[Surgical treatment of nevi in children in a dermatological surgery center : Histopathology and complications]. / Operative Therapie von Nävi bei Kindern in einem dermatochirurgischen Zentrum : Histopathologie und Komplikationen.

BACKGROUND: The indication for surgical management and histological diagnosis of melanocytic nevi in children is a major challenge in clinical routine. In consultations with children and parents, the exclusion of malignant findings, on the one hand, and the risk of complications, on the other hand, are important. PATIENTS AND METHODOLOGY: Included were 946 children under the age of 10 years who underwent surgery with a suspected diagnosis of melanocytic nevus at the University Department of Dermatology, Tübingen, Germany, between 2008 and 2018. Dermatohistopathologic findings and postoperative complications were recorded. RESULTS: A clinical diagnosis of melanocytic nevus was histologically confirmed in 93.2% (882/946) of cases, whereby there were 41 Spitz nevi and 18 pigmented spindle cell tumors. Melanoma was diagnosed in 2 of the children (0.2%). In another 6.6%, non-melanocytic findings (e.g., nevus sebaceous, epidermal nevi) were diagnosed. The complication rate was low at 3%. The most common complication was the occurrence of postoperative wound infection in 1.7%. CONCLUSION: It is possible to take a biopsy or surgically remove congenital nevi of different sizes even in infants. Serial excision of congenital nevi is an important tool for this purpose. In the investigated cohort, the complication rate was low. Histological confirmation is essential in case of clinically suspicious or atypical findings.

Schwannoma del nervio facial intraparotídeo. Un dilema terapéutico / Intraparotid facial nerve schwannoma. A therapeutic dilemma

Los schwannomas del nervio facial intraparotídeos son tumores benignos poco frecuentes, suponiendo frecuentemente un reto diagnóstico y terapéutico. La mayoría de los pacientes presentan una masa parotídea asintomática y las pruebas de imagen y la punción con aguja fina no suelen ser concluyentes en el diagnóstico. Tras la revisión de la literatura a propósito de un caso, pretendemos proporcionar cierta guía para el tratamiento de esta rara patología (AU)

Intraparotid schwannomas of the facial nerve are uncommon benign tumors that present a challenge in diagnosis and management. An asymptomatic parotid mass is the main clinical presentation, and image studies and fine-needle aspiration biopsy do not usually give a conclusive diagnosis. The main purpose of this study of a case report and a literature review is to provide some surgical guidance for the treatment of this rare pathology (AU)

Oncocitoma fusocelular hipofisario / Spindle cell oncocytoma of the pituitary gland

El oncocitoma fusocelular de adenohipófisis (OFC) es una neoplasia no endocrina que simula macroadenoma no funcionante y está constituida por células fusiformes ricas en mitocondrias, positivas para proteína S-100, vimentina, galectina 3, EMA y TTF1. En general presenta curso clínico benigno, si bien se ha observado un significativo número de casos recidivantes. En el presente trabajo se describen 2 casos de OFC con los aspectos morfológicos característicos, uno de los cuales recidivó 7 años después del diagnóstico inicial (AU)

Spindle cell oncocytoma (SCO) is a non endocrine tumour that closely resembles a non functioning macroadenoma. SCO is composed of mitochondria-rich fusiform cells, positive for S-100 protein, vimentin, galectine-3, EMA and TTF1. Even though most SCOs have a benign course, a significant number of recidivant cases have been reported. We describe 2 cases of SCO, one of them with a late recurrence occurring 7 years after the initial diagnosis. The clinical and morphological features, immunohistochemistry and the probable origin of this unusual tumour are discussed (AU)

Tumor maligno de vaina de nervio periférico con diferenciación heteróloga: una entidad poco frecuente de pronóstico desfavorable / Malignant peripheral nerve sheath tumour with heterologous differentiation: An unusual entity with a poor prognosis

El tumor maligno de vaina de nervio periférico (TMVNP) es una neoplasia de mal pronóstico originada sobre nervios periféricos o que muestra diferenciación propia de diferentes elementos de la vaina nerviosa. Constituye un grupo de lesiones con gran heterogeneidad morfológica, cuyo diagnóstico diferencial requiere de estudios complementarios apropiados. Presentamos el caso de un varón de 42 años de edad con una masa dolorosa pretibial y signos radiológicos de agresividad. El estudio histopatológico demostró una lesión mesenquimal maligna compuesta por una proliferación fusocelular predominante, alternando con estructuras glandulares atípicas y grupos de células con diferenciación rabdomioblástica. El estudio inmunohistoquímico y molecular determinó el diagnóstico de TMVNP con diferenciación heteróloga. La presencia de elementos heterólogos en el TMVNP es muy poco habitual, lo que añade dificultad al diagnóstico histológico e implica un peor pronóstico de la enfermedad(AU)

Malignant peripheral nerve sheath tumour (MPNST) is a malignant neoplastic proliferation arising from peripheral nerves or displaying differentiation along the different elements of the nerve sheath. It includes a group of lesions with high morphologic heterogeneity and further investigations are required for their differential diagnosis. We present a case of a 42 year-old male presenting with a painful, pretibial mass which had an aggressive appearance on imaging studies. Histopathology showed a malignant mesenchymal lesion, composed of a proliferation of predominant spindle cells alternating with atypical glandular structures and groups of cells with rabdomyoblastic differentiation. Immunohistochemical and molecular assay was consistent with MPNST with heterologous differentiation. The presence of heterologous elements in a MPNST is unusual and difficult to diagnose and is associated with a poor prognosis(AU)

Spitz nevus arising upon a congenital glomuvenous malformation.

