Histological Features, p53, c-Kit, and Poliomavirus Status and Impact on Survival in Merkel Cell Carcinoma Patients.

BACKGROUND: Merkel cell carcinoma (MCC) is a rare and aggressive malignancy from neuroendocrine cells in the skin. Despite being one of the most life-threatening of skin cancers, little is known about the potential signaling mechanism that drives carcinogenesis in MCC. The purpose of this study is to assess the impact of Merkel cell polyomavirus (MCPyV), p53, and c-kit on the histological features and clinical prognosis of MCC treated in our regional hospitals. METHOD: The design was a retrospective study. The specimens were taken between 1993 and 2013 in 2 referral hospitals of Southern Spain. Data were collected retrospectively and analyzed using SPSS software. RESULTS: Thirteen lesions from 13 subjects were included in the study. Positivity for c-kit was associated with the absence of MCPyV viral DNA (P = 0.048) and positivity for p53 (P = 0.002). More rate of mitoses per high-power field was presented significantly in those specimens with: positivity for c-kit (P = 0.046), positivity for p53 (P = 0.05), lesions with infiltrative growth pattern (P = 0.008), and lymphovascular invasion (P = 0.034). We observed an inverse relationship between p53 expression and MCPyV infection (Pearson's coefficient: -0.524; P = 0.046) and between c-kit expression and MCPyV infection (Pearson's coefficient: -0.548; P = 0.05), whereas the relationship was positive between p53 expression and c-kit expression (Pearson's coefficient: 0.884; P < 0.001). CONCLUSION: We conclude that presence of MCPyV DNA has no effect on overall survival. MCCs with p53 and c-kit expressions are associated with the absence of or low MCPyV DNA showing an inverse relationship. A multifactorial molecular pathogenesis where positivity for p53 and c-kit are associated with other mechanisms different than MCPyV (such as pro-mitotic factors) may lead to aggressive clinical behavior.

Molluscum Contagiosum Infection Involving a Benign Epidermoid Cyst in an Immunocompetent Patient.

A 78-year-old man presented with a round- to oval-shaped nodule on his right eyebrow. The lesion first developed 5 years ago as a small solitary white nodule and subsequently enlarged over the past 2 years. His medical history was unremarkable. Clinical examination revealed a 2-cm round to oval dome-shaped yellowish nodule with a dimple on the top center (Figure 1). No similar lesions were found elsewhere. With a clinical suspicion of sebaceous carcinoma, an excision of the lesion was performed under local anesthesia. The histopathologic analysis showed an epidermal cyst containing molluscum bodies along the keratin inside the cyst (Figure 2). With these findings, the diagnosis of molluscum contagiosum (MC) infection into an epidermoid cyst was made. Neither recurrence nor new similar lesions were observed at follow-up.

Spontaneous hemoperitoneum secondary to retroperitoneal tumor rupture.

OBJECTIVE: To report a case of GIST type retroperitoneal tumor with spontaneous rupture to the abdominal cavity causing acute abdomen secondary to hemoperitoneum. METHODS/RESULTS: We report the case of an 84 year-old man with history of BPH and chronic atrial fibrillation. He presented to the Emergency Department with diffuse abdominal pain, syncope and accompanying vegetative symptoms. Diagnostic work up showed a 19 cm retroperitoneal mass dependent of the left kidney with active bleeding and secondary hemoperitoneum. Left radical nephrectomy was performed with pathology report of gastrointestinal stromal tumor attached to the renal capsule. CONCLUSIONS: Spontaneous hemoperitoneum is a rare entity and it has various etiologies. It is rarely described in retroperitoneal tumors.

[Gastric bronchogenic cysts. An unusual location]. / Quistes broncogénicos gástricos. Una localización inhabitual.

Bronchogenic cysts are rare benign lesions that arise during embryogenesis of the primitive proximal intestine and are located primarily in the lung. They are lined with cuboid or columnar pseudostratified and ciliated epithelium and have elastic fibers, smooth muscle, bronchial glands and cartilage in their walls. Gastric bronchogenic cysts are extremely rare with very few reported cases. Symptoms are usually caused by compression. GIST is the main differential diagnosis and the treatment is surgical resection.

[Dendritic infraclavicular fibromyxolipoma: At the boundary between spindle cell lipoma and solitary fibrous tumour]. / Fibromixolipoma dendrítico infraclavicular: en el límite entre el lipoma de células fusiformes y el tumor fibroso solitario.

Dendritic fibromyxolipoma (DFML) is a rare soft tissue tumor recently described with only 27 cases reported in the literature. None of them have shown recurrence or metastasis after excision. Histologically it is composed of small stellate or spindle cells in a myxoid stroma with abundant collagen bundles mixed with mature adipose tissue. The proliferating cells typically show immunoexpression positive for CD34 and bcl-2. A cytogenetic analysis reveals deletion involving 13q14.3 region. We describe the first reported case to date located in the infraclavicular region. A 69 year old male with a painless mass well circumscribed and 5cm in size of several years of evolution, that after excision (2 years ago) he has not shown signs of recurrence or metastasis. This study reflects the clinicopathological features, differential diagnosis and a review of the literature of the DFML.

Hiperplasia muscular paratesticular (epididimaria) pseudotumoral. Descripción de 2 casos / Pseudotumoral paratesticular (epididymal) muscle hyperplasia. Two case reports

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Quistes broncogénicos gástricos. Una localización inhabitual / Gastric bronchogenic cysts. An unusual location

Los quistes broncogénicos son lesiones benignas inhabituales que tienen su origen durante la embriogénesis del intestino proximal primitivo y se localizan principalmente en el pulmón. Están revestidos por epitelio cuboideo o columnar seudoestratificado y ciliado, conteniendo en su pared fibras elásticas, músculo liso, glándulas seromucinosas análogas a las bronquiales y/o cartílago. Los quistes broncogénicos gástricos son extremadamente raros, existiendo muy pocos casos descritos en la literatura. Suelen dar sintomatología compresiva, siendo el principal diagnóstico diferencial el GIST y el tratamiento habitual es resección quirúrgica del mismo

Bronchogenic cysts are rare benign lesions that arise during embryogenesis of the primitive proximal intestine and are located primarily in the lung. They are lined with cuboid or columnar pseudostratified and ciliated epithelium and have elastic fibers, smooth muscle, bronchial glands and cartilage in their walls. Gastric bronchogenic cysts are extremely rare with very few reported cases. Symptoms are usually caused by compression. GIST is the main differential diagnosis and the treatment is surgical resection

Fibromixolipoma dendrítico infraclavicular: en el límite entre el lipoma de células fusiformes y el tumor fibroso solitario / Dendritic infraclavicular fibromyxolipoma: at the boundary between spindle cell lipoma and solitary fibrous tumour

El fibromixolipoma dendrítico (FMLD) es un tumor de partes blandas recientemente descrito, muy poco frecuente, de los cuales tan solo hay 27 casos reseñados en la literatura, ninguno con recidivas y/o metástasis tras su extirpación. Histológicamente está constituido por una proliferación de células pequeñas, estrelladas o fusiformes, en un estroma mixoide con abundantes haces de colágeno y áreas dispersas de tejido adiposo maduro. Dichas células proliferantes se tiñen, de forma característica, con CD34 y bcl-2, y muestran, entre otras aberraciones citogenéticas, deleción en la región 13q14.3. Presentamos un caso de FMLD localizado en la región infraclavicular, en un hombre de 69 años que refiere una masa de 5cm de diámetro, circunscrita y no dolorosa, de varios años de evolución, que tras su extirpación y durante un periodo de seguimiento de 2 años no ha mostrado recidivas ni metástasis. Describimos las características clinicopatológicas de esta entidad y sus diagnósticos diferenciales, haciendo una revisión amplia de la literatura (AU)

Dendritic fibromyxolipoma (DFML) is a rare soft tissue tumor recently described with only 27 cases reported in the literature. None of them have shown recurrence or metastasis after excision. Histologically it is composed of small stellate or spindle cells in a myxoid stroma with abundant collagen bundles mixed with mature adipose tissue. The proliferating cells typically show immunoexpression positive for CD34 and bcl-2. A cytogenetic analysis reveals deletion involving 13q14.3 region. We describe the first reported case to date located in the infraclavicular region. A 69 year old male with a painless mass well circumscribed and 5cm in size of several years of evolution, that after excision (2 years ago) he has not shown signs of recurrence or metastasis. This study reflects the clinicopathological features, differential diagnosis and a review of the literature of the DFML (AU)

Extrapulmonary lymphangioleiomyomatosis in pelvic lymphadenectomy associated with invasive endometrial carcinoma / Linfangioleiomiomatosis extrapulmonar en linfadenectomía pélvica asociada a carcinoma endometrial invasivo

La linfangioleiomiomatosis (LAM) es una rara enfermedad que afecta con más frecuencia a mujeres jóvenes en edad reproductiva y conlleva una destrucción quística progresiva de los pulmones por acumulación de células de músculo liso epitelioides (células LAM) a nivel parenquimatoso y en linfáticos. LAM se presenta como una enfermedad esporádica o integrante del complejo de esclerosis tuberosa (ET), asociado con otras neoplasias de la familia de tumores de células epitelioides perivasculares ("PEComas"). Presentamos un caso de LAM de tipo esporádico extrapulmonar en una mujer postmenopaúsica que afectaba a los ganglios linfáticos de las cadenas pélvicas con la peculiaridad de ser un hallazgo casual en la linfadenectomía electiva y reglada asociada a la cirugía de un carcinoma endometrioide (AU)

Lymphangioleiomyomatosis (LAM) is a rare disease occurring mostly in young women of childbearing age. This condition leads to a progressive cystic destruction of lungs due to the accumulation of epithelioid smooth muscle cells (LAM cells) in parenchymal and lymph nodes. LAM presents as a sporadic disease or a tuberous sclerosis complex (TSC) associated with other neoplasms within the family of the perivascular epithelioid cell tumours ("pecomas"). We present a case of a sporadic LAM affecting the lymph nodes of the pelvic chains observed as an incidental finding in a postmenopausal woman after a formal elective lymphadenectomy for endometrioid carcinoma (AU)

Hemoperitoneo espontáneo por rotura de tumor retroperitoneal / Spontaneous hemoperitoneum secondary to retroperitoneal tumor rupture

OBJETIVO: Describimos un caso de tumor retroperitoneal tipo GIST con rotura espontánea a cavidad abdominal, ocasionando un cuadro de abdomen agudo secundario a hemoperitoneo. MÉTODO Y RESULTADOS: Varón de 84 años que acude a servicio de Urgencias de nuestro hospital por cuadro sincopal, con dolor abdominal difuso y cortejo vegetativo acompañante. Tras la realización de diferentes pruebas complementarias se objetiva una masa retroperitoneal de pendiente de riñón izquierdo de unos 19 cm con sangrado activo y hemoperitoneo secundario, por lo que se realiza una nefrectomía radical izquierda con resultado anatomopatológico de tumor estromal gastrointestinal dependiente de la capsula renal. CONCLUSIONES: El hemoperitoneo espontáneo es una entidad poco frecuente y de una etiología variada estando descrita en muy raras ocasiones en tumores retroperitoneales

