Tumor neuroectodérmico maligno del tracto gastrointestinal: a propósito de un caso / Malignant neuroectodermal tumor of the gastrointestinal tract: case report

El tumor neuroectodérmico maligno del tracto gastrointestinal es una neoplasia rara con pocos casos reportados en la literatura, especialmente en América Latina. Descrito por primera vez en 2003, se trata de una entidad sin tratamiento estandarizado y de pobre pronóstico. Se presenta el caso de una paciente de 22 años de edad que acude a la consulta por dolor abdominal, anemia y masa abdominal palpable. Luego de estudios pertinentes se decide la conducta resectiva y el posterior tratamiento oncológico. (AU)

Malignant gastrointestinal neuroectodermal tumor (GNET), formerly known as clear cell sarcoma of the gastrointestinal tract, is an extremely rare tumor of mesenchymal origin, which presents great microscopic and molecular similarity to clear cell sarcoma found in other parts of the body, such as tendons and aponeurosis. It is characterized by its rapid evolution, high recurrence rate and frequent diagnosis as metastatic disease.1,2 (AU)

Tumor de partes blandas de bajo potencial maligno: caso clínico y revisión de la literatura / Soft tissue tumor of low potential malignancy: clinic case and review of literature

El tumor de células gigantes de partes blandas de bajo potencial de maligno, es una neoplasia poco frecuente, clasifica dentro de las lesiones fibrohistiocíticas. Histológicamente es un tumor con hallazgos idénticos al tumor de células gigantes del hueso. Presentamos un caso correspondiente a esta neoplasia. Se trató de paciente de 26 años con una lesión tumoral 20 cm x15 cm x15 cm, en ambas regiones lumbares y región sacra. El estudio histológico reveló una neoplasia con abundantes células gigantes, células fusiformes, hemosiderina y hueso metaplásico. Los estudios inmunohistoquímicos demostraron fuerte positividad de CD68 para las células osteoclásticas. La evolución del paciente fue favorable, sin evidencia de recidivas. Es imprescindible realizar el diagnóstico diferencial de este tumor con otras neoplasias con abundantes células gigantes, como el tumor de células gigantes de la vaina tendinosa y el fibrohistiocitoma maligno rico en células gigantes, el cual es un sarcoma de alto grado

The giant cell tumor of soft tissues of low potential malignancy is a very rare tumor. It’s classified in fibrohistiocytic neoplasm and has features identical to giant cell tumor of bone. We present a clinical case for this less frequent malignancy. Patient 26 years old man with a tumor of 20 cm x 15 cm x 15 cm, in both lumbar and the sacral regions. Histological examination revealed a neoplasm with abundant giant cells, spindle cells, hemosiderin and metaplastic bone. The immunohistochemistry studies practice showed strong positivity of CD68 for the osteoclastic cells. The patient outcome was favorable, without evidence of recurrence. It is essential to make the differential diagnosis of this kind of tumor with other neoplasm with abundant giant cells, such as the giant cell tumor of the tendon sheath and malignant giant cell, also malignant fibrous hystiocitoma, which is rich in giant cells and high grade sarcoma

Fibrohistiocitoma maligno en pared abdominal: reporte de un caso / Malignant fibrohystiocytoma of the abdominal wall: a case report

El fibrohistiocitoma maligno es el sarcoma de partes blandas más común en la edad adulta, en un grupo etario mayor de 50 años y afecta principalmente a hombres. Estos tumores mesenquimales, con sus distintas variedades morfológicas, precisan de un análisis inmunohistoquímico para su diagnóstico diferencial, fundamentalmente con el carcinoma sarcomatoide, el tumor miofibroblástico inflamatorio y el leiomiosarcoma. Macroscópicamente son neoplasias voluminosas, con múltiples áreas de necrosis en la superficie de corte; desde el punto de vista microscópico el tumor muestra una proliferación desordenada de células fusocelulares, con un patrón esteliforme y/o presencia de células multinucleadas con atipia, mitosis atípicas y un estroma que muestra gran cantidad de colágeno así como un número variable de células inflamatorias mononucleadas e histiocitos espumosos. Su diagnóstico es realizado en base a su morfología e inmunohistoquímica. Presentamos el caso de un paciente masculino de 54 años y la forma como se manejó en nuestro departamento.

