[Post-traumatic striate melanokeratosis in a Caucasian woman]. / Melanoqueratosis estriada postraumática en mujer de raza blanca.

CLINICAL CASE: A fair-skinned woman presented marked striate melanokeratosis in her left eye related to recurrent corneal erosion. The source of pigmentation was a conjunctival melanosis. The conjunctival melanosis responded to treatment with topical mitomycin, while the corneal pigmentation persisted. DISCUSSION: Striate melanokeratosis is a condition described in dark-skinned patients who show a well-defined pigmentation of the limbal area, with only one case of striate melanokeratosis reported previously in a Caucasian person. The stimuli for this proliferation are corneal lesions or melanosis close to the limbus. Avoiding both stimuli are the main steps in its management.

Melanoqueratosis estriada postraumática en mujer de raza blanca / Post-traumatic striate melanokeratosis in a caucasian woman

Caso clínico: Se presenta el caso de una mujer deraza blanca y piel clara, con erosión corneal recurrentey con melanosis conjuntival en OI, que desarrollóuna opacidad corneal por melanoqueratosis estriada.La melanosis conjuntival se trató con mitomicinatópica, manteniendo la pigmentación corneal.Discusión: La melanoqueratosis estriada se presentaen personas de piel oscura, con un único casodescrito en personas de raza caucásica. Ésta se originapor daño corneal o por migración de pigmentodesde melanosis conjuntivales, debiendo tratarambas condiciones para detener la pigmentación corneal(AU)

Clinical case: A fair-skinned woman presented markedstriate melanokeratosis in her left eye related torecurrent corneal erosion. The source of pigmentationwas a conjunctival melanosis. The conjunctivalmelanosis responded to treatment with topicalmitomycin, while the corneal pigmentation persisted.Discussion: Striate melanokeratosis is a conditiondescribed in dark-skinned patients who show awell-defined pigmentation of the limbal area, withonly one case of striate melanokeratosis reportedpreviously in a Caucasian person. The stimuli forthis proliferation are corneal lesions or melanosisclose to the limbus. Avoiding both stimuli are themain steps in its management(AU)

La enfermedad de Kimura: comunicación de un caso en un varón caucásico y revisión de la literatura / Kimura disease: Report of a case in a Caucasian male and revision of the literature

La enfermedad de Kimura es un trastorno inflamatorio que afecta principalmente a sujetos de origen asiático. Se manifiesta por la existencia de masas de tejido subcutáneo y subplatismal generalmente en la cabeza y el cuello. Suele acompañarse de linadenopatías, y aumento de los niveles de eosinófilos e IgE en sangre periférica. Comunicamos un caso de enfermedad de Kimura, descubierto en un varón caucasiano, tras una biopsiaexcisional de una masa cervical

Kimura disease is a chronic inflammatory disorder mainlyinvolving Asians. It often presents as soft-tissue nodes or lymphadenopathy of the head and neck. Commonly associated with increased levels of eosinophils and IgE in peripheral blood. We report a case of Kimura disease in a Caucasian, diagnosed after lymph node and submaxillary gland removal

[Thoracic kaposiform hemangioendothelioma. Four consecutive cases with distinct outcome]. / Hemangioendotelioma kaposiforme torácico. Cuatro casos con evolución variable.

INTRODUCTION: Kaposiform hemangioendothelioma (KHE) is a rare, frequently congenital, neoplasm associated with the Kasabach-Merritt phenomenon (KMP) and predilection for the trunk. Its clinical course is unpredictable. A child with KHE can die as a result of hemorrhage or show early spontaneous regression. In addition various and concurrent therapies can be used to treat this tumor and it is difficult to predict which treatment will be successful. MATERIAL, METHODS AND RESULTS: We present four consecutive cases of KHE (larger than 20 cm) of the thorax. All patients developed KMP, without skin involvement in one patient. Patient 1, a neonate with KHE occupying both hemithoraces (nearly 50% of the body surface area), died shortly after birth due to coagulopathy and generalized bleeding. Patient 2, a neonate, was treated with interferon alpha-2a and showed accelerated correction of coagulopathy and complete tumoral regression at 9 months. Patient 3 showed no response to steroids, interferon or vincristine therapy. Thrombocytopenia (platelet count 40,000) persisted for 8 years and was resolved by administration of aspirin plus ticlopidine, without tumoral disappearance. Patient 4 underwent incomplete removal of the tumor on her right chest wall after showing no response to antiangiogenic therapy. The coagulopathy persisted and a second radical surgical procedure resolved KMP. CONCLUSION: Given the variable response to pharmacological treatment and the scant possibilities of surgical resection, the management of KHE with KMP must include a multidisciplinary approach. As little is known about the pathogenesis of these highly aggressive vascular tumors, further molecular research is needed to understand their long-term behavior.

Hemangioendotelioma kaposiforme torácico. Cuatro casos con evolución variable / Thoracic kaposiform hemangioendothelioma. Four consecutive cases with distinct outcome

Introducción. El hemangioendotelioma kaposiforme (HEK) es un raro tumor con frecuencia neonatal y asociado a coagulopatía de Kasabach-Merritt con preferencia por la localización en tórax y abdomen. Su evolución clínica es impredecible y puede causar la muerte por coagulopatía o evolucionan de forma rápida y espontánea. Por otra parte, no existe un tratamiento uniformemente eficaz con respuesta variable en cada caso a los diferentes agentes farmacológicos. Material, método y resultados. Se presentan 4 casos consecutivos de HEK de gran tamaño (> 20 cm) que afectaban al tórax y desarrollando trombocitopenia grave. En un paciente no había afectación cutánea. En el primer caso, un recién nacido con HEK que ocupaba todo el tórax y el abdomen (aproximadamente el 50 % de superficie corporal) falleció a las pocas horas de nacer por coagulopatía y hemorragia generalizada. El segundo recién nacido fue tratado con interferón α2a y experimentó una rápida resolución de la coagulopatía y una involución tumoral completa en 9 meses. El tercer paciente no respondió a los corticoides, el interferón y la vincristina y permaneció 8 años con trombocitopenia (40.000 plaquetas), la cual se resolvió con la administración de ácido acetilsalicílico y ticlopidina aun sin la desaparición del tumor. El cuarto paciente fue intervenido quirúrgicamente, por falta de respuesta antiangiogénica, mediante resección incompleta de la tumoración. La persistencia de la coagulopatía obligó a la extirpación radical de parte de la pared torácica en una segunda intervención. Conclusión. Dada la variable respuesta farmacológica y las escasas posibilidades de extirpación quirúrgica, el HEK que provoca coagulopatía de Kasabach-Merritt debe ser tratado en el contexto de un equipo multidisciplinar. Sólo los avances en el conocimiento de la biología molecular de la angiogénesis ayudarán a predecir el comportamiento de estos tumores a largo plazo

Introduction. Kaposiform hemangioendothelioma (KHE) is a rare, frequently congenital, neoplasm associated with the Kasabach-Merritt phenomenon (KMP) and predilection for the trunk. Its clinical course is unpredictable. A child with KHE can die as a result of hemorrhage or show early spontaneous regression. In addition various and concurrent therapies can be used to treat this tumor and it is difficult to predict which treatment will be successful. Material, methods and results. We present four consecutive cases of KHE (larger than 20 cm) of the thorax. All patients developed KMP, without skin involvement in one patient. Patient 1, a neonate with KHE occupying both hemithoraces (nearly 50 % of the body surface area), died shortly after birth due to coagulopathy and generalized bleeding. Patient 2, a neonate, was treated with interferon α -2a and showed accelerated correction of coagulopathy and complete tumoral regression at 9 months. Patient 3 showed no response to steroids, interferon or vincristine therapy. Thrombocytopenia (platelet count 40,000) persisted for 8 years and was resolved by administration of aspirin plus ticlopidine, without tumoral disappearance. Patient 4 underwent incomplete removal of the tumor on her right chest wall after showing no response to antiangiogenic therapy. The coagulopathy persisted and a second radical surgical procedure resolved KMP. Conclusion. Given the variable response to pharmacological treatment and the scant possibilities of surgical resection, the management of KHE with KMP must include a multidisciplinary approach. As little is known about the pathogenesis of these highly aggressive vascular tumors, further molecular research is needed to understand their long-term behavior

[Lymphoproliferative disorders of Waldeyer's ring]. / Linfomas primarios del anillo de Waldeyer.

OBJECTIVE: To investigate the incidence and characteristics of lymphoproliferative disorders of Waldeyer's ring in our pediatric patients. MATERIAL AND METHODS: We retrospectively reviewed 20 children under 14 years of age who underwent surgery in our department for adenoidectomy and/or tonsillectomy between 1 January 1996 and 30 November 2000. In non-immunocompromised children, surgical indication was the recent development of progressive unilateral tonsillar hyperplasia, and in immunocompromised patients indication was mainly the enlargement of adenoids and/or tonsils, although eradication of local Ebstein-Barr virus infection and recurrent acute tonsillitis were also indications. RESULTS: The mean age was 4.6+/-2.3 years (range: 16.9 months-13.9 years). Sixteen patients (80.0 %) were male. In the nine patients with unilateral hyperplasia, histopathological diagnosis was diffuse lymphoid hyperplasia. Of the 11 immunocompromised patients, 5 (45.5 %) had some type of lymphoproliferative disorder. DISCUSSION AND CONCLUSIONS: Lymphoproliferative disorders of Waldeyer's ring are relatively frequent in immunocompromised children who have undergone surgery of the adenoids and/or tonsils. Although no cases of unilateral hyperplasia of the tonsils were diagnosed in our series, tonsillectomy is indicated in patients with this diagnosis, independent of their immunological status.

Linfomas primarios del anillo de Waldeyer / Lymphoproliferative disorders of waldeyer's ring

Objetivo: Investigar la incidencia y características de los procesos linfoproliferativos del anillo de Waldeyer en pacientes pediátricos. Material y métodos: Se recogieron retrospectivamente 20 pacientes menores de 14 años intervenidos en nuestro servicio de adenoidectomía y/o amigdalectomía entre el 1 de enero de 1996 y el 30 de noviembre de 2000. La indicación en niños no inmunodeprimidos fue la aparición de una hiperplasia unilateral reciente y progresiva de amígdala y en el caso de inmunodeprimidos fue principalmente el crecimiento adeno y/o amigdalar, aunque también se intervinieron para erradicar infección focal por el virus de Epstein-Barr y por amigdalitis de repetición. Resultados: La media de edad fue de 4,6 ± 2,3 años (límites, 16,9 meses-13,9 años). Del total de los pacientes de nuestra serie, 16 fueron varones (80,0 %). El diagnóstico anatomopatológico de los 9 pacientes con hiperplasia unilateral de amígdala fue hiperplasia folicular linfoide. Por otro lado, en 5 de los 11 sujetos con inmunodeficiencias (45,5 %) se evidenció algún tipo de proceso linfoproliferativo. Discusión y conclusiones: Los procesos linfoproliferativos del anillo de Waldeyer son relativamente frecuentes en niños inmunodeprimidos intervenidos de adenoides y/o amígdalas. Aunque no se diagnosticó ningún caso en nuestra serie, consideramos conveniente operar a sujetos pediátricos con hiperplasia unilateral de amígdala, con independencia de su estado inmunológico (AU)

[New histopathologic data of prognostic value in extra-adrenal paragangliomas. Study of 9 cases]. / Nuevos factores histopatológicos de valor pronóstico en paragangliomas extraadrenales. Estudio de 9 casos.

BACKGROUND: The biological behavior of paragangliomas is difficult to evaluate by classic histological criteria thus justifying the use of immunohistochemical markers as prognostic factors. METHODS: Nine extra-adrenal paragangliomas (three jugulo-tympanic, four carotid-body tumors, and two retroperitoneal) were studied by conventional histological criteria, and also by chromogranin A and neuron-specific enolase (NSE) immunohistochemical staining for the study of chief cells, and S-100 as a marker of sustentacular cells. The rate of cell proliferation was studied by the proliferating cell nuclear antigen (PCNA). The correlation between these parameters and the clinical evolution of the neoplasms, which were classified as benign, locally aggressive, and malignant (with metastasis), were also analyzed. RESULTS: The atypia and the mitotic rate did not correlate with the behavior of the tumor. Less immunostaining with the anti-S-100 and anti-chromogranin A antibodies was observed in the malignant paragangliomas and in those which were locally aggressive. In the benign tumors the proliferative rate (PCNA) oscillated between 0.7% and 3.7%, and 40 or less PCNA positive cells were counted in 10 high-power field (HPF) (40x). In malignant and locally aggressive tumors the proliferative rate was 5% or more, with 60 or more cells that were positive for PCNA being found in 10 HPF. CONCLUSIONS: The histopathologic signs implying worse prognosis in extra-adrenal paragangliomas are a decrease in chromogranin A and S-100 immunoreactivity and a rate of cell proliferation of 5% or greater, or a number of cells stained for proliferating cell nuclear antigen greater than 50 in 10 high-power field.

Salivary duct carcinoma: a report of nine cases.

Nine cases of salivary duct carcinoma are presented. Eight lesions were located in the parotid gland and one in the submandibular gland. Tumor size was greater than 3 cm in four cases (44%), and facial paralysis was observed in five tumors (62.5%). Lymph node metastases were observed macroscopically or microscopically in seven patients. Perineural and lymphatic invasion were detected in eight and seven cases, respectively. Free surgical margins were obtained in eight of the nine patients (88%). Adjuvant postoperative irradiation was delivered in eight patients. Five patients died of disease in spite of the different clinicopathologic characteristics and aggressiveness of the surgery and irradiation. None of the parameters studied correlated with the final outcome.

[Infantile hamartoma of the chest wall]. / Hamartoma infantil de la pared costal.

Although chest wall osseous tumors are frequent in infancy, a few cases have been reported in the last years. Most of these cases had a benign course although in many of them a malignant histological diagnosis was formerly made. We report a case of chest wall tumor in a two months old boy. The histological pattern was similar to that described by McLeod and Dahlin as "chest wall hamartoma of the infancy". Seven months after the tumor resection the child lived well and without recurrence.