Tumor neuroectodermal melanótico de la infancia en los maxilares: reporte de dos casos / Melanotic neuroectodermal tumor of infancy on the jaws report of two cases

El Tumor Neuroectodermal Melanótico de la Infancia (TNMI) es un tumor raro que se presenta en infantes antes de un año de edad. Es un tumor de crecimiento rápido, que afecta el maxilar. En la mayoría de los casos es benigno. Reportamos dos casos atendidos en el Instituto Nacional de Enfermedades Neoplásicas. Los pacientes fueron 2 Infantes, de 4 a 7 meses de edad, en el maxilar inferior y superior respectivamente. En ambos casos se realizó resección quirúrgica del Tumor. El diagnóstico se corrobora con Inmunohistoquímica marcadores S-100 y Sinaptofísina y Panqueratina. La evolución fue favorable y ambos pacientes viven.

Melanotic Neuroectodermal Tumor of Infancy (MNTI) is a rare neoplasm occurring in infants before one year of age. It is a neoplasm of fast growth that affects maxilla. In most of the cases it is benign. We reported two cases attended at the Instituto Nacional de Enfermedades Neoplásicas. The patients were two infants, of 4 and 7 months old, in the mandible and maxilla respectively. In both cases the treatment was surgical excision. The diagnosis is corroborated with Inmunohistochemestry markers, S-100, Sinaptofisin and Pankeratin. The evolution was favorable and both patients are alive.

Epidemiology of the sarcomas of the jaws in a Peruvian population

Objective: Analysis of the clinical characteristics of patients with Sarcomas of the Jaws treated in the "Instituto Nacional de Enfermedades Neoplasicas. Dr. Eduardo Caceres Graziani" from 1952-2007.Study Design: Review of 155 clinical records of patients with Sarcomas of the Jaws and record of age, gender, size, location, clinical symptoms and signs, histopathological diagnoses and type of treatment. The data obtained were analyzed by means of Student’s statistical t-test, Fisher and Friedman’s test. Results: Analysis of 155 Sarcomas of the Jaws. The average age of patients was 36.8 years old (range: 1-80 years);the female gender was the most frequent (52.9%); the average tumor size was 5.5 cm; in upper jaw 54.84% occurred and 45.16% in the lower jaw; the predominant sign was facial asymmetry (87.74%) and the predominant symptom: pain (63.23%). The most frequent diagnosis was Osteosarcoma 50.3% followed by Chondrosarcoma18%. Surgery plus radiation therapy was the treatment type of choice with 21.94% of cases. Conclusion: The results of this study demonstrate the delayed diagnosis and facial asymmetry and pain appear as the most important events for the diagnosis of Sarcomas of the Jaws (AU)

Malignant melanoma of the oral cavity. Review of the literature and experience in a Peruvian Population

Objective: To determine the epidemiological profile of malignant melanoma cases treated at the National Institutefor Neoplastic Diseases "Dr. Eduardo Caceres Graziani" (INEN) over the period 1952 to 2008.Study Design: All clinical records with complete data of patients presenting a histopathological diagnosis of malignant melanoma of the oral cavity were reviewed. Data such as age, gender, location, tumor size, disease length, presence of metastasis, treatment received and year of admission were recorded. Results: During the study period 97 cases were found. The average age of patients was 52.85±1.6 years old mostly between 50 and 59 years old; the predominant gender was the female. The most common location was the palate and there was 58.8% of cases with a tumor size bigger than or equal to 4 cm. The length of the disease in 38.1% of the cases was longer than a year and in great part of the cases (69.1%) there was no metastasis. The treatment of choice was the surgery plus radiotherapy in 38.1% of the cases. According to the admission date it was also noted that the number of cases is increasing. Conclusion: The results of this study demonstrate a late diagnosis and an increasing frequency of this neoplasia in the oral cavity (AU)

Carcinoma gástrico precoz tipo superficial (Spreading carcinoma). Correlación clínico-patológica de 33 casos / Superficial type early gastric carcinoma (Spreading carcinoma). Clinicopathological correlation of 33 cases

Objetivo: Estudio de los hallazgos clínico-patológicos de 33 pacientes con cáncer gástrico precoz de tipo superficial (spreading type). Resultados: Es más frecuente el tipo IIc o deprimido. La incidencia de metástasis en ganglios linfáticos fue de 27% (9/33). La invasión de la submucosa ocurrió en 67% (22/33). Conclusiones: El tipo IIc es el más frecuente de los Carcinomas Gástricos Precoces Superficiales. Todos los casos son carcinomas de Células en Anillo de Sello. Se recomienda un buen manejo del margen quirúrgico.

