination demonstrated a papillary oncocytic cystadenoma, a tumor that has been noted for its low frequency, and with extremely rare parotid manifestation and varied histological patterns. Its recognition is relevant for the professional in cases such as the one presented, since its differential diagnosis includes lesions with similar clinical appearance and in some cases aggressive behavior.

Renal oncocytoma in a kidney transplant patient: the imaging features on contrast-enhanced ultrasonography (CEUS): a case report / Oncocitoma renal em paciente transplantado: achados de imagem na ultrassonografia com contraste

Abstract Renal oncocytoma is an infrequently reported renal neoplasm, often asymptomatic, which usually behaves as a benign entity and is identified accidentally on radiological imaging. Transplant patients under long-term immunosuppressive drugs have a high prevalence of cancers, such as skin cancers, lymphoproliferative disorders, and renal carcinomas. We present a case report of an asymptomatic renal oncocytoma in a kidney transplant recipient presenting persistent hematuria. The features of computed tomography and contrast-enhanced ultrasound (CEUS) are presented. This was the first time we used CEUS in a transplant kidney recipient presenting a renal mass, allowing the real-time visualization of contrast-enhancement patterns during all vascular phases for the differential diagnosis of renal tumors. Although the pattern of intense vascularization could mislead to an early judgment as a malignant lesion, it could help to exclude other renal lesions without inducing nephrotoxicity.

Resumo O oncocitoma renal é uma neoplasia renal raramente relatada, muitas vezes assintomática, que geralmente se comporta como uma entidade benigna e é identificada acidentalmente em imagens radiológicas. Pacientes transplantados em regimes imunossupressores de longa duração apresentam alta prevalência de neoplasias tais como câncer de pele, distúrbios linfoproliferativos e carcinomas renais. Apresentamos o relato de um caso de oncocitoma renal assintomático em receptor de transplante renal com hematúria persistente. São apresentados os achados de imagens de tomografia computadorizada e ultrassonografia (US) com contraste. Foi a primeira vez que utilizamos a US com contraste em um receptor de transplante renal que apresentava massa renal, permitindo a visualização em tempo real dos padrões de realce do contraste em todas as fases vasculares para o diagnóstico diferencial dos tumores renais. Embora o padrão de vascularização intensa possa induzir uma avaliação precoce de lesão maligna, o exame ajuda a excluir outras lesões renais sem induzir nefrotoxicidade.

Oncocitoma fusocelular hipofisario / Pituitary spindle cell oncocytoma

El oncocitoma fusocelular es una neoplasia selar primaria no endocrina infrecuente, de curso clínico benigno. Debido a su similitud morfológica con los adenomas hipofisarios, considerar al oncocitoma como diagnóstico diferencial lleva a un abordaje quirúrgico cuidadoso, con el objetivo de evitar el sangrado intraquirúrgico y lograr la resección más completa posible, de la que parecería depender la evolución a largo plazo. Se presenta el caso de un hombre de 60 años que consultó por alteración campimétrica. La evaluación bioquímica evidenció panhipopituitarismo y la resonancia magnética (RM) una lesión selar. Se indicó tratamiento quirúrgico por compromiso visual con diagnóstico presuntivo de macroadenoma hipofisario no funcionante. El diagnóstico anatomopatológico fue compatible con oncocitoma fusocelular. En la RM selar, postquirúrgica (a los 5 meses), se observó remanente tumoral y se decidió realizar radiocirugía, constatándose en las subsiguientes RM disminución tumoral sin evidencia de remanente ni recidiva en 4 años de seguimiento. La comunicación de nuevos casos de esta entidad permitirá aumentar la disponibilidad de evidencia y ayudará a determinar la eficacia de los tratamientos disponibles y el pronóstico.

Spindle cell oncocytoma is an infrequent benign non-endocrine sellar neoplasm. Due to its similar morphology to pituitary adenomas, consideration of this differential diagnosis would conduce to a more careful surgical approach in order to avoid intraoperative bleeding and aiming to a complete resection, on which depends long-term outcomes. We present the case of a 60-year-old male who complained about visual abnormalities, with computerized visual field confirmation. On biochemistry, a panhypopituitarism was detected. The brain magnetic resonance images showed a sellar mass. A non-functioning pituitary macroadenoma was presumptively diagnosed and due to the visual impairment, surgical transesphenoidal treatment was indicated. The histological diagnosis was spindle cell oncocytoma. Five months after surgery, the control image demonstrated a lesion that was considered as remnant tumor, hence radiosurgery was performed. During the follow-up, the tumor reduced its size and four years after initial treatment, the sellar resonance imaging showed disappearance of the residual tumor. Communication of new cases of this rare entity will enlarge the existing evidence and will help to determinate the most effective treatment and prognosis.

Tratamiento del papiloma oncocítico nasosinusal avanzado sin malignización asociada, con resección endoscópica y radioterapia en arcos de volumen modulado (RAVM). Reporte de un caso / Management of advanced sinonasal non-malignant oncocytic papilloma with endoscopic resection and volumetric modulated arc radiotherapy (VMAR). Case report / Gestão do Papiloma Oncocítico Nasossinusal avançado sem malignidade associada, com ressecção endoscópica e Radioterapia em Volume de Arcos de Modulado. Relato de caso

RESUMEN El papiloma oncocítico (PO) es una neoplasia rara de la cavidad nasal y los senos paranasales y es el subtipo histológico menos frecuente del papiloma rinosinusal. Al igual que el papiloma invertido (PI), se lo considera un tumor benigno, pero su comportamiento biológico no lo es tanto por su crecimiento local agresivo, su tasa alta de recurrencia y el riesgo de transformación maligna con asociación a carcinoma. El tratamiento de elección es la resección endoscópica. Se ha usado exitosamente la radioterapia para el tratamiento de los PI extensos, pero no se conocen informes documentados de su uso en pacientes con PO avanzado o con extensión extrasinusal y sin malignización asociada. Se presenta un caso de PO unilateral izquierdo con enfermedad extensa que comprometía la pared nasal lateral y los senos maxilar, etmoidal y frontal del mismo lado, tratado con radioterapia en arcos de volumen modulado.

SUMMARY Oncocytic papiloma (OP) is a rare neoplasm of the nasal cavity and paranasal sinuses. It is the least common histological subtype of sinonasal papilloma. OP is regarded as a benign tumor like inverted papilloma (IP), but its biological behavior is not so benign due to its aggressive local growth, the high rate of recurrences and the risk of malignant transformation with associated carcinoma. Endoscopic resection is the treatment of choice for OP. No relevant reports on the use of radiotherapy are known in patients with OP with advanced disease or extrasinonasal extension without associated malignancy, although its application in advanced IP has been successfull. We report a case of unilateral left OP with advanced disease that involved lateral nasal wall, maxillary, ethmoidal and frontal sinus on the same side, treated with volumetric modulated arc radiotherapy.

RESUMO O Papiloma Oncocítico (PO) é uma neoplasia rara da cavidade nasal e os Seios Parinasais menos comum e representa o subtipo histológicos menos frequente dos Papilomas Nasossinusais (PNS). Assim como o Papiloma Invertido (PI), o PO é considerado um tumor benigno, mas o seu comportamento biológico não é tão benigno devido a seu crescimento local agressivo, a sua elevada taxa de recorrência e o risco potencial de transformação maligna com carcinoma associado. A ressecção endoscópica é o tratamento de preferência do PO. Em pacientes com doença avançada ou extensão extrassinusal sem malignidade associada, não há relatórios relevantes do uso de radioterapia (RT), embora a sua aplicação no caso dos PI avançados, em que tem sido utilizado com sucesso. Um caso de PO unilateral é apresentado com doença extensa que comprometeu a parede lateral nasal e os seios maxilar, etmoidal e frontal do mesmo lado, que foi tratado com sucesso com cirurgia endoscópica e radioterapia em arco volumetricamente modulada (RAVM).

Oncocytoma of the lacrimal gland: a case report / Oncocitoma de glândula lacrimal: relato de caso

ABSTRACT Here we describe a rare case of a benign tumor in the lacrimal gland of a healthy 4-year-old girl. Mild proptosis was the only abnormality observed on clinical examination. Magnetic resonance imaging of the right orbit revealed an oval, solid, well-circumscribed, homogeneous mass extending from the lacrimal gland and measuring 2.5 × 2.3 × 1.7 cm without any evidence of invasion into adjacent bones. The lesion was surgically excised and histological analyses defined the diagnosis of oncocytoma of the lacrimal gland. Although rare, oncocytoma should be included in the differential diagnosis of lacrimal gland tumors.

Resumo Nós descrevemos um raro caso de tumor benigno na glândula lacrimal em uma criança sadia de 4 anos de idade. Clinicamente, a paciente apresentava apenas uma discreta proptose. A ressonância nuclear magnética (RNM) de órbita direita revelou a presença de uma massa oval, sólida, bem-circunscrita, homogênea, se extendendo a partir da glândula lacrimal, medindo 2,5 cm x 2,3 cm x 1,7 cm, sem nenhum sinal evidente de invasão a estrutura óssea adjacente. A lesão foi cirurgicamente removida e analizada histopatologicamente, sendo estabelecido o diagnóstico de oncocitoma de glândula lacrimal. Apesar de raro, o oncocitoma deve ser incluído no diagnóstico diferencial de qualquer tumor originado da glândula lacrimal.

Oncocitoma: caso clínico / Oncocytoma: a clinical case

El oncocitoma es un tumor benigno que afecta varios órganos como tiroides, paratiroides, riñón y glándulas salivales, que consiste en la proliferación de células oncocíticas producidas por una gran hiperplasia mitocondrial. El oncocitoma representa menos del 1 por ciento de todos lostumores de las gandulas salivales. Cuando en estudios por imágenes de glándula parótida se observen múltiples pequeños nódulos con unamasa sólida o quística, el diagnóstico de oncocitoma debe ser considerado, especialmente en pacientes de sesenta o más años. El objetivo de este artículo es la presentación de un caso de oncocitoma y analizar el estado de arte de los casos reportados en la temática.

The oncocytoma is a benign neoplastic tumor that occurs in several organs, including the thyroid gland, parathyroid gland, kidneys, and salivary glands consisting of a proliferation of oncocytic cells produced by a large mitochondrial hyperplasia. The oncocytoma accounts for less than 1% of the whole salivary gland tumors.When multiple small nodules are found in the parotid gland with a large solid or cystic mass that is evident on imaging, a diagnosis of oncocytoma should be considered, particularly in patients of sixty years of age or older. The objective of this article is the presentation of a case of oncocytoma and the state of art of reported cases in this field.

Carcinoma renal con translocación XP11.2: informe de un caso con énfasis en los diagnósticos diferenciales / Renal carcinoma with translocation XP11.2: a case report with emphases in differential diagnostic

En adultos el carcinoma de células renales con translocación Xp11.2 es una neoplasia poco frecuente representando menos del 1% de todas las neoplasias renales. Analizar las características morfológicas de un carcinoma con translocación Xp11.2 y establecer los posibles diagnósticos diferenciales de este tumor renal. Paciente femenina de 45 años de edad, quien en mayo de 2008 acudió a emergencia por dolor en flanco izquierdo. Un ecosonograma abdominal evidenció litiasis renal izquierda y lesión sólida en polo inferior, la cual fue confirmada por tomografía abdominal. Se realizó nefrectomía laparoscópica izquierda, sin complicaciones. El espécimen quirúrgico fue enviado al departamento de patología fijado en formol tamponado al 10% y procesado para histología e inmunohistoquímica. Macroscópicamente, se evidenció en el polo inferior renal una lesión tumoral de 45 mm x 40 mm x 35 mm, amarillenta-anaranjada, con áreas de necrosis. Las secciones histopatológicas mostraron una lesión epitelial con patrón predominante papilar. Las células tumorales mostraban bordes citoplasmáticos discretos, con citoplasma eosinófilo granular y zonas vacuoladas. Se evidenció variabilidad de la forma y contorno nuclear. La inmunohistoquímica mostró reactividad nuclear para el TFE3. La correcta clasificación de estos carcinomas es importante por los factores pronósticos y predictivos, ya que estas neoplasias pueden ser sensibles a agentes quimioterapéuticos que afectan los micro túbulos.

In adult life, the kidney carcinomas associated with Xp11.2 translocations are an infrequent neoplasia representing less than the 1% of all kidneys neoplasias. To analyze the morphologic characteristics of a carcinoma with Xp11.2 translocation and to establish the probable differential diagnostics of this kidney tumour. A 45 years-old female patient, who at May 2008 was admitted to emergency by left flank pain. An abdominal ultrasound showed a left renal lithiasis and a solid lesion at the inferior pole, which was confirmed by the abdominal tomography. A left laparoscopic nephrectomy was performed without complications. The surgical specimen was send to the pathology department in 10% buffered formalin and processed to histology and immunohistochemestry. Macroscopically was observed at the inferior kidney pole a yellow orange tumor lesion with necrosis and 45 mm x 40 mm x 35 mm in size. The histopathologic sections showed an epithelial lesion with papillary pattern. The tumour cells showed discrete cytoplasmatic borders, with granular eosinophil to vacuolated appearance. Nuclei showed variability in form and size. The immunohistochemical study showed a nuclear positivity to TFE3. The correct classification of these carcinomas is important due to the prognostic and the predictive factors, since these neoplasias could be sensible to the chemotherapeutics agents that affect the micro tubules.

Expresión de renina en oncocitomas renales / Renin expression in renal oncocytomas

Introducción: algunos tumores renales han sido asociados con secreción de hormonas, incluyendo renina. Nuestro objetivo fue investigar la frecuencia de expresión de esta hormona en un tumor renal epitelial infrecuente, el oncocitoma, y si esta expresión se asocia con hipertensión arterial sistémica (HTA).Métodos: examinamos una serie de 13 oncocitomas (provenientes de igual número de pacientes) usando un anticuerpo monoclonal para detectar la hormona por inmunohistoquímica, y su asociación con HTA. Las características de la inmunotinción se evaluaron microscópicamente. Obtuvimos, de los archivos de historias, las características clínicas y de la presión arterial sistémica antes y después de la resección tumoral. Resultados: ocho de los 13 tumores (61,5 por ciento) tenían inmunorreactividad para renina, en todos ellos difusa. En tres casos la inmunomarcación fue como un punto paranuclear (dot-like) y en otros tres estaba en la porción apical del citoplasma de las células neoplásicas. En tres de los ocho casos con expresión de renina (37,5 por ciento) y en tres de los cinco sin dicha expresión (60,0 por ciento) se detectó HTA (p = 0,59). Después de la resección tumoral ninguno de los pacientes con expresión de renina e HTA mostró remisión de esta. Conclusión: la renina se expresa frecuentemente en oncocitomas renales pero es clínicamente inactiva. Serán necesarios más estudios para conocer las implicaciones de esta expresión en la presentación clínica, el diagnóstico y la histogénesis.

Introduction: Renal cell tumors have been shown to be associated with secretory products, including renin. Our aim was to investigate the frequency of renin expression in an infrequent kind of epithelial renal neoplasm, the oncocytoma, and to find out if this expression was associated to systemic high blood pressure. Methods: We examined a series of 13 tumors (from 13 patients) using a monoclonal antiserum to detect the hormone by immunohistochemistry (IHC), and its association with hypertension. IHC features were evaluated. Systemic blood pressure information before and after neoplasm resection was obtained from clinical charts.Results: We found that eight of the 13 tumors (61.5 percent) were immunoreactive for renin, all of them with diffuse staining. In three cases the immunolabeling was paranuclear dot-like and in three more it was found in the apical portion of the cytoplasm. Systemic hypertension was detected in three of the eight (37.5 percent) patients with renin expression and in three of the five (60.0 percent) without it (p = 0.59). After tumor resection none of the patients with renin expression and high blood pressure showed remission of the hypertension.Conclusion: Renin is frequently expressed in renal oncocytomas, but it appears to be clinically inactive. More studies will be necessary in order to define the implications of this expression on clinical presentation, diagnosis and histogenesis.

Adenoma oncocítico de tiroides (adenoma de células de Hürthle): revisión del espectro morfológico a propósito de cuatro casos / Thyroid oncocytic adenoma: a review of the morphological spectrum in four cases

El cambio oncocítico es un agrandamiento del citoplasma ocasionado por la acumulación de mitocondrias, que se presenta en una amplia variedad de condiciones reactivas y neoplásicas. En la glándula tiroides, dicho cambio es un fenómeno de metaplasia que ocurre en distintas situaciones que provocan estrés celular y en ocasiones da lugar a verdaderas neoplasias benignas y malignas, entre las que destaca el adenoma oncocítico. A propósito de cuatro casos estudiados en el Departamento de Patología del Hospital ABC con características citomorfológicas inusuales que representaron un reto diagnóstico, hacemos una revisión del espectro morfológico de estos tumores. Las características presentadas incluyeron necrosis isquémica masiva posterior a toma de biopsia por aspiración, tamaño mayor al esperado en un adenoma, coexistencia con una neoplasia maligna independiente y características histológicas similares a las observadas en carcinoma papilar. Es de gran importancia para el patólogo conocer estas características, para evitar errores diagnósticos que puedan implicar un inadecuado manejo terapéutico.

Oncocytic change involves a cytoplasmic enlargement due to mitochondrial accumulation observed in a wide variety of conditions and in multiple organs. It can be reactive or neoplastic. In the thyroid gland, this change is a metaplastic phenomenon that takes place under different circumstances that promote cellular stress, and could even produce a true neoplasm, both benign or malignant. The oncocytic adenoma, a malignant tumor will be described. We analyzed four cases studied at the surgical pathology department of the American British Cowdray Medical Center in Mexico City that displayed unusual cytomorphological features. They became a diagnostic challenge and in order to solve it we reviewed their morphological spectrum. Tumor characteristics in all cases included: massive ischemic necrosis short after a fine needle aspiration biopsy was performed, unexpected large size, coexistence with a malignant independent neoplasm and cytological features similar to those observed in papillary carcinomas. It is of utmost importance for the pathologist to acknowledge the existence of these features in order to avoid diagnostic mistakes that could lead to unsuccessful treatment.