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Resumen El tumor desmoplásico de célula redonda y pequeña (TDCRP) es una patología neoplásica maligna agresiva y poco común. Afecta predominantemente a hombres entre la segunda y tercera década de la vida. Los pacientes que la padecen tienen un pronóstico pobre, con una supervivencia global a 5 años de hasta el 30%. Por lo general se presenta como una masa en la cavidad abdominal, frecuentemente multifocal. Para su tratamiento se recomienda un enfoque multimodal con cirugía, quimioterapia y radioterapia. Poco más de 20 casos de TDCRP a nivel testicular/paratesticular se han reportado en la literatura. A continuación, se presenta un caso ilustrativo en esta localización, se discute el caso y se realiza revisión de la literatura.

Abstract Desmoplastic small round cell tumor (DSRCT) is an aggressive and rare malignant neoplasm. It mainly affects young men in their twenties and thirties. Patients with it have a poor prognosis, with a 5-year survival rate of up to 30%. It generally presents as a mass in the abdominal cavity, often multifocal. A multimodal approach is recommended for its treatment, with surgery, chemotherapy, and radiotherapy. Just over 20 cases of testicular/paratesticular DSRCT have been reported in the literature. Below, we present an illustrative case in this location, we discuss the case and review the literature.

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Paget's disease, described by Sir James Paget in 1874, is classified as mammary and extramammary. The mammary type is rare and often associated with intraductal cancer (93-100% of cases). It is more prevalent in postmenopausal women and it appears as an eczematoid, erythematous, moist or crusted lesion, with or without fine scaling, infiltration and inversion of the nipple. It must be distinguished from erosive adenomatosis of the nipple, cutaneous extension of breast carcinoma, psoriasis, atopic dermatitis, contact dermatitis, chronic eczema, lactiferous ducts ectasia, Bowen's disease, basal cell carcinoma, melanoma and intraductal papilloma. Diagnosis is histological and prognosis and treatment depend on the type of underlying breast cancer. Extramammary Paget's disease is considered an adenocarcinoma originating from the skin or skin appendages in areas with apocrine glands. The primary location is the vulvar area, followed by the perianal region, scrotum, penis and axillae. It starts as an erythematous plaque of indolent growth, with well-defined edges, fine scaling, excoriations, exulcerations and lichenification. In most cases it is not associated with cancer, although there are publications linking it to tumors of the vulva, vagina, cervix and corpus uteri, bladder, ovary, gallbladder, liver, breast, colon and rectum. Differential diagnoses are candidiasis, psoriasis and chronic lichen simplex. Histopathology confirms the diagnosis. Before treatment begins, associated malignancies should be investigated. Surgical excision and micrographic surgery are the best treatment options, although recurrences are frequent.

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Paget's disease, described by Sir James Paget in 1874, is classified as mammary and extramammary. The mammary type is rare and often associated with intraductal cancer (93-100% of cases). It is more prevalent in postmenopausal women and it appears as an eczematoid, erythematous, moist or crusted lesion, with or without fine scaling, infiltration and inversion of the nipple. It must be distinguished from erosive adenomatosis of the nipple, cutaneous extension of breast carcinoma, psoriasis, atopic dermatitis, contact dermatitis, chronic eczema, lactiferous ducts ectasia, Bowen's disease, basal cell carcinoma, melanoma and intraductal papilloma. Diagnosis is histological and prognosis and treatment depend on the type of underlying breast cancer. Extramammary Paget's disease is considered an adenocarcinoma originating from the skin or skin appendages in areas with apocrine glands. The primary location is the vulvar area, followed by the perianal region, scrotum, penis and axillae. It starts as an erythematous plaque of indolent growth, with well-defined edges, fine scaling, excoriations, exulcerations and lichenification. In most cases it is not associated with cancer, although there are publications linking it to tumors of the vulva, vagina, cervix and corpus uteri, bladder, ovary, gallbladder, liver, breast, colon and rectum. Differential diagnoses are candidiasis, psoriasis and chronic lichen simplex. Histopathology confirms the diagnosis. Before treatment begins, associated malignancies should be investigated. Surgical excision and micrographic surgery are the best treatment options, although recurrences are frequent.

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PURPOSE: Paratesticular sarcomas are rare and frequently reported as isolated case reports. Studies evaluating the relative frequency of the paratesticular sarcomas are limited, and to the best of our knowledge, this is the first study of paratesticular sarcomas in the Brazilian population. PATIENTS AND METHODS: Medical records of all patients undergoing treatment for paratesticular sarcomas between 1993 and 2006 were retrieved from the archives of our institution. RESULTS: Complete data from 12 patients (39 +/- 23 years, range 13-78) with paratesticular sarcomas were available, which represented 6.7% of all orchiectomies performed for testicular malignancies in the same period. At the time of diagnosis, 3 patients had retroperitoneal spread of the disease, all of which had elevated serum lactic dehydrogenase levels. The remaining 9 patients had normal serum markers. There were 6 rhabdomyosarcomas, 4 leiomyosarcomas, 1 liposarcoma and 1 undifferentiated sarcoma. Median follow-up was 31.4 months. Primary surgical excision by inguinal approach was performed in all cases (radical orchiectomy in 10 and preservation of the testis in 2). Retroperitoneal lymph node dissection was performed in 3 patients and excision of the hemiscrotum in 1. Eight patients received adjuvant chemotherapy. Mean overall survival time was 27.8 +/- 6.2 months after orchiectomy. CONCLUSION: Patients with paratesticular sarcomas are at high risk of disease progression, and systemic relapse remains a significant problem, determining poor prognosis. The high risk of local recurrence demands long-term follow-up, and intraoperative frozen section analysis might be of benefit. Elevated lactic dehydrogenase might also be a marker of retroperitoneal disease and poor prognosis. Improvement in survival requires effective systemic adjuvant therapy.

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El carcinoma de Verrucous en el área anogenital ha sido reportado bajo una variedad de nombres, incluyendo el condiloma acuminado gigante o tumor de Buschke-Loewenstein. Histológicamente, corresponde a un carcinoma espinocelular bien diferenciado. Se ha descrito una variedad de alternativas terapéuticas, siendo de primera elección la extirpación quirúrgica. El Imiquimod en crema al 5 por ciento representa un modificador tópico de la respuesta inmune. Actualmente está siendo utilizado en el tratamiento de una variedad de enfermedades cutáneas. Objetivo: Revisión del tema y presentación del primer caso de carcinoma verrucous anogenital tratado con cirugía shaving más criocirugía e imiquimod al 5 por ciento en crema con un seguimiento de siete años. Caso clínico: Paciente de sexo masculino, de 55 años, heterosexual, con antecedentes de lesión tumoral inguino-escroto-perineal derecha de 15 años de evolución y de crecimiento lentamente progresivo. Su primera consulta dermatológica se efectúa el 04.02.99. Se extirpa nódulo de tamaño mayor tamaño para examen histopatológico tipificación VPH por PCR (positivo para genotipos 6 y 11) Se diagnostica carcinoma espinocelular de bajo grado de malignidad. Se utilizaron en su tratamiento múltiples técnicas combinadas con métodos citodestructivos e inmunoterapia. Posteriormente se iniciaron ciclos de imiquimod 5 por ciento (una aplicación nocturna tres veces por semana) por seis semanas y luego ciclos de cuatro semanas cada uno, dos-tres veces al año. Se hizo un seguimiento a siete años, logrando erradicar exitosamente la enfermedad clínica y controlando las recidivas de forma satisfactoria.

Verrucous carcinoma of the anogenetal region has been reported under a series of names, including giant condyloma acuminatum or Buschke-Loewenstein tumor. Histologically, it corresponds to a well differentiated squamous carcinoma. A number of therapeutic alternatives have been described, the first choice being surgical removal. Imiquimod cream 5 percent represents a topical modifier of the immune response, and is currently being used in the treatment of a series of skin diseases. Objective: We present a review of the literature and the first case of anogenital verrucous carcinoma treated with a seven-year follow-up. Clinical case: A55-year-old man, with a history of a giant condyloma acuminatum with histological evidence of a low degree multiple techniques combined with cytodestructive methods and immunotherapy. A seven-year follow-up was carried out, success-fully eradicating the clinical disease and controlling recurrence with cycles of imiquimod cream 5 percent treatment (average 3 monthly treatment cycles per year).

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Interferon-alpha (IFN-alpha) has antitumor and antiangiogenic effects. The purpose of this work was to evaluate its efficacy and safety in the treatment of infancy hemangioma and to monitor the appearance of anti-IFN antibodies in these patients. Thirty-nine children (29 girls) aged 1.5-158 months, with 19 younger than 1 year and 9 older than 5, were treated with 3 x 10(6) IU/m(2) IFN-alpha 2b, subcutaneously (s.c.) daily. Inclusion criteria were life-threatening or life-limiting hemangioma and parents' informed consent. Regression was considered if tumor size diminished by 50% or more. Of the 38 patients who completed 6 months of treatment, 27 (71.1%) had regression and 11 (28.9%) had stable disease. No patient experienced progression. Regression was more frequent (100%) among patients between 1 and 5 years old, but it was particularly important (68%) among those under 1 year old, when spontaneous regression is rare. The main side effects were the IFN-related flulike syndrome (79%), increase in serum alanine aminotransferase (ALT) (28%), anorexia (19%), and mild inflammation at the injection site (19%). There was no effect on psychomotor or physical development. On the contrary, 1 patient with neurologic symptoms improved remarkably, including seizure disappearance. Eight patients developed anti-IFN-alpha 2 neutralizing antibodies, and 7 of them responded to IFN treatment. IFN-alpha 2b is a safe and efficacious treatment of infancy hemangioma. Further work should look for other treatment schedules and ways of administration and carefully monitor anti-IFN neutralizing antibodies, which does not seem to interfere with response.

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Os tumores de testiculo representam uma forma rara de neoplasia no sexo masculino. No periodo de 12 anos, foram atendidos 234 individuos portadores de tumor germinativo do testiculo, e destes, 173 foram seguidos no periodo pos-operatorio. Do total analisado, 137 eram seminomas que responderam satisfatoriamente ao tratamento e com sobrevida de 96 por cento em 3 anos, e 38 tumores nao-seminomatosos, cuja taxa de sobrevida foi de 84 por cento , no mesmo periodo. Dois pacientes apresentaram tumores em ambos os testiculos, de forma assincronica. O acompanhamento pos-operatorio medio foi de 36 meses

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Se analizaron 50 parejas que presentaban evidencia citológica y o clínica por Papiloma virus en el tracto genital. Las edades de las mujeres fluctuaron entre 18 y 52 años y la de los hombres entre 25 y 58 años. Se realizaron estudios de rutina, histológicos en las mujeres y citológicos en los hombres. Se trataron los casos con criocirugía, efudix o tratamiento mixto. Se destaca la importancia de la colposcopia ya que un porcentaje no despreciable (38%), no da información citológica. La asociación de HPV-NIE es frecuente (44%)

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