The malignant fibrohystiocytoma is the most frequent soft tissue sarcoma in the adult age, in an age group mayor of 50 years old, and affect principal to the man. These mesenquimal tumors with distinct morphological varieties need an immunohistochemistry analysis for his differential diagnostic, principal with the sarcomatoide carcinoma, the miofibroblastic inflammatory tumor and the leiomiosarcomas. Macroscopically there are bulky neoplasm with multiples necrosis areas in the superficial incision, of the microscopically point of view, the tumor show a no order proliferation of the fusocellular cells, with a esteliform patron and or the presence of multinucleate cells with atypical mitosis and a stroma, with show a big quantities of collagen and a variety numbers of inflammatory cells mononucleotic and spumoni hystiocyts. The diagnostic is realized in base to his morphologic and the immunohistochemistry. We present the case of a male patient 54 years old and the form of his management.

Renal tumors presentation: Changing trends over two decades.

Purpose: We have analyzed the changing trends in surgical treatment of renal tumors over the last 2 decades with regard to age incidence, presentation, incidental detection, and histopathology. Materials and Methods: Records of renal tumors were analyzed from January 1, 1988 to December 31, 2007. Data were split into 4 parts based on a 5-year time period, 1 for each cohort of patients: cohort 1 (1988-1992)-103 patients, cohort 2 (1993-1997)-161 patients, cohort 3 (1998-2002)-243 patients, and cohort 4 (2003-2007)-304 patients. A comparative study was performed with regard to age incidence, presentation, incidentallomas, histopathology, and management with statistical analysis. Results: Out of 811 renal tumors, 17.63% cases were benign and 82.37% were malignant. In the first cohort, 34.95% cases were detected in the seventh decade as compared with cohort 4 in which these were detected in the sixth (34.86%) and fifth decades (21.38%). Incidentallomas increased from 11 (10.67%) in cohort 1 to 84 (27.63%) in cohort 4 (P = 0.001). The cases of surgically treated tumors increased in number from 103 to 304 in cohort 4. Among the presenting features, incidence of weight loss, flank pain, and lump decreased while other clinical syndromes were constant. Only open radical nephrectomy was performed in the first 2 consecutive timeperiods. Laparoscopic radical nephrectomy was increasingly used in cohort 4 as compared with cohort 3 (121 vs 32, respectively). Similarly, open nephron sparing surgery (NSS) was increasingly used in cohort 4 as compared with cohort 3. Among the histopathologies, clear cell carcinoma was most common (73.35 %), but Fuhrman grading showed a trend toward more cases detected with grade 1 and 2 in cohort 4; 23.73% and 61.86%, respectively, as compared with 15.85% and 45.12% in cohort 1 (P = 0.001); more T1 tumors were detected (63.42% in cohort 4 as compared with 41.46% in cohort 1). Conclusions: A majority of renal tumors presented as symptomatic tumors. Recently, tumors are being detected at an early stage and grade; in the younger patients, with an increasing trend of laparoscopic and open NSS.

Review of the clinical features of skin cancer: a study of 1, 048 cases from a hospital in West China