Objetive: 33 patients with superficial spreading type of early gastric cancer. We study the clinicopathological features. Results: The IIc type macroscopic lesion occurred most frequently. The incidence of lymph node metastasis was 27% (9/33). The submucosal invasion was 67% (22/33). Conclusions: Type IIc occurred more frequently in the superficial spreading cancer. All cases were signet ring cell carcinoma. It is recommended a safe surgical margin.

Teratoma inmaduro del testículo en niños, Instituto Nacional de Enfermedades Neoplásicas / Immature teratoma of the testis in children Instituto Nacional de Enfermedades Neoplasicas

Presentamos 8 pacientes con Teratomas Inmaduros Puros del Testículo en niños. Son tumores de testículo en niños, poco frecuentes. La elevación de la alfafetoproteína mayor de 100 ng/dL indica la presencia de focos de tumor del seno endodermal. Se sugiere que en grupos pediátricos la gradación de teratoma inmaduro no es necesaria.

We presented 8 patients with pure inmatures Teratomas of Testis in childrens. They are unusual Testis tumors in children. The elevation of serum alfa fetoprotein greater than 100 ng/dL indicate the presence of foci of yolk sac tumor. We suggest that in pediatric age group, grading of Inmature Teratoma is unnecessary.

Sarcoma de Kaposi del estómago asociado a VIH: Presentación de una caso y revisión de la literatura / Kaposi Sarcoma of the stomach associated with HIV: Making a case and review of the literature

Presentamos un paciente con SIDA y Sarcoma de Kaposi del estómago sin compromiso de la piel. El caso corresponde a un varón de 48 años de edad, con dolor de estómago, llenura precoz, dispepsia y pérdida de peso. La gastroscopía mostró lesiones elevadas con ulceración central. Las biopsias mostraron Sarcoma de Kaposi con marcadores positivos para CD34, CD117 y Ki 67. El paciente recibe tratamiento Antiretroviral de gran actividad. Un control posterior al completar el tratamiento, las biopsias fueron negativas.

We presented a patient with AIDS and Stomach KaposiÆs Sarcoma without skin disease. The case correspond a 48 years old male with Stomach pain, early feeling full, dyspepsia, and lost of weight. The gastroscopy showed elevated lesions with central ulceration. The biopsies showed sarcoma of Kaposi with CD34, CD117 and Ki 67 positive markers. The patient receives antiretroviral of big activity treatment. The biopsies were negative after he completed the treatment.

Disgerminoma de ovario asociado a gestación y síndrome de Pseudo Meigs: Reporte de un caso y revisión de la literatura / Ovarian dysgerminoma associated with pregnancy and Pseudo Meigs syndrome: A case report and literature review

Asociados al embarazo, los tumores de ovario más frecuentes son benignos como el teratoma quístico y el cistoadenoma seroso. Los tumores malignos más frecuentes son el carcinoma de ovario y el de bajo grado de malignidad seguido por el disgerminoma. El síndrome de Meigs se define como la presencia de ascitis e hidrotórax asociado a fibroma o tecoma ovárico. También se ha descrito en tumores malignos, entre ellos el disgerminoma definiéndose en estos casos síndrome de pseudo-Meigs. Se presenta el caso de una paciente de 26 años con gestación de 27 semanas que presentó un tumor sólido de ovario derecho asociado a ascitis e hidrotórax derecho. Fue operada y el resultado anatomopatológico fue disgerminoma. Una revisión de los aspectos clínicos, diagnósticos y terapéuticos de estos tumores, es presentada.

