Tumor uterino que recuerda a los tumores de los cordones sexuales del ovario (UTROSCT): un caso clinicopatológico / Uterine tumour resembling ovarian sex cord tumours (UTROSCT): A clinicopathological case

Introducción: El tumor uterino que recuerda al tumor de los cordones sexuales del ovario (UTROSCT) es un tumor muy infrecuente de histogénesis incierta, incluido en la actual clasificación de tumores del estroma endometrial de la Organización Mundial de la Salud. Principales síntomas y/o hallazgos clínicos: Mujer de 48 años, sin antecedentes de interés, acudió a consulta por hipermenorreas en los últimos ciclos. La exploración ginecológica era normal. Diagnósticos principales, intervenciones terapéuticas y resultados: El estudio ecográfico mostró una lesión nodular submucosa de 26×21mm. Se le realizó histeroscopia diagnóstica, identificándose un mioma submucoso no accesible para extirpación ambulatoria. Se llevó a cabo histeroscopia quirúrgica procediéndose a miomectomía con asa de diatermia. Histológicamente se observó tejido muscular liso masivamente infiltrado por células de poco citoplasma con diferenciación glandular positivas con citoqueratinas, interpretándose como fragmentos de pared miometrial infiltrados por carcinoma. Se le realizó estudio de extensión e histerectomía. En la pieza quirúrgica no se identificó neoplasia residual. Se revaluó la biopsia previa y se amplió el estudio inmunohistoquímico, observándose positividad para marcadores de los cordones sexuales, epiteliales y musculares lisos. Ante estos hallazgos, el diagnóstico definitivo fue UTROSCT. Conclusión: El UTROSCT muestra generalmente un comportamiento benigno. Sin embargo, se considera de potencial maligno incierto, debido a que presenta una baja tasa de recurrencias y metástasis excepcionales. Desde el punto de vista histopatológico es importante reconocer esta entidad, ya que su histológica es variada imitando una amplia gama de tumores tanto benignos como malignos, por lo que es necesario realizar estudio inmunohistoquímico para su correcto diagnóstico.(AU)

Introduction: Uterine tumour resembling ovarian sex cord tumour (UTROSCT) is a very rare tumour of uncertain histogenesis, included in the current classification of endometrial stromal tumours of the World Health Organization. Main symptoms and/or clinical findings: 48-year-old woman with no history of interest, consulted for hypermenorrhoea in recent cycles. The gynaecological examination was normal. Main diagnoses, therapeutic interventions, and results: The ultrasound study showed a submucosal nodular lesion of 26×21mm. A diagnostic hysteroscopy was performed, identifying a submucosal myoma not accessible for outpatient removal. Surgical hysteroscopy was performed, proceeding to myomectomy with loop diathermy. Histologically, smooth muscle tissue was observed massively infiltrated by cells with scant cytoplasm and positive glandular differentiation for cytokeratins, interpreted as fragments of the myometrial wall infiltrated by carcinoma. An extension study and hysterectomy were performed. No residual neoplasia was identified in the surgical piece. A previous biopsy was re-evaluated and an immunohistochemical study was extended, showing positivity for sexual cord, epithelial, and smooth muscle markers. Given these findings, the definitive diagnosis was UTROSCT. Conclusion: UTROSCT generally shows benign behaviour. However, it is considered to be of uncertain malignant potential, due to its low rate of recurrence and rare metastases. From the histopathological point of view, it is important to recognize this entity, since its histology is varied, mimicking a wide range of tumours, both benign and malignant, making it necessary to perform an immunohistochemical study for its correct diagnosis.(AU)

[Vulvar squamous cell carcinoma in young women with HPV negative]. / Carcinoma epidermoide de vulva en mujer joven con VPH negative.

BACKGROUND: The vulvar cancer is the fourth more frequent neoplasia after the endometrial, cervix and ovarian cancer. Normally, it has been related to old women of ages from 70 to 80 years old. Rarely, it has been detected cases in adult or young women. However, its incidence has been increased in the last years and in more early years. It is for this change in the incidence and its appearance in early years why a possible etiology has been looked for, opening different hypothesis that go from that related to the HPV to those that study an inflammatory chronic process as the basis for the carcinogenesis. CLINICAL CASE: In this article, it has been presented the case of a woman who is 34 years old with negative VPH that made her debut with epidermoid carcinoma of the vulva moderately different and on purpose of the case, we do a revision of the literature existent. CONCLUSIONS: Vulvar cancer diagnosed in young women as in older, but with different trends, risk factors and natural history. The case reported here escapes the theories studied so far so needed new lines of inquiry to investigate this form of presentation young woman, without HPV infection.