Skin cancer [SC] is a group of malignancies which include primary and metastatic tumors which involve the skin and its appendages. Up to the present, only a few studies on the clinical features and the trend of S have been reported but the status in West China is still undetermined. The S cases were from a major hospital in West China. A total of 1048 cases from 1981 to 2006 were included in our study. The clinical features of S including age, gender, lesion location and pathological diagnosis were analyzed. In order to illustrate the trend of S incidence, the patients from 1981-1993 and 1994- 2006 were assigned to group A and B respectively. The percentage of S in all malignancies [Ms], including all kinds of internal carcinomas and skin cancers, and the percentage of S in inpatients and outpatients [IOPs] between group A and B were separately compared to illustrate the trend in S incidence in this area. [1] Of the 1048 S s included, 308 [29.4%] were squamous cell carcinoma [S C], 293 [28.0%] basal cell carcinoma [B] and 168 [16.0%] cutaneous malignant melanoma [MM] .Ratio of male to female was 1.5:1.0.Median age was 54.0 +/- 23.0 years.40.8%of the S s occurred on the head, 35.0%on the trunk and 24.2%on the extremities. Median age of MM [53.0 +/- 22.5] was less than those of B [58.0 +/- 18.3 years] and S [57.0 +/- 20.0 years] .83.6%of the B s, 49.8%of the S s and 13.5% of the CMMs occurred on the head. [2] Of the 168 MMs, 106 [63.1%] occurred on the acral, 23 [13.7%] on the head, 24 [14.3%] on the trunk and 15 [8.9%] on the limbs. Of the 106 acral melanoma [AM], 41 [38.7%] occurred on the plantar skin, 19 [17.9%] on the heel, 15 [14.2%] on the subungual skin of thumbs, 13 [12.3%] on the subungual skin of big toes and 18 [17.0%] on other acra. [3] The percentages of S in IOPs [S s/IOPs] in Group A and B were 0.0038% [325/8, 457, 672], 0.0066% [723/11, 037, 720], an increase of by 74%.The percentages of S in all Ms [S /Ms] were 2.1% [325/15, 363] and 3.1% [723/23, 364], an increase of 48%.During the same period, the percentages of Ms in IOPs [Ms/IOPs] were 0.18% [15, 363/8, 457, 672] and 0.21% [23, 364/11, 037, 720], increased only by 17%. In our study, S C, B and MM were major S types. The head and trunk are the main sites for S occurring. AM is the most common MM. In past 26 years, the percentages of S in all malignancies and in inpatients and outpatients have increased in this hospital. The finding in our study provides a clue for understanding of the trend of S in West China

Tumoración de crecimiento rápido en muslo de paciente femina de 72 años de edad / Rapid growth tumor in thigh of patient 72 year-old femina

Paciente femenina de 72 años de edad hipertensa, portadora de enfermedad de Rendu Osler Weber, quien consultó por presentar una masa de aproximadamente 6 meses de evolución en la cara anterior del muslo izquierdo; la resonancia magnética de la zona demostró la presencia de lesión ocupante de espacio en los comportamientos musculares del muslo, de aspecto sólido bien delimitada por los planos aponeuróticos; en forma ambulatoria le fue tomada biopsia con aguja de Trucut y la biopsia resultó en un liposarcoma pleomórfico; resecó quirúrgicamente y se aplicó radioterapia local. La evolución de la paciente fue satisfactoria sin evidencia recidiva en los siguientes 12 meses de control.

Sarcomas de partes blandas de cabeza y cuello: factores de pronósticos / Soft part sarcomas of head and neck: prognoses factors

Evaluar los factores pronósticos que afectan la sobrevida de los pacientes con sarcomas de partes blandas de cabeza y cuello. Se evalúan retrospectivamente 24 pacientes evaluados y tratados en el Servicio de Cabeza y Cuello del Hospital Oncológico Padre Machado entre enero de 1980 y diciembre de 2000 con diagnóstico de sarcoma de partes blandas de cabeza y cuello. La relación hombre:mujer fue de 1,18:1 con una edad media de 44,04 años. Veinte pacientes fueron sometidos a cirugía con 35 por ciento de lesiones con márgenes microscópicamente positivos, Se aplicó radioterapia postoperatoria en 11 pacientes. El seguimiento promedio fue de 45,3 meses, la recaída en general fue de 40 por ciento. La recaída local fue del 35 por ciento, la recaída a distancia del 10 por ciento y la regional del 5 por ciento. La tasa de sobrevida actuarial libre de enfermedad y sobrevida global a los 5 años fue de 38 por ciento y 82 por ciento, respectivamente. Los factores pronósticos más relevantes para recaída local fueron: un tamaño tumoral mayor de 5 cm, márgenes de resección completa del tumor, parece ser el mejor tratamiento para esta patología. La ubicación anatómica de los sarcomas de partes blandas en la región de la cabeza y cuello, juega un papel determinante en el pronóstico de estos pacientes

Clear cell sarcoma of soft parts. A case report and review of the literature

The case of a 25-year-old woman with recurring and metastasizing clear cell sarcoma of soft parts (CCS) is presented. The clinical setting and pathological appearance were in agreement with the original cases described. This report serves to highlight the clinico-pathological features of this rare and aggressive malignancy with specific reference to prognostic markers and treatment.