Associated with pregnancy, most common ovarian tumors are benign as cystic teratoma and serous cystadenoma. The most common malignant tumors are ovarian carcinoma and low grade malignancy followed by dysgerminoma Meigs syndrome is defined as the presence of ascites and hydrothorax associated with ovarian fibroma or thecoma. Has also been reported in malignant tumors, including dysgerminoma defined in these cases pseudo-Meigs syndrome. The case of a patient of 26 years with gestation of 27 weeks showed a solid right ovarian tumor associated with ascites and right hydrothorax. She was operated and the pathological result was dysgerminoma. A review of clinical aspects, diagnosis and treatment of these tumors is presented.

Tumor de células esteroideas de ovario: reporte de un caso y revisión de la literatura / Steroid cell tumor of the ovary: a case report and literature review

El tumor de células esteroideas de ovario es una neoplasia poco frecuente que se origina del estroma o de los cordones sexuales y es potencialmente maligno. La mayoría de estos tumores producen hormonas esteroides siendo la testosterona la que se eleva frecuentemente. Se presenta el caso de una paciente de 30 años que presentó signos de virilización asociado a masa pélvica, ascitis e incremento de la testosterona. Fue operada y sometida a estadiaje de ovario. El resultado del estudio anatomopatológico fue tumor de células esteroideas. Una revisión de los aspectos clínicos, diagnósticos y terapéuticos de estos tumores, es presentada.

Steroid cell tumor of the ovary is a rare malignancy originating from the stromal or sex cord and is potentially malignant. Most of these tumors produce steroid hormones testosterone being which arises frequently. We report a case of a patient 30 who showed signs of virilization associated with pelvic mass, ascites and increased testosterone. She was operated and subjected to ovarian cancer staging. The result of the pathology was steroid cell tumor. A review of clinical aspects, diagnostic, and treatment of these tumors, is presented.

Factores de sobrevida en 152 pacientes con tumores estromales gastrointestinales / Survival factors in 152 patients with gastrointestinal stromal tumors

INTRODUCCIÓN: El tumor estromal gastrointestinal (GIST), es la designación para un tipo específico de tumor mesenquimal del tracto digestivo que proviene de las células intersticiales de Cajal (ICC) o de sus precursores multipotenciales, es de presentación infrecuente y por ello, su mejor conocimiento deberá incidir en un adecuado manejo diagnóstico y terapéutico. OBJETIVOS: Valorar las características clínicas e identificar los factores que influencian la sobrevida de los pacientes con diagnóstico de tumor estromal gastrointestinal. MATERIAL Y MÉTODOS: El presente estudio es de diseño cuantitativo, no experimental, descriptivo, retrospectivo y transversal. Ha sido realizado en 152 pacientes con diagnóstico de tumor estromal gastrointestinal que fueron atendidos en el Instituto Nacional de Enfermedades Neoplásicas (INEN), Lima-Perú, desde Enero del año 1999 hasta Diciembre del año 2009. Para el diagnóstico definitivo se consignó el resultado de la pieza quirúrgica y la histología se complementó con test de inmuno histoquímica. Para valorar la distribución normal de la población se usaron las pruebas de Shapiro-Wolk, Anderson-Darling, Lilliefors, respecto a la estadística inferencial se usó tablas de supervivencia y para valorar la significancia en el análisis univariado (p<0.05 significativa), se usaronlas pruebas de Wilcoxon, Tarone-ware y Log-rank, además para evaluar la diferencia entre grupos en las tablas de contingencia se utilizó el chi cuadrado y el test de Fisher. El análisis multivariado se realizó utilizando el modelo de riesgo proporcional de Cox.RESULTADOS: El grupo de 152 pacientes comprendió 78 mujeres (51%) y 74 varones (49%) con rango etáreo de 16 a 92 años, con una edad promedio de 54 años. La frecuencia de presentación se incrementó a partir de la cuarta década de vida y alcanzó su mayor expresión entre los 50 y 70 años. La presentación inicial fue de enfermedad localizada para 79 pacientes (52%) y con metástasis primaria...