OBJECTIVE: To report a case of GIST type retroperitoneal tumor with spontaneous rupture to the abdominal cavity causing acute abdomen secondary to hemoperitoneum. METHODS/RESULTS: We report the case of an 84 year-old man with history of BPH and chronic atrial fibrillation. He presented to the Emergency Department with diffuse abdominal pain, syncope and accompanying vegetative symptoms. Diagnostic work up showed a 19 cm retroperitoneal mass dependent of the left kidney with active bleeding and secondary hemoperitoneum. Left radical nephrectomy was performed with pathology report of gastrointestinal stromal tumor attached to the renal capsule. CONCLUSIONS: Spontaneous hemoperitoneum is a rare entity and it has various etiologies. It is rarely described in retroperitoneal tumors

Tumor carcinoide de células claras rico en lípidos del apéndice cecal / Clear cell/lipid-rich carcinoid tumor of the caecal appendix

Introducción. Los cambios de células claras se han descrito en los tumores carcinoides de diversas localizaciones. En el apéndice cecal son raros. Estos cambios suelen asociarse a la enfermedad de Von Hippel Lindau. Método. Revisión de los datos clínicos, analíticos, características histológicas y literatura médica. Cortes representativos se tiñen con hematoxilina-eosina. El estudio inmunohistoquímico se realiza con el método estreptavidina-biotina-peroxidasa para CD56, cromogranina A, sinaptofisina, PGP 9,5, proteína S-100, citoqueratinas (AE1/AE3, 7,19 y 20), CEA e inhibina. Se realizan técnicas histoquímicas de PAS, PAS-diastasa, azul-alcián y sudán negro. Resultado y conclusión. Apéndice cecal con apendicitis aguda supurada, periapendicitis y nidos murales de un tumor carcinoide de células claras microvesicular rico en lípidos. Es muy importante conocer esta variante porque puede crear confusión con el carcinoide de células caliciformes(AU)

Introduction. Although clear cell changes have been described in carcinoid tumors in various locations, they are rarely found in the appendix. These changes are usually associated with von Hippel Lindau disease. Methods. Clinical, biochemical and histological features are analyzed, together with a review of the literature. Representative sections were stained with H&E. Immunohistochemistry was performed using streptavidin-biotin-peroxidase for CD56, chromogranin A, synaptophysin, PGP 9.5, S-100 protein, cytokeratins (AE1/AE3, 7.19 and 20), CEA and inhibin. Histochemistry using PAS, PAS-diastase, alcian blue and sudan black was also carried out. Results and conclusions. The caecal appendix was seen to have suppurative appendicitis and periappendicitis, with nests of a microvesicular clear cell, lipid-rich carcinoid tumour in the wall. This variant should be considered in the differential diagnosis with goblet cell carcinoid(AU)

Inclusiones benignas glandulares en ganglio centinela de mama en paciente con carcinoma mamario / Benign glandular inclusions in the sentinel gland in a breast cancer patient

Las inclusiones benignas glandulares en ganglios linfáticos axilares son lesiones muy poco comunes y su interpretación histológica puede ser realizada erróneamente como metástasis de carcinoma de mama. Este problema es aún mayor en estudios intraoperatorios de ganglio centinela de mama en los que puede ser causa de falsos positivos y conducir a un innecesario vaciamiento ganglionar axilar, con la morbilidad que ello conlleva. Presentamos un caso de una paciente con carcinoma de mama en cuya intervención quirúrgica se remitió un ganglio centinela para estudio intraoperatorio que contenía inclusiones benignas glandulares y que ilustra la necesidad de conocer esta patología para evitar sobretratamiento(AU)

Benign glandular inclusions in axillary lymph nodes are very rare lesions which may be mistaken for metastatic carcinoma of the breast. This problem is exacerbated when sentinel nodes are studied intraoperatively during breast surgery, as misdiagnosis can result in false positives leading to unnecessary axillary lymph node dissection and the increased associated morbidity. We report a case of a patient with breast cancer who underwent surgery and the interaoperative histology of the sentinel node revealed the presence of benign glandular inclusions. The diagnostic problem illustrated by this case, together with the importance of avoiding overtreatment, is discussed(AU)