There are several reports of the collision of vascular and pigmentary anomalies (e.g., phakomatosis pigmentovascularis) and the association between congenital melanocytic nevi and infantile hemangiomas. We report a case of Spitz nevus arising in skin overlying a congenital plaque-like glomuvenous malformation (GVM). This is the first report of a Spitz nevus arising in direct contiguity to a GVM.

Agminated atypical Spitz tumor: large nasal lesion in a child with Down syndrome.

We present a 5-year-old girl with Down syndrome and a 2.5-cm agminated atypical Spitz tumor of the nose. Although atypical Spitz tumor has uncertain malignant potential, trisomy 21 is protective against melanoma. This is the first description of an atypical Spitz tumor in a patient with Down syndrome and the largest nasal lesion reported.

Acquired bilateral agminated Spitz nevi in a child with Langerhans cell histiocytosis.

Multiple Spitz nevi are rare and may occur in agminated, widespread, or dermatomal distributions. Agminated Spitz nevi usually arise in children, presenting on grossly normal, hyperpigmented, or most rarely, hypopigmented skin. We present a child with Langerhans cell histiocytosis who developed bilateral agminated Spitz nevi in the inguinal area. Unusual features included the multifocal distribution, bilateral inguinal location, and co-occurrence with Langerhans cell histiocytosis.

Sarcoma de Kaposi primario del pene en paciente VIH negativo / Primary Kaposi sarcoma of the penis in an HIV-negative patient

No disponible

No disponible

[Agminated Spitz nevi on a hyperpigmented macule]. / Nevos de Spitz agrupados sobre una mácula hiperpigmentada.

We present the case of a 2-year-old boy with multiple Spitz nevi clustered on a hyperpigmented macule that obeyed a quadrant distribution. Multiple Spitz nevi are rare and can be disseminated or clustered. A third of the cases of agminated lesions appear on hyperpigmented macules.

Lesión policroma indurada en antebrazo / Polychromic and indurated lesion in the forearm

No disponible

No disponible

Tumor amarillento-marronáceo en la espalda de 5 años de evolución / 5-year-old yellowish-brownish tumor of the back

No disponible

Epidermal nevus syndrome associated with adnexal tumors, spitz nevus, and hypophosphatemic vitamin D-resistant rickets.

The epidermal nevus syndrome is the association of epidermal nevi with abnormalities in other organ systems, most commonly the central nervous system, the skeletal system, and the eyes. We present a patient with epidermal nevus syndrome associated with hypophosphatemic vitamin D-resistant rickets and multiple adnexal and spindle cell tumors.

Multiple agminated Spitz nevi arising on a café au lait macule: review of the literature with contribution of another case.

The majority of Spitz nevi are acquired solitary lesions. Multiple Spitz nevi are rare and may develop on hyperpigmented skin. We report a 16-year-old girl with multiple Spitz nevi arranged on a café au lait macule. Immunohistochemistry showed positivity for S-100 and HMB-45. Of interest, expression of Polo-like kinase (PLK), a novel proliferation marker that recently proved to be positive in up to 98% of malignant melanoma cells, showed positivity in 40% of the nevus cells. The clinical development of multiple Spitz nevi is not yet clear, as they are preferentially excised. Reviewing 70 cases in the literature we found that multiple agminated Spitz nevi occur more frequently than reported previously. In about one-third of these cases Spitz nevi arose on congenitally hyperpigmented skin.

Epithelioid blue nevus: a rare variant of blue nevus not always associated with the Carney complex.

Epithelioid blue nevus is a rare variant of blue nevus that has been recently described in patients with Carney complex. Some of the patients with Carney complex have multiple epithelioid blue nevi and a familial history of similar lesions is often recorded. Epithelioid blue nevus consists of an intradermal melanocytic nevus composed of polygonal epithelioid cells laden with melanin. Neoplastic cells show no maturation at the base of the lesion and, in contrast with the usual stromal changes in blue nevi, epithelioid blue nevus exhibits no fibrosis of the dermis. We have studied three cases of epithelioid blue nevus in three patients with no evidence of Carney complex. The lesions were solitary and there was no family history of similar lesions. Therefore, epithelioid blue nevus is a distinctive variant of blue nevus that may also appear as a sporadic lesion and is not always associated with Carney complex.

Extensive naevus comedonicus in a child with Alagille syndrome.

Naevus comedonicus is a rare condition, thought to be a hamartoma in which cells of the pilosebaceous unit fail to develop appropriately. Although it is usually an isolated phenomenon, it has been associated with other defects, including those of the skeletal, ocular and central nervous system. A girl with this condition is described who also had Alagille syndrome (arteriohepatic dysplasia), a previously unreported association.