INTRODUCTION: The gastrointestinal stromal tumor (GIST) is the designation for a specific type of mesenchymal tumor of the digestive tract that is origin in the interstitial cells of Cajal (ICC) or its precursor multipotentials, its presentation is rare and therefore its best knowledge must affect a proper diagnosis and treatment. OBJECTIVES: To evaluate the clinical characteristics and to identify factors influencing survival of patients with gastrointestinal stromal tumor. MATERIAL AND METHODS: This study design is quantitative, non experimental, descriptive, retrospective and transversal. The study has been performed in 152 patients with gastrointestinal stromal tumor who were treated at the National Institute of Neoplastic Diseases (INEN), Lima, Peru, from January 1999 through December 2009. For the final diagnosis was registered the outcome of the surgical and histology was complemented by immunohistochemical test. To assess the normal distribution of the population was used the Shapiro-Wolk, Anderson-Darling, Lilliefors, regarding the use of inferential statistical tables for survival and to assess its significance in the univariate analysis (p<0.05 significance), was used the Wilcoxon test, Tarone-Ware Log-rank and also to evaluate the difference between groups in contingency tables used the chi square and FisherÆs test. Multivariate analysis was performed using the proportional hazards model of Cox. RESULTS: The group of 152 patients included 78 women (51%) and 74 men (49%) with age range from 16 to 92 years, with an average age of 54. The frequency presentation was increased from the fourth decade of life and reaches its highest expression between 50 and 70. The initial presentation was with localized disease to 79 patients (52%) and primary metastases in 73 patients (48%), with an average time of disease 14 months. The prevalence of GIST tumor in the differents organs was as follows: stomach with 77 patients (50.65%), jejunum with 21 patients...

Metástasis de carcinoma de estómago que simula cáncer primario de ovario: importancia de la biopsia por congelación / Metastasis of carcinoma of stomach cancer simulating primary ovarian tumor: importance of frozen biopsies

Objetivo: Importancia de la Bíopsia por congelación en el diagnóstico y manejo de las pacientes con tumores de ovario. Resultados: Comunicamos nueve casos de pacientes con tumores de ovario.El carcinoma primario se encontró durante la operación programada para resección del tumor de ovario.En estos casos durante el acto operatorio y por la biopsia por congelación se hizo diagnóstico de tumor de krukenberg y el carcinoma primario se encontró en el estómago. Conclusiones: El resultado del estudio de nueve pacientes con carcinoma gástrico que simulaba cancer primario de ovario, muestra la importancia de la biopsia por congelación y la comunicación con los médicos cirujanos durante el acto operatorio.

Objetive: The importance of frozen section in the Diagnosis and manegment of patients with ovarian tumors. Results: We report 9 cases of patients with ovary tumors. The primary carcinoma was found during an operation for the ovarian tumor. In these cases the diagnosis of krukenberg tumor was made intraoperatively by frozen section evaluation and the primary carcinoma were found in the stomach. Conclusions: The results of the study of 9 patients with metástasis from gastric carcinoma simulating primary carcinoma of the ovary show us the importance of frozen section and the comunication with the surgeon intraoperatively.

Quiste epidermoide de testículo controversias de su origen histológico: correlación clínico patológica / Testicular epidermoid cyst originated disputes histológico: correlación clinical pathology

Presentamos 6 pacientes con Quistes Epidermoides de Testículo. Es una neoplasia benigna. Patológicamente se encuentra bien delimitada y encapsulada. Al corte se presenta como una masa blanquecina con material laminado, pastoso, blanco amarillento. En resumen, es un tumor poco frecuente, intraparenquimal, quístico sin componentes teratomatosos. Sugerimos sólo resección parcial del tumor sobre todo en pre-púberes.

We presented 6 cases of Epidermoid cyst of the testis. ItÆs a benign neoplasia. Pathological evaluation consisted of encapsulated neoplasia, sharply delineated. The sectioned surgical specimen show a white mass with laminated white-yellow pastelike material. In conclusion is a rare bening tumour. It is intraparenchymal testicular cyst without teratomatous components. We suggest organ preserving surgery, in prepuberal ages.