Carcinoma de trompa de Falopio: revisión de casos en el Hospital General Universitario de Albacete / Primary fallopian tube carcinoma. Review of cases. Hospital General Albacete

Introducción: El carcinoma de trompa de Falopio primario (CTFP) constituye la neoplasia maligna menos frecuente del aparato genital femenino. Su incidencia es mayor entre la cuarta y la sexta década de la vida. Debido a su baja prevalencia y a su sintomatología inespecífica, el diagnóstico de esta enfermedad raramente se realiza antes de la cirugía. Objetivo: El principal objetivo de esta revisión ha sido conocer la forma de presentación y evolución del CTFP. Para ello se han analizado los casos de CTFP ocurridos en el Hospital General Universitario de Albacete desde el año 2000. Material y métodos: Hemos realizado un estudio descriptivo retrospectivo que incluye una serie de 5 casos de CTFP tratados en nuestro centro. Resultados: La edad media de las pacientes en el momento del diagnóstico es de 55 años. El síntoma más frecuente ha sido la hemorragia genital. En una de las pacientes, a pesar de que el diagnóstico de CTFP se realiza de manera post-operatoria, se sospechó un CTFP con las pruebas diagnósticas previas a la cirugía. El examen histopatológico reveló 3 casos de adenocarcinomas de tipo seroso y 2 de tipo endometrioide. La citología de líquido peritoneal fue positiva en el 80% de los casos. En cuanto al estadio clínico, 2 pacientes se encontraban en estadio I, 2 en estadio II y sólo un caso en estadio III. El seguimiento medio de estas pacientes ha sido de 31 meses, produciéndose recidiva en 3 de los 5 casos, a nivel local, dentro del primer año tras el diagnóstico. Conclusiones: ElCTFP, aunque es una entidad rara, no podemos olvidarlo ante una paciente con hidromenorrea, dolor abdominal, masa anexial y alteraciones citológicas cervicovaginales y/o endometriales (AU)

Introduction: Primary fallopian tube carcinoma (PFTC) is the rarest of all gynecologic cancers. It frequently occurs between fourth and sixth decade of life. Because of its low prevalence and its unspecific symptoms, PFTC is rarely diagnosed before surgery. Objetive: The main objective of this study is to analyze the patients who were diagnosed of PFTC in the General Hospital of Albacete between 2000 and 2009. Material and methods: We present a retrospective descriptive study involving 5 patients with PFTC treated in our hospital. Results: The average age of the patients at the moment of diagnosis is 55 years. The most frequent symptom has been the vaginal bleeding. In one of the patients, although the diagnosis of PFTC was done postoperative, we suspected PFTC with the preoperative study. Histopathological examination revealed 3 cases of serous adenocarcinoma and 2 cases of endometroid adenocarcinoma. Peritoneal cytology was positive in 80% of the cases. As far as the surgical staging is concerned, 2 patients were diagnosed in stage I, 2 patients in stage II and only one patient in stage III. The average follow has been 31 months, appearing recurrence in 3 of the 5 cases during the first year, as a local recurrence. Conclusion: Although PFTC is a very rare malignancy, it should be suspected in a patient with vaginal bleeding, abdominal pain, adnexal mass and abnormalities of cervical or endometrial cytologies (AU)

Tumores de parótida en niños / Parotid gland´s tumors in children

Los tumores de glándulas salivares son infrecuentes en niños, afectando el 80% a la parótida y presentando una mayor proporción de malignidad que en adultos. Revisamos 8 casos en menores de 15 años tratados en nuestro hospital, atendiendo a sus antecedentes, expresividad clínica, tipo tumoral, diagnóstico, tratamiento empleado y seguimiento. Todos debutaron con masa parotídea asintomática y de crecimiento lento a una edad entre 10 meses y 14 años. Cuatro fueron adenomas pleomorfos, dos angiomas capilares, un quiste epidérmico y un carcinomamioepitelial. Destacamos la excepcionalidad del carcinoma por su rareza y por el alto grado de malignidad expresado. Se realizó punción-aspiración con aguja fina en cuatro pacientes, siendo concluyente sólo en tres. En todos se practicó tumorectomía, excepto en el carcinoma mioepitelialy en uno de los adenomas pleomorfos recidivado, en los que se realizó parotidectomía total. Los tumores malignos de la parótida son clínicamente indistinguibles de los benignos, por lo que ante toda masa palpable en la zona parotídea deberíamos establecer un diagnóstico sin demora. El tratamiento de elección es la exéresis quirúrgica con márgenes amplios, siendo otros tratamientos coadyuvantes poco útiles en niños (AU)

