Bilateral metastases to the extraocular muscles from small cell lung carcinoma / Metástases bilaterais aos músculos extraoculares de um carcinoma de pequenas células do pulmão

ABSTRACT Bilateral orbital metastases restricted to the extraocular muscles (EOMs) are exceedingly rare. We report a case of bilateral extraocular muscle metastases from a small cell lung carcinoma and provide a review of the relevant literature. A 56-year-old smoker presented with proptosis, motility changes, and a relative afferent pupillary defect of the left eye, with a previous history of a small cell lung carcinoma. An orbital computerized tomography scan revealed a mass restricted to the left medial rectus. An incisional biopsy confirmed metastasis. Visual acuity of the left eye decreased rapidly, and right globe proptosis became evident. Orbital magnetic resonance imaging at two months follow-up showed marked left orbital mass enlargement and a new right lateral rectus mass. The patient was maintained on palliative care and died from metastatic disease-related complications.

RESUMO As metástases orbitárias bilaterais restritas aos mús­culos extraoculares são extremamente raras. Os autores apresentam um caso de metástases bilaterais, localizadas aos musculares extraoculares com base num carcinoma de pequenas células do pulmão e revisão da literatura relevante. Um homem, fumador, de 56 anos recorreu ao serviço de urgência por proptose, alterações de motilidade ocular extrínseca e um defeito pupilar aferente relativo do olho esquerdo, com história pessoal de carcinoma de pequenas células do pulmão. A tomografia computadorizada orbitária revelou uma massa restrita ao reto medial esquerdo. Uma biópsia incisional confirmou o diagnóstico de metástase. A acuidade visual do olho esquerdo diminuiu rapidamente e surgiu uma proptose do globo ocular direito. A ressonância magnética orbitária aos dois meses de seguimento revelou um aumento da massa orbitária esquerda e uma nova massa no reto lateral direito. O paciente foi mantido em cuidados paliativos e faleceu devido a complicações relacionadas com doença metastática.

Carcinoma neuroendocrino primario de vagina. Revisión de literatura a propósito de un caso / Primary neuroendocrine carcinoma of the vagina. Literature review on the subject of a case

RESUMEN El carcinoma primario de vagina representa 1 a 2% de los cánceres ginecológicos, siendo la diferenciación histológica neuroendocrina de células pequeñas extremadamente infrecuente, sólo se han reportado 28 casos en la literatura, describiéndose en orden de frecuencia en cérvix, endometrio, ovario, vagina y vulva. Se observa con más frecuencia en mujeres postmenopáusicas. Se presenta el caso de paciente femenino, de 39 años de edad, quien acude a la consulta por presentar secreción genital serohemática, fétida, de 1 mes de evolución, evidenciándose al examen físico lesión exofítica, friable, renitente, de aproximadamente 4 cm de diámetro, en tercio superior y cara posterolateral izquierda de vagina, por lo que se realiza biopsia excisional de dicha lesión, siendo el diagnóstico anatomopatológico carcinoma neuroendocrino de células pequeñas, grado histológico: 3. A propósito de este caso, se realiza una revisión del tema, haciendo hincapié en la importancia de la exploración ginecológica exhaustiva que incluya tomas de citologías y colposcopias periódicas, tomando en cuenta la vagina y así, realizar diagnóstico precoz en vista que el riesgo de carcinoma en dicha zona es infrecuente, mejorando el pronóstico y sobrevida de las pacientes.

ABSTRACT The primary carcinoma of the vagina represents 1 to 2 % of gynecologic cancers, being the histological differentiation neuroendocrine of small cells extremely infrequent, only 28 cases have been reported in the literature, describing in order of frequency: in the cervix, endometrium, ovary, vagina and vulva. It is most commonly seen in postmenopausal women. We present the case of a female patient, 39 years old, who comes to medical consult due to serohematic, fetid, genital discharge of 1 month of evolution, evidencing at physical examination an exophytic lesion, friable, renitent, with a diameter of approximately 4 cm, in the upper third and posterolateral left side of the vagina, for which an excisional biopsy of said lesion is performed, being the anatomopathological diagnosis neuroendocrine carcinoma of small cells, histological grade: 3. A bibliography reviewed was made, emphasizing the importance of exhaustive gynecological exploration with periodic cytology and colposcopy examinations, including the vagina for early diagnosis in view of carcinoma risk in this area is uncommon, improving the prognosis and survival of patients.

Carcinoma de células pequeñas de vejiga, a propósito de un caso / Carcinoma of bladder small cells: with regard to a case

Introducción: El carcinoma de células pequeñas de vejiga es una neoplasia poco frecuente que puede coexistir con un carcinoma urotelial. Ocurre principalmente en el sexo masculino en la séptima y octava décadas de vida. Macroscópicamente se observa como una masa sólida, polipoide y microscópicamente se conforma por células atípicas con núcleos hipercromáticos y una alta tasa mitótica. Caso: Paciente de 56 años, masculino, fumador que refiere que desde hace 6 meses presentó hematuria, con disuria y dificultad para orinar. Se realizó cistoscopía encontrándose lesión tumoral en vejiga por lo que se sometió a cistoprostatectomía radical. Discusión: El carcinoma vesical de células pequeñas es poco frecuente y puede coexistir con el carcinoma urotelial. El estudio anátomo patológico es fundamental para determinar el diagnóstico siendo necesarios marcadores de inmunohistoquímica como sinaptofisina, CD56, cromogranina y enolasa. La cirugía, quimioterapia y radiación se utilizan para dar un tratamiento multidisciplinario de la enfermedad.

Introduction: Small cell carcinoma of the bladder is a rare neoplasm, which can coexist many times with an urothelial carcinoma. It occurs mainly in males in the seventh and eighth decades of life. Macroscopically it is observed as a solid and polypoid mass and microscopically it is conformed by atypical cells with hyperchromatic nuclei and a high mitotic rate. Case: A 56-year-old man, smoker who has hematuria for six months, and dysuria and difficulty to urinate. In a cystocopy a bladder tumor is identified and a radical cistoprostectomy is performed. Discusion: Small cell vesical carcinoma is uncommon and may coexist with urothelial carcinoma. The anatomical pathological study is fundamental to determine the diagnosis, requiring immunohistochemical markers such as synaptophysin, CD56, chromogranin and enolase. Surgery, chemotherapy and radiation are used to provide a multidisciplinary treatment of the disease.

Small cell carcinoma of the bladder / Carcinoma de células pequenas de bexiga

Small cell carcinoma of the urinary bladder is an extremely aggressive and rare tumor. Even though small cell carcinoma most commonly arises from the lungs there are several reports of small cell carcinoma in extrapulmonary sites. Due to its low frequency there is no well-established management for this disease. We report the case of a 61 year-old man with small cell carcinoma of the bladder who underwent radical cystectomy following neoadjuvant chemotherapy. We also reviewed the literature for the optimal treatment strategy.

O carcinoma de células pequenas da bexiga urinária é um tumor extremamente agressivo e raro. Apesar desses tumores terem como sítio principal o pulmão, existem diversos relatos de carcinoma de pequenas células extrapulmonares. Pela baixa frequência, ainda não existe um tratamento bem estabelecido para essa neoplasia. Relatamos o caso de um homem de 61 anos de idade com carcinoma de células pequenas da bexiga urinária que foi submetido à quimioterapia neoadjuvante seguida de cistectomia radical. Fazemos ainda revisão na literatura em busca dos métodos de maior sucesso para o tratamento.

Tumor neuroendocrino en cérvix uterino: reporte de caso / Neuroendocrine tumor of the uterine cervix: case report

Los tumores neuroendocrinos de cuello uterino son extremadamente raros. Las mujeres con diagnóstico de carcinoma neuroendocrino de células pequeñas del cuello uterino tienen mayor frecuencia de metástasis en los ganglios linfáticos, invasión linfovascular, recurrencia y peor pronóstico en comparación con aquellos con otros tipos de neoplasias cervicales. Se presenta el caso de una mujer de 58 años, con un tiempo de enfermedad de seis años antes del ingreso, caracterizado por sangrado vaginal irregular posmenopáusica, además de sintomatología relacionada a anemia crónica. En el examen ginecológico, se evidenció tumoración de 4 cm que ocupaba tercio superior de vagina y protruía por el cérvix. Fue diagnosticado como mioma abortivo y enviada a estudio anatomopatológico. El resultado fue carcinoma neuroendocrino de células pequeñas grado III en el 90% y carcinoma epidermoide en el 10%. La paciente fue sometida a histerectomía radical más salpingo-ooferectomía bilateral y linfadenectomía pélvica bilateral y para-aortica. El estudio anatomopatológico de la pieza quirúrgica encontró endometrio y miometrio comprometido por neoplasia maligna. Parametrios, anexos y ganglios linfáticos se encontraron libres de neoplasia. A la microscopía el resultado fue carcinoma neuroendocrino grado III (carcinoma de células pequeñas, infiltrante), con extensa embolia linfovascular. El estudio de inmunohistoquímica arrojó sinaptofisina positivo en las áreas con diferenciación neuroendocrina.