Quiste dermoide de testículo evidencias de su separación del quiste epidermoide, teratoma maduro y teratoma del adulto / Testicular dermoid cyst evidence of separation epidermoid cyst, mature teratoma and teratoma in adults

Presentamos 3 casos de Quistes Dermoides del Testículo. Es una entidad clínica rara. La ausencia de neoplasia germinal intratubular demuestra su historia natural benigna. Microscópicamente se caracteriza por tener folículos pilosos con glándulas sebáceas con una orientación cutánea hacia una superficie epidermal. Es diferente del Quiste epidermoide y del Teratoma maduro en especial en pacientes postpuberales por su significativa asociación con metástasis. También llamado Teratoma del adulto.

We presented 3 cases of Dermoid Cyst of the Testis. It is a rare clinical entity. The absence of intratubular germ cell neoplasia (IGCNU) demonstrated their benign natural history. Microscopic examination had hair folicles with sebaceous glands showing a typical, cutaneos-type orientation to an epidermal surface. It is distinct from epidermoid cyst and from mature teratoma in especial of postpuberal patients because of its significant association with metastases. Adult teratoma.

Carcinoma gástrico precoz originado en adenoma tubular estudio clínico-patológico / Early gastric carcinoma arising in tubular adenoma clinicopathologic study

Objetivo: Estudio clínico patológico de casos de pacientes con cáncer gástrico precoz originado en adenoma tubular. Resultados: De 332 casos de pacientes con cáncer precoz de estomago 65.6% (218/332) son adenocarcinomas diferenciados, tubulares, papilares o de tipo intestinal. 28.8% (63/218) se originan de adenoma tubular. La gran mayoría son de tipo I, IIa y combinados con IIc y IIb. Es importante el tamaño del tumor. Mas de 2 cm hay infiltración de la submucosa y metástasis. Conclusiones: Cáncer gástrico precoz originado en adenoma tubular, menores de 2 cm e intramucosos con ecoendoscopia deben ser resecados endoscópicamente.

Objective: Clinic pathologic study of patients with early gastric cancer originated in tubular adenoma. Results: From 332 patients with early gastric cancer 65.6% (218/332) are well differentiated adenocarcinomas tubular papilar, or intestinal type. 28.8% (63/218) are originated from tubular Adenoma. The mayority are I, II a or Combination with II c and II b type. It is important the size of tumor. More than 2cm had Infiltration of submucous and metastasis . Conclusions: Early gastric cancer originated in Adenoma tubular less than 2 cm intramucous with echoendoscopy may be resected endoscopically.

[Survival factors in 152 patients with gastrointestinal stromal tumors]. / Factores de sobrevida en 152 pacientes con tumores estromales gastrointestinales.

INTRODUCTION: The gastrointestinal stromal tumor (GIST) is the designation for a specific type of mesenchymal tumor of the digestive tract that is origin in the interstitial cells of Cajal (ICC) or its precursor multipotentials, its presentation is rare and therefore its best knowledge must affect a proper diagnosis and treatment. OBJECTIVES: To evaluate the clinical characteristics and to identify factors influencing survival of patients with gastrointestinal stromal tumor. MATERIAL AND METHODS: This study design is quantitative, non experimental, descriptive, retrospective and transversal. The study has been performed in 152 patients with gastrointestinal stromal tumor who were treated at the National Institute of Neoplastic Diseases (INEN), Lima, Peru, from January 1999 through December 2009. For the final diagnosis was registered the outcome of the surgical and histology was complemented by immunohistochemical test. To assess the normal distribution of the population was used the Shapiro-Wolk, Anderson-Darling, Lilliefors, regarding the use of inferential statistical tables for survival and to assess its significance in the univariate analysis (p <0.05 significance), was used the Wilcoxon test, Tarone-Ware Log-rank and also to evaluate the difference between groups in contingency tables used the chi square and Fisher's test. Multivariate analysis was performed using the proportional hazards model of Cox. RESULTS: The group of 152 patients included 78 women (51%) and 74 men (49%) with age range from 16 to 92 years, with an average age of 54. The frequency presentation was increased from the fourth decade of life and reaches its highest expression between 50 and 70. The initial presentation was with localized disease to 79 patients (52%) and primary metastases in 73 patients (48%), with an average time of disease 14 months. The prevalence of GIST tumor in the different organs was as follows: stomach with 77 patients (50.65%), jejunum with 21 patients (13.82%), retroperitoneum with 17 patients (11.18%), duodenum 11 patients (7.24%), colon 11 patients (7.24%), ileum 8 patients (5.26%),pancreas, 3 patients (1.97%), rectum, 3 patients (1.97%) and esophagus with 1 patient (0.66%). The most common symptoms of GIST tumors in general were gastrointestinal bleeding, abdominal tumor and abdominal pain. There are organ-specific symptoms such as jaundice in pancreas, dysphagia in esophagus and obstruction in the ileum. The tumor size greater than 10 centimeters was found in 92 patients (60.51%), 39 patients had size between 5 and 10 centimeters (25.65%) and 20 patients had lesions smaller than 5 centimeters (13.15%). Immunohistochemistry tests performed in 75 patients show that for all locations, the expression of KIT (CD117) is 94.8%, followed by CD34 to 70.35%; on the other hand, actin (61.68%) and S-100 (57.56%) have a smaller range of expression. Retroperitoneal GIST tumors had an expression of CD117 of 92.86% and CD34 of 60%, and GIST tumors of the pancreas had an expression of CD117 of 100% and CD34 of 100%. We evaluated 27 patients with low mitotic index, of which 10 had primary metastases (37%), in turn, of 25 patients with high mitotic index, 8 had primary metastases (32%). Of the 152 patients, 93 had complete resection of the disease, 28 had partial resection, 24 were unresectable and 07 did not undergo surgery, the more aggressive behavior was observed in ileum, 03 patients were unresectable, 02 patients had partial resection and only 02 could be completely resected, the rest of the series in general, for each location, the GIST tumors completely resected outscored the unresectable and partially resected. Of 93 patients that had completely resected, recurrence was found in 32 of these patients (34.4%), recurrence was local in 8 patients, metastases in 18 patients and local recurrence + metastases in 6 patients, with an average time of recurrence 22 months. The overall cumulative survival at 5 years was 81.35%. The survival of patients under and over 50 expressed a p = 0.08, cumulative survival rates by tumor size expressed p = 0.56, cumulative survival rates for stomach and intestinal location shows a p = 0.056. The 5-year survival of completely resected patients was 87.70%. Overall survival of patients with and without metastasis expressed p = 0.001, the cumulative survival function completely resected patients, the resected and partially resected, expresses with p <0.0001. Multivariate analysis showed that the most significant factor for disease progression was the primary metastases with p = 0.007, and that survival was directly related to complete resection of the disease which is expressed with p <0.0001. CONCLUSIONS: The most important prognostic factor of survival for gastrointestinal stromal tumors (GIST) is the complete resection of the disease. The factor that is associated with progression of the disease is the presence of metastases. In our series of 152 patients, tumor locations tend to relate better survival in gastric GIST that in intestinal GIST. Similarly, we found a tendency to express a lower survival in patients younger than 50 years. Differentiated tumor size in three size categories expressed no more related to survival. The low mitotic index associated with metastasis, not reflected a good prognosis of disease.

[Metastasis of carcinoma of stomach cancer simulating primary ovarian tumor. Importance of frozen biopsies]. / Metástasis de carcinoma de estómago que simula cancer primario de ovario. Importancia de la biopsia por congelación.

OBJECTIVE: The importance of frozen section in the Diagnosis and management of patients with ovarian tumors. RESULTS: We report 9 cases of patients with ovary tumors. The primary carcinoma was found during an operation for the ovarian tumor. In these cases the diagnosis of krukenberg tumor was made intraoperatively by frozen section evaluation and the primary carcinoma were found in the stomach. CONCLUSIONS: The results of the study of 9 patients with metastasis from gastric carcinoma simulating primary carcinoma of the ovary show us the importance of frozen section and the communication with the surgeon intraoperatively.