The tumors of the salivary glands are infrequent in children, and parotid gland is involved in 80% of them. When a salivary gland tumor is present, the chance of malignancy is greater in the child than in the adult. We reviewed 8 cases identified in patients aged 14 years and younger in our hospital, analyzing its antecedents, signs and symptoms, histological features, diagnosis, treatment and evolution. All the patients displayed preauricular painless, non-inflammatory and slow-growing masses to an age between 10 months and 14 years. Four of them were pleomorphic adenomas, two haemangiomas, one epidermal cysts and onemyoepithelial carcinoma. We emphasize the exceptional nature of the carcinoma for its rareness and for the high degree of malignancy expressed. We made a fine needle aspiration biopsy in four cases but they were conclusive only in three. All were treated by surgical resection of the tumour except for the myoepithelial carcinoma and the recurrent pleomorphic adenoma that were treated by total parotidectomy. The malignant tumours of the parotid gland are clinically indistinguishable of the benign ones, thus when any palpable mass appears in the zone of the parotid gland, an accurate diagnosis should be made without delay. The treatment of choice is the surgical excision with wide margins, being other adjuvant treatments less useful to this age that in the adult age (AU)

[Primary testicular lymphoma. Report of a case]. / Linfoma testicular primario. Presentación de un caso.

Case report of a primary testicular lymphoma in a sixty-two years old man, presenting initially as an enlargement of the testicle as the only symptom. Orchiectomy of left testicle was performed, with the diagnosis of Non-Hodgkin B lymphocytic lymphoma. The treatment applied was CHOP, presenting poor evolution with cerebral metastasis. He dead twenty-five months later diagnosis.

[Leydig cell tumor: report of 8 cases and review of the literature]. / Tumor de células de leydig: presentación de ocho casos y revisión de la literatura.

Leydig cell tumor is the most frequent non-germ cell tumors of testis, included in the group of specialized gonadal stromal neoplasms. It has a low incidence, accounting for 1-3% of testicular neoplasms. This tumor is characterized by its endocrine manifestations, due to the tumor's capacity to secrete hormones. We report eight cases, including the description of their clinical, diagnosis and therapeutic features, as well as their follow-up. We also make a review of the literature about this rare testicular tumor.

Tumor de células de Leydig: presentación de ocho casos y revisión de la literatura / Leydig cell tumor: report of eight cases and review of the literature

El tumor de células de Leydig es el más frecuente de los tumores no germinales de testículo, perteneciendo al grupo de los tumores del estroma gonadal especializado. Su incidencia es baja, ya que representa el 1-3 por ciento del total de las neoplasias testiculares. Puede manifestarse mediante alteraciones endocrinas debido a su capacidad para secretar hormonas.Presentamos nuestra serie de ocho casos con la descripción de sus aspectos clínicos, diagnósticos y terapéuticos, así como la evolución de los mismos. Asimismo realizamos una revisión de la literatura sobre este raro tumor testicular (AU)

[Renal plasmacytoma. Report of a new case]. / Plasmocitoma renal. Presentación de un nuevo caso.

OBJECTIVE: To present a case of extramedullary plasmacytoma localized in the kidney. The clinical features, diagnostic tests, treatment and outcome are discussed. METHODS/RESULTS: A 59-year-old patient presented with a right renal mass and renal failure. The complementary tests showed a lambda monoclonal band in blood and urine, and a left renal biopsy showed changes compatible with myeloma. The definitive diagnosis of plasmacytoma was based on the findings of open renal biopsy since fine needle punction findings were compatible with a carcinoma. The patient received polychemotherapy, but died one year after the diagnosis due to a rapidly progressing plasmatic cell dyscrasia. CONCLUSION: Renal plasmacytoma is rare and should be suspected when paraprotein is detected in blood and urine, and when the patient has a history of plasmatic cell dyscrasia. There is no widely-established treatment. Surgery, radiotherapy or chemotherapy, alone or in combination, can be utilized.

[Mesoblastic nephroma in the adult: report of a new case]. / Nefroma mesoblástico del adulto: aportación de un nuevo caso.

OBJECTIVE: A case of adult mesoblastic nephroma is presented. The clinical features, treatment, histological diagnosis and outcome are discussed. METHODS/RESULTS: A 68-year-old patient in whom a renal mass had been detected by ultrasound is described. The patient underwent radical nephrectomy. The anatomopathological analysis demonstrated a mesoblastic nephroma. No signs of recurrence have been observed at 3 years' follow-up. CONCLUSIONS: Mesoblastic nephroma of adulthood has a benign behavior and recurrence is rare after surgery.