Soft-Tissue Sarcomas of the Genitourinary Tract with Radiologic-Pathologic Correlation.

Genitourinary tract soft-tissue sarcomas are rare neoplasms with varied pathologic and clinical features. While some of these tumors may be aggressive high-grade malignancies, others are low grade with a relatively better prognosis. Given that the grade and extent of the disease are important prognostic factors in these tumors, timely diagnosis is crucial. Unfortunately, most imaging features of these malignancies are not pathognomonic, and various histologic subtypes do not manifest with typical classic imaging features. Therefore, reliable differentiation of the various histologic tumor types is not always possible based solely on the radiologic manifestations. Imaging findings need to be considered in the context of clinical history in corroboration with radiologic-pathologic correlation. The authors discuss the specific imaging and pathologic characteristics of various genitourinary tract soft-tissue sarcomas, emphasizing diagnostic difficulties and differential diagnoses. ©RSNA, 2024 Test Your Knowledge questions for this article are available in the supplemental material.

Clinicopathologic Classification of Renal Cell Carcinoma in Patients ≤40 Years Old From Peru.

INTRODUCTION: There are scant data on renal cell carcinoma (RCC) from relatively younger patients in South America using contemporary classification. METHODS: Fifty-nine consecutively treated patients with RCC (≤40 years old) were assessed from the National Institute of Neoplastic Diseases in Peru from 2008 to 2020 (34 males; 25 females), age range of 13 to 40 years. RESULTS: Most common presenting symptoms were flank pain (n = 40), hematuria (n = 19), and weight loss (n = 12). Associated conditions included 4 patients with proven or presumed tuberous sclerosis and 1 patient with von Hippel Lindau syndrome, all with clear cell RCC. Tumor histopathology was clear cell RCC in 32 of 59 (54%), chromophobe RCC in 6 of 59 (10%), and 5 of 59 (8%) each of papillary RCC and MiT family translocation-associated RCC. Four of 59 (7%) were FH-deficient RCC and 2 of 59 (3%) remained unclassified. The remaining tumors were isolated examples of clear cell papillary renal cell tumor, eosinophilic solid and cystic RCC (ESC RCC), RCC with fibromyomatous stroma, sarcomatoid RCC, and sarcomatoid clear cell RCC. Of the 4 FH-deficient RCCs, none had the classic morphology. The 5 MiT family translocation RCCs had variable morphology. There were 41 tumors without recurrence or metastases, 3 tumors with local recurrence only, 8 tumors with metastases only, and 7 tumors with both local recurrence and metastases. CONCLUSIONS: The current study demonstrates the importance of special studies in accurately classifying RCC in younger individuals. The distribution of RCC subtypes in younger individuals is similar between 2 representative large institutions of the United States and Peru.

[Malignant gastrointestinal neuroectodermal tumor: A report of 2 cases and a review of the literature]. / Tumor neuroectodérmico maligno del tracto gastrointestinal: Reporte de 2 casos y revisión de la literatura.

Malignant gastrointestinal neuroectodermal tumour (GNET) is an extremely rare neoplasm first described by Zambrano in 2003 as clear cell sarcoma like tumor of the gastrointestinal tract. In contrast to clear cell sarcoma, it has giant osteoclast cells and shows diffuse and intense positivity for S-100 with no immunohistochemical or ultrastructural melanocyte differentiation. We present the first cases of GNET reported in South America, occurring in Peru. Two cases of GNET, one in a female and one in a male, both between 60 and 70 years of age, were referred to our hospital for reevaluation. One underwent further treatment in our centre, but with an unfavourable evolution. Pathologists should be aware of the diagnostic criteria for GNET in order to avoid misdiagnosis due to confusion with other non-epithelial gastrointestinal neoplasms.

[Inflammatory miofibroblastic tumor of the biliary tract in a pediatric patient]. / Tumor miofibroblástico inflamatorio de vías biliares en un paciente pediátrico.

The inflammatory myofibroblastic tumor is an infrequent mesenchymal neoplasm, making the clinical as well as the pathological diagnosis by biopsies is a challenge. We present a case of a pediatric patient with an inflammatory myofibroblastic tumor located at the level of the bile ducts. We sent the lesion sample for intraoperative analysis through freezing techniques, the microscopic report was also incorrect. However, when the plates were reviewed after the inclusion of the lesion and supplemented by immunohistochemical studies, it was concluded that the lesion corresponded to an inflammatory myofibroblastic tumor.

Benign Multicystic Peritoneal Mesothelioma in a Male Teen: Case Report and Review of the Literature.

The case of a Peruvian 15-year-old male with a left-flank abdominal mass suspected to be a peritoneal pseudomyxoma is presented. The patient underwent a R0 surgery, and the pathology review showed a benign multicystic peritoneal mesothelioma. Characterized by recurrent mesothelial peritoneal cysts originating in the epithelial and mesenchymal elements of mesothelial tissue, this benign tumor is unusual among young males.

Tumor miofibroblástico inflamatorio de vías biliares en un paciente pediátrico / Inflammatory miofibroblastic tumor of the biliary tract in a pediatric patient

RESUMEN El tumor miofibroblástico inflamatorio es una neoplasia mesenquimal infrecuente, realizar el diagnóstico clínico así como el patológico por biopsias es un desafío. Presentamos un caso de un paciente pediátrico con tumor miofibroblástico inflamatorio localizado a nivel de las vías biliares. Se realizaron estudios de laboratorio así como imagenológicos en los cuales se planteó un diagnóstico inexacto, del mismo modo cuando se envió la muestra de la lesión para el análisis intraoperatorio a través de técnicas de congelación, el reporte microscópico también fue incorrecto. Sin embargo cuando se realizó la revisión de las láminas tras la inclusión de la lesión y complementando con estudios de inmunohistoquimica, se concluyó que la lesión correspondió a un tumor miofibroblástico inflamatorio.

ABSTRACT The inflammatory myofibroblastic tumor is an infrequent mesenchymal neoplasm, making the clinical as well as the pathological diagnosis by biopsies is a challenge. We present a case of a pediatric patient with an inflammatory myofibroblastic tumor located at the level of the bile ducts. We sent the lesion sample for intraoperative analysis through freezing techniques, the microscopic report was also incorrect. However, when the plates were reviewed after the inclusion of the lesion and supplemented by immunohistochemical studies, it was concluded that the lesion corresponded to an inflammatory myofibroblastic tumor.

Fibrohistiocitoma maligno de las extremidades - una revisión retrospectica en el Instituto Nacional de Enfermedades Neoplásicas Dr. Eduardo Cáceres Graziani del año 1996 al 2006 / Malignant Fibrohistiocytoma of limbs - a review retrospectica at the National Institute of neoplastic diseases Dr. Eduardo Cáceres Graziani from 1996 to 2006

Objetivo: Conocer las características clínicas y patológicas de los pacientes con fibrohisticitoma maligno (FHM) de las extremidades diagnosticados en el Instituto Nacional de Enfermedades Neoplásicas. Material y Metodos: Se realizó un estudio descriptivo retrospectivo tipo serie de casos de 222 pacientes con diagnostico de FHM de las extremidades del año 1997 al 2006. Resultados: El promedio de edad fue 56.3 años (rango 18-96 años). El tamaño tumoral medio fue de 10.3 cm (rango 1-35 cm). 2,7% fueron grado histológico I; el 12.2%, grado II y el 66%, grado III. La recurrencia local fue 23.1% y la metástasis a distancia ocurrió en 27.6% (68.2 % a los pulmones). El tiempo de sobrevida media a 5 años fue 58%. La profundidad del tumor influenció en la SLE, p < 0.015. En el análisis multivariado el único factor asociado a una mayor tasa de recurrencia local fueron los márgenes quirúrgicos positivos (p < .003). Los factores pronósticos significativos en el desarrollo de enfermedad a distancia fueron la profundidad (p < .004) y la recurrencia local (p < .02); mientras que los factores pronostico que afectan la sobrevida fueron, la profundidad del tumor (p < .04), la presencia de metástasis a los ganglios linfáticos regionales (p < .02) y los márgenes quirúrgicos positivos (P < .03). Los tratamientos adyuvantes no mejoraron la sobrevida. Conclusiones: Los factores pronóstico de recurrencia local fueron los márgenes quirúrgicos positivos. Los factores pronóstico de recaídas a distancia fueron la profundidad y recurrencia local, mientras que los factores importantes que afectan la sobrevida fueron la profundidad del tumor, la presencia de metástasis a los ganglios linfáticos regionales al momento del diagnostico y los márgenes quirúrgicos positivos.

Objective: Malignant fibrous histiocytoma (FHM) is the most common subtype of soft tissue sarcoma (STS). The purpose of this study is to analyze the prognostic factors influencing local recurrence and survival. Material and Methods: A retrospective review was performed at the National Institute of Neoplastic Diseases (INEN) in all patients with a diagnosis of extremity FHM, between 1997 and 2006. Results: We reviewed cases histories of 222 patients with histologically confirmed FHM. Mean age was 56.3 years (range 18-96 years). Mean tumor size was 10.3 cm (range 1-35 cm). 2.7% of the cases had histological grade I, 12.2%, grade II and 66%, grade III. Local recurrence was 23.1% with an average of 16 months. Distant metastasis occurred in 27.6% of patients. The median survival time was 108 months with a cumulative survival rate at 5 years of 58%. The depth of the tumor influenced the disease-free survival (DFS) (depth vs. superficial, P <0.015). In the multivariate analysis the only factor associated with a higher rate of local recurrence was positive surgical margins (P<.003). Significant prognostic factors in the development of distant disease were depth (P <.004) and the recurrence (P <.02), while still important prognostic factors affecting survival were tumor depth (P <.04), presence of metastases to regional lymph nodes (P <.02) and positive surgical margins (P <.03). Adjuvant radiotherapy reduced local recurrence but did not reach statistical significance. Chemotherapy had no significant improvement in survival or local recurrence rates and metastasis. Conclusion: The only prognostic factor for local recurrence was positive surgical margins. Prognostic factor for distant relapse were the depth of tumor and local recurrence, while other important factors affecting survival were tumor depth, presence of metastases to regional lymph nodes at diagnosis and positive surgical margins.

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.

Angiomixoma agresivo: presentación clínico patológica de caso y revisión de la literatura / Aggressive angiomyxoma: clinical pathological case presentation and review of literature

El angiomixoma agresivo es una neoplasia mesenquimal benigna, poco frecuente con predilección por la pelvis y periné femeninos, con tendencia a la recurrencia después de la excisión. Se presenta el caso de una mujer de 31 años con una tumoración polipoide en vulva. Histológicamente la tumoración presentaba estroma mixoide, hipocelular, vasculatura variable. Las células tenían aspecto miofibroblástico y su inmunofenotipo fue positivo para Desmina, Alfa Actina, Actina HHF-35 y receptor de estrógenos.

Aggressive angiomyxoma is a benign mesenchymal neoplasm, uncommon with a predilection for the female pelvis and perineum, with tendency to recur after excision. A case of a 31 year old woman with a polypoid tumor in the vulva. Histologically the tumor stroma showed myxoid, hypocellular, vasculature variable. Myofibroblastic cells had the appearance and the immunophenotype was positive for desmin, alpha actin, actin HHF-35 and estrogen receptor.