Neuroendocrine tumors of the cervix are extremely rare. Women diagnosed with small cell neuroendocrine carcinoma of the cervix have a higher frequency of metastases in the lymph nodes, lymphovascular invasion, recurrence and worse prognosis compared to those with other types of cervical neoplasia. We report the case of a 58-year-old female, with a history of six years of postmenopausal irregular vaginal bleeding, in addition to symptoms related to chronic anemia. Gynecological examination showed a tumor of 4 cm that occupied the upper third of the vagina and protruded through the cervix initially diagnosed as an abortifacient myoma, and sent to histopathology study. 90% of the tumor was small cell neuroendocrine carcinoma grade III, and the remaining 10% was squamous cell carcinoma. The patient underwent into a radical hysterectomy plus bilateral salpingo-oophorectomy, and bilateral pelvic and para-aortic lymphadenectomy. Histopathologic examination of the surgical specimen found endometrium and myometrium compromised by malignancy. Parametrium, annexes and lymph nodes were free of neoplasia. At microscopy, the result was a grade III neuroendocrine carcinoma (small cell carcinoma, infiltrating), with extensive lymphovascular emboli. The immune-histochemical study showed synaptophysin positive in areas with neuroendocrine differentiation.

Tratamiento del carcinoma de células pequeñas del pulmón y supervivencia a 5 años / Treatment of small cell lung carcinoma survival at 5 years

Se reportaron los resultados de un estudio retrospectivo de pacientes con carcinoma de células pequeñas de pulmón en todos los estadios de la enfermedad, diagnosticado por citología y/o biopsia de la lesión. Métodos: Se estudiaron 68 enfermos que padecían un carcinoma de células pequeñas de pulmón en todos los estadios y recibieron tratamiento de primera línea con cisplatino-etopósido y de segunda línea, con ciclofosfamida, doxorubicina y vincristina. Los pacientes de enfermedad limitada fueron evaluados al final del primer ciclo para planificar el tratamiento con radiaciones; los de enfermedad extendida recibieron radiaciones sobre sitios metastásicos o para mejorar algún síntoma. Resultados: La respuesta al tratamiento fue: 25 por ciento de respuesta objetiva (RC y RP) y 56 por ciento de control de la enfermedad. La mediana de supervivencia global fue 10,10 meses. En el sexo femenino se observó tendencia a un incremento de la supervivencia en relación con el masculino (10,23 vs. 9,33 meses). La supervivencia en la enfermedad limitada fue de 12,5 meses y en la enfermedad extendida, de 6,57 meses lo cual fue estadísticamente significativo (p = 0,004).

The results of a retrospective study of patients with small cell lung carcinoma in all the stages of the disease diagnosed by cytology and/or biopsy of the lesion were reported. Methods: 68 patients that suffered from a small cell lung carcinoma in all the stages that received first-line treatment with cisplatin-etoposide, and second line treatment with cyclophosphamide, doxorubicin and vincristine were studied. The patients with limited disease were evaluated at the end of the first cycle to plan the treatment with radiations; those with extended disease received radiations on metastic sites or to improve some symptom. Results: The response to the treatment was as follows: 25 percent of objective response (CR and PR) and 56 percent of control of the disease. The global mean survival was 10.10 months. Among females, it was observed a trend towards an increase of survival compared with males (10.23 vs. 9.33 months). The survival of the limited disease was 12.5 months, whereas in the extended disease it was 6.57 months, which was statistically significant (p = 0,004).

Small cell carcinoma of the esophagus / Carcinoma de pequenas células do esôfago

BACKGROUND: Small cell carcinoma of the esophagus is a rare disease, characterized by aggressive progression, which is similar in appearance and behavior to its counterpart of the lung. It has a high incidence of metastatic disease at presentation and a poor prognosis. Treatment protocols are not well established because of the small number of cases and lack of large studies. CASE REPORT: 66 year-old woman, was admitted to the hospital with persistent dysphagia. Radiologic and endoscopic examination revealed an stenosing tumor in the lower third of the esophagus. The biopsy specimen was diagnosed as small cell carcinoma of the esophagus. The patient underwent an esophagectomy with esophagogastric anastomosis. CONCLUSION: The role of surgery remains controversial and the improvement of life quality is to be considered. Therapeutic approach is mainly esophageal resection.

RACIONAL: O carcinoma de pequenas células do esôfago é rara afecção, caracterizada por uma progressão agressiva, sendo seu aspecto e comportamento semelhantes aquele do pulmão. Detém elevada incidência de doença metastática em sua apresentação inicial, dado que lhe empresta prognóstico reservado na maioria dos casos. Protocolos de tratamento não são bem definidos, devido ao pequeno número de casos relatados na literatura. RELATO DE CASO: Apresenta-se caso de mulher de 66 anos admitida no hospital com queixa de disfagia. Avaliação radiológica e endoscópica revelaram lesão estenosante no terço inferior do esôfago, cuja biópsia mostrou tratar-se de carcinoma de pequenas células do esôfago. A paciente foi submetida à esofagectomia e reconstrução com tubo gástrico. CONCLUSÃO: O papel da cirurgia permance controverso e a melhora na qualidade de vida deve ser considerada. O tratamento terapêutico principal continua sendo a resecção esofágica.

Revisión sobre carcinoma vesical de células pequeñas / Small cell bladder carcinoma

Small cell carcinoma comprises 0.5%-0.7% of the total amount of malignant tumors from the urinary bladder. There are three main theories regarding its histogenesis: derivation from some particular neuroendocrine cells; metaplasia which starts from other high-grade malignant neoplasms; or stem cells origin. Clinical manifestations, imaging, and cistoscopy have no specificity. Three histopatological types have been described, sometimes coexisting with another different local neoplasm (usually transitional carcinoma). The anatomicopathological diagnosis relies on immunohistochemical techniques which detect the expression of several markers. Up to 94% and 56%- 67% of the cases present at diagnosis, muscular invasion and metastases, respectively. Mean five-year survival is 8%. Prognosis has been generally linked to the present clinical stage, although there is probability of pre-diagnosis micrometastases, even with apparent confined disease. Surgery without complementary therapy has provided unsatisfactory outcomes. The most generalized option is a combination of surgery plus cysplatinbased chemotherapy. Association of radiotherapy with antineoplastic agents (without radical surgery), as an attempt for bladder preservation, also seems to offer reliability

El carcinoma de células pequeñas supone 0.5% a 0.7% del total de los tumores malignos de la vejiga urinaria. Existen tres teorías principales acerca de su histogénesis: derivación a partir de determinadas células neuroendocrinas; metaplasia surgida a partir de otras neoplasias malignas de alto grado, u origen a partir de una célula madre pluripotencial. Las manifestaciones clínicas, pruebas de imagen y cistoscopia resultan inespecíficas. Se han descrito tres tipos histopatológicos, que coexisten a veces con otra neoplasia local diferente (generalmente carcinoma transicional). El diagnóstico anatomopatológico se apoya en técnicas inmunohistoquímicas de detección de la expresión de determinados marcadores. Hasta en un 94% y 56% a 67% de los casos presenta invasión muscular y metástasis, respectivamente, en el momento del diagnóstico. La supervivencia media a cinco años es del 8%. El pronóstico se vincula clásicamente con el estadio clínico presente, aunque existe probabilidad de micrometástasis previas al diagnóstico, incluso en enfermedad aparentemente confinada. La cirugía sin tratamiento complementario ha proporcionado resultados insatisfactorios. La opción más generalizada es la combinación de cirugía y quimioterapia basada sobre todo en el cisplatino. La combinación (sin cirugía radical) de radioterapia y administración de agentes antineoplásicos en un intento de preservación vesical parece ofrecer asimismo resultados satisfactorios

Sindrome de Eagle associado a carcinoma de pequenas células / Eagle syndrome associated to OAT cell carcinoma

Este trabalho trata de caso de síndrome de Eagle associada ao carcinoma de pequenas células em um paciente do sexo masculino, 58 anos. O objetivo deste estudo é relatar a associação destas doenças, visto que nenhum relato foi encontrado na literatura.

This work shows the syndrome of Eagle associated to the Oat cells carcinoma in a masculine patient with 58 years of age. The goal of this study is to report the association of such diseases, because no report was found in the literature.