Dermatofibroadenoma apocrino hemosiderótico/quiste glandular apocrino con estroma similar al dermatofibroma hemosiderótico / Apocrine hemosiderotic dermatofibroadenoma/apocrine glandular cyst with hemosiderotic dermatofibroma-like stroma

Presentamos un nuevo caso de una infrecuente lesión cutánea caracterizada por un quiste glandular apocrino rodeado por un estroma similar al dermatofibroma hemosiderótico. Revisamos la literatura, en particular con respecto a las características morfológicas, patogénicas y terminológicas de esta curiosa lesión (AU)

A new case of an unusual cutaneous lesion characterized by an apocrine gland cyst surrounded by a hemosiderotic dermatofibroma-like proliferating stroma is reported. We review the literature, especially with regard to the morphology, pathogenesis and terminology of this curious lesion (AU)

Dermatofibroma epitelioide esclerótico. Una variante histológica singular / Sclerotic epithelioid dermatofibroma. An unusual histological variant

El dermatofibroma es una frecuente lesión cutánea con muchos subtipos sobre la base de la gran variedad de patrones histológicos. La presencia de patrones inusuales y/o la coexistencia de 2 o más patrones en una misma lesión causan dificultades diagnósticas. La variante epitelioide es un infrecuente y poco reconocido subtipo de dermatofibroma y debería ser diferenciado de otros tumores benignos y malignos cutáneos con patrón epitelioide. Nosotros presentamos un nuevo caso de dermatofibroma que combina características epitelioides y esclerosantes, y revisamos la literatura, en particular con respecto a acontecimientos morfológicos y de diagnóstico diferencial (AU)

Dermatofibroma is a common skin lesion with many subtypes based on the great variety of architectural patterns. The presence of unusual patterns and/or coexistence of two or more patterns in the same lesion can cause diagnostic difficulties. The epithelioid variant is an uncommon and poorly recognized subtype of dermatofibroma and should be differentiated from other benign and malignant skin tumours with an epithelioid pattern. We present a case of dermatofibroma with both epithelioid and sclerosing features and review the literature paying particular attention to morphology and differential diagnosis (AU)

Lesión lipomatosa fibrohistiocítica hemosiderótica (tumor fibrolipomatoso hemosiderótico). Presentación de un caso y revisión de la literatura / Hemosiderotic fibrohistiocytic lipomatous lesion (hemosiderotic fibrolipomatous tumor). A case report and a review of the literature

La lesión lipomatosa fibrohistiocítica hemosiderótica, también llamada tumor fibrolipomatoso hemosiderótico, es una rara y recientemente descrita entidad fibrolipomatosa. Inicialmente considerada como el resultado de un proceso inflamatorio reactivo secundario a trauma o enfermedad vascular, evidencias más recientes sugieren que pueda ser de origen neoplásico. Presentamos el caso de una mujer de 57 años y revisamos la literatura, particularmente con respecto a acontecimientos genéticos, morfológicos, inmunohistoquímicos y de diagnóstico diferencial, con especial atención a su relación con el tumor angiectásico hialinizante pleomorfo precoz y el sarcoma fibroblástico mixoinflamatorio (AU)

A hemosiderotic fibrohistiocytic lipomatous lesion, also called hemosiderotic fibrolipomatous tumour, is a newly described, rare fibrolipomatous entity. Initially considered to be the result of a reactive inflammatory process subsequent to trauma or vascular disease, recent evidence suggests that it may have a neoplastic origin. We report a case occurring in a 57-year-old woman and review recent reports on current genetic developments, morphology, immunohistochemistry and differential diagnosis, paying special attention to its relation with early pleomorphic hyalinizing angiectatic tumour and myxoinflammatory fibroblastic sarcoma (AU)

Tumor de Sertoli-Leydig como hallazgo casual en quistectomía por endometriosis / Sertoli-Leydig tumour as a casual finding during laparoscopy for endometriosis

Los tumores de ovario se diagnostican en su mayoría mediante hallazgos casuales en pruebas diagnósticas realizadas por otro motivo. La endometriosis sintomática es un motivo de consulta frecuente en mujeres jóvenes. Los tumores de Sertoli-Leydig son tumores de origen embrionario muy poco habituales. En este caso, el diagnóstico del tumor embrionario se produce en una intervención quirúrgica por endometriosis y como hallazgo de una biopsia del ovario contralateral en una mujer con deseo reproductivo y sin sospecha previa (AU)

Ovarian tumours are mostly casual findings in imaging tests performed for another reason. Symptomatic endometriosis is a common reason in young women attending clinics. Sertoli-Leydig tumours have an embryological origin and are very rare. In this case the diagnosis was made during surgery for an endometriosis and as a finding in the contralateral ovarian biopsy (AU)

Tumor de la granulosacoexistente con cistoadenomamucinoso en el ovariocontralateral / Concurrent granulosa tumor and mucinous cystoadenoma in the contralateral ovary

Presentamos el caso de una mujer de 57 años concistoadenoma mucinoso en un ovario y tumor dela granulosa como hallazgo casual en el anejocontralateral. Clínicamente, la paciente, que referíauna amenorrea primaria, debutó con metrorragia ytumoración abdominal.Los tumores de la granulosa del adulto, quepertenecen a las neoplasias del estroma gonadaldel ovario, son más frecuentes en mujeresposmenopáusicas. En la clínica suelen debutar consíntomas secundarios a la secreción estrogénica,aunque la proporción de tumores de la granulosasecretores de hormonas es difícil de establecer (AU)

We present the case of a 57-year-old womanwith mucinous cystoadenoma in one ovary andgranulosa tumor as an incidental finding in theother. The patient reported primary amenorrhea,and clinical presentation consisted of metrorrhagiaand an abdominal mass.Adult granulosa tumors, which belong to thegroup of gonadal stroma neoplasms of the ovary,are more common in postmenopausal women.Clinically, these tumors usually manifest withsymptoms secondary to estrogen secretion, althoughthe proportion of tumors of this kind that secretehormones is difficult to establish (AU)

Atypical primary carcinoid tumour of the skin.

We present a new case of a primary carcinoid tumour of the skin. The mitotic index (4/10 HPF) warrants classification of this case as atypical. The patient was a 58-year-old woman with a 1-year history of a mass on the scalp. Literature review showed this to be only the seventh case of primary carcinoid tumour of the skin. Importantly, the evolution has been favourable in all seven tumours, with a mean follow-up of 2.5 years for the previous six cases. Although the number of cases is too small to draw definitive conclusions, information to date suggests that this type of tumour can be expected to have a benign behaviour, despite the presence in some cases of criteria suggestive of uncertainty, such as the presence of mitosis.

[Adrenal carcinoma: 7 year disease free survival after complete primary tumor resection and repeated resection of local-regional and distant recurrences. Review after one case with poor initial life expectancy]. / Carcinoma suprarrenal: supervivencia a 7 años libre de enfermedad tras resección completa del tumor primario y resecciones repetidas de recidivas locorregional y a distancia. Revisión a raiz de un caso con una pobre esperanza de vida inicial.

OBJECTIVES: We report the case of a female patient with adrenal carcinoma who had undergone surgery and presented with local-regional and distant recurrences, emphasizing the importance of the aggressive surgical treatment to achieve long-term survival which is unexpected sometimes. Currently, it represents the gold standard and all cases should be reported to stimulate other groups to work in this line. METHODS/RESULTS: We report the case of a 29-year-old female patient who consulted for left flank pain, being diagnosed of an adrenal tumor by radiological tests; she underwent surgical excision of a left adrenal carcinoma (stage II). Later on she presented with local-regional recurrences (2 times) and distant metastases (liver) undergoing excision in three procedures. Currently, the patient is alive and free of disease 7 years after diagnosis. CONCLUSIONS: Adrenal cancer recurrences have been considered lethal in the short-term. Nevertheless, an aggressive surgical approach of local recurrences and metastasic disease may significantly prolong patient's survival and, sometimes, leave the patient disease free several years after the diagnosis of the primary tumor.

Carcinoma suprarenal: supervivencia a 7 años libre de enfermedad tras resección completa del tumor primario y resecciones repetidas de recidivas locorregional y a distancia. Revisión a raíz de un caso con una pobre esperanza de vida inicial / Adrnal carcinoma: 7 year disease free survival after complete primary tumor resection and repeated resection of local-regional and distant recurrences. Review after one case with poor initial life expectancy

OBJETIVO: Comunicamos el caso de unapaciente con un cáncer suprarrenal intervenido que presentóposteriormente recidivas locorregional y a distancia,enfatizando la importancia del tratamiento quirúrgicoagresivo para lograr supervivencias a veces noesperadas a largo plazo. En la actualidad, representael “gold standard” y todos los casos deben comunicarsepara estimular a los distintos grupos a trabajar enesta línea.MÉTODO/RESULTADOS: se presenta el caso de unapaciente de 29 años que consultó por dolor en flancoizquierdo, diagnosticándose en los estudios de imagenun tumor suprarrenal; fue intervenida, realizándoseresección de un carcinoma suprarrenal izquierdo(Estadio II). Posteriormente presentó recidiva locorregional(en dos ocasiones) y a distancia (en hígado), siendoresecada la enfermedad en las tres ocasiones en sutotalidad. En el momento actual, 7 años después deldiagnóstico, está viva y libre de enfermedad.CONCLUSIÓN: la recidiva del cáncer suprarrenal seha considerado letal a corto plazo desde el punto devista pronóstico. Sin embargo, un abordaje quirúrgicoagresivo de la enfermedad recurrente y metastásicapuede prolongar de forma significativa la supervivenciadel paciente y lograr, en ocasiones, “status” de libre deenfermedad varios años después del diagnóstico deltumor primario

OBJECTIVES: We report the case of afemale patient with adrenal carcinoma who had undergonesurgery and presented with local-regional and distantrecurrences, emphasizing the importance of theaggressive surgical treatment to achieve long-term survivalwhich is unexpected sometimes. Currently, it representsthe gold standard and all cases should be reported tostimulate other groups to work in this line.METHODS/RESULTS: We report the case of a 29-yearoldfemale patient who consulted for left flank pain,being diagnosed of an adrenal tumor by radiologicaltests; she underwent surgical excision of a left adrenalcarcinoma (stage II). Later on she presented with localregionalrecurrences (2 times) and distant metastases(liver) undergoing excision in three procedures.Currently, the patient is alive and free of disease 7 yearsafter diagnosis.CONCLUSIONS: Adrenal cancer recurrences havebeen considered lethal in the short-term. Nevertheless,an aggressive surgical approach of local recurrencesand metastasic disease may significantly prolongpatient’s survival and, sometimes, leave the patient diseasefree several years after the diagnosis of the primarytumor