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)

Sarcoma de Células Claras en pierna de mujer de 28 años / Clear cell sarcoma on 28 years old woman leg

Los tumores de partes blandas son tumores que se presentan en adultos, aproximadamente a los 60 años. De estos tumores los sarcomas son poco frecuentes y tienen preferencia en las extremidades y muchas veces invaden estructuras músculo aponeuróticas cercanas, y poca invasión vascular o nerviosa lo que las hace de fácil acceso con poca morbilidad para su biopsia. El diagnóstico de los tumores de partes blandas se basa en el estudio histológico de anatomía patológica y, a veces se debe asociar a estudios de citogenética para poder tener un diagnóstico específico. El tratamiento principal es quirúrgico y puede llegar estar asociado a terapia adyuvante.

Soft tissue tumors are tumors that occur in adult's age, at about 60 age. Of these tumors, sarcomas are rare and have preference in the extremities, and often invade nearby muscle-fascia structures, and little vascular or nerve invasion, making them easily accessible with little morbidity for biopsy. The diagnosis of soft tissue tumors is based on the histological study of pathological anatomy and, sometimes, it must be associated with cytogenetic studies in order to have specific diagnosis. The main treatment is surgical and may end up being associated with adjuvant therapy.

Clear cell sarcoma like tumor of gastrointestinal tract: Experience of three cases and review of literature

Clear cell sarcoma-like tumor of the gastrointestinal tract (CCSGT) is a rare, aggressive tumor with many histological mimickers. Herein, we have documented our experience of three cases of CCSGT and reviewed the literature. The index cases were identified in male patients in their twenties, one in jejunum and two in the distal colon. Histomorphological examination revealed the characteristic heterogeneous histomorphology with patchy immunohistochemical positivity with S100 protein and negative melanocytic markers. The fluorescence in-situ hybridization test showed translocation of the EWSR1 (22q12) gene in >80% tumor cells. While one of our patients died after 2 years with lung metastasis, the other two patients are still alive on 1.5 years and 3 months follow up, respectively. CCSGT is a rare malignant tumor of the gastrointestinal tract. Although characteristic morphology, use of a judicial panel of immunohistochemical stains, and translocation study for EWSR1 gene can establish the diagnosis, experience in adjuvant therapy is still limited.

Sarcoma de células claras del pie. Presentación de un caso / Clear cell sarcoma of the foot. Case presentation

RESUMEN El sarcoma de células claras fue descrito por primera vez por Franz M. Enzinger en 1965. Está íntimamente asociado a tendones y aponeurosis, excepcionalmente compromete la epidermis. Afecta fundamentalmente a pacientes jóvenes y se caracteriza por múltiples recurrencias locales y metástasis tardías. Se presenta un paciente de 22 años de edad, masculino que fue sometido a tratamiento quirúrgico radical (amputación transmetatarseana del 1er y 2do rayo). Los estudios anatomopatológicos confirmaron el diagnóstico de un sarcoma de células claras. El paciente se encuentra libre de la enfermedad después de 6 años de operado e incorporado a su vida social (AU).

ABSTRACT The clear cell sarcoma was firstly described by Franz M. Enzinger in 1965. It is intimately associated to tendons and aponeurosis, exceptionally compromising the epidermis. It mainly affects young patients and is characterized by multiple local recurrences and late metastases. We present a male patient, aged 22 years, who underwent a radical surgical treatment (transmetatarsal amputation of the 1st and 2nd rays). The anatomic-pathological studies confirmed the diagnosis of clear cell sarcoma. 6 years after surgery, the patients is free of the disease and reincorporated to his social life (AU).

Clear cell sarcoma arising from paraspinal ligament of thoracic spine: Report of an unusual case

Clear cell sarcoma (CCS) is an unusual but aggressive soft-tissue tumor with an incidence of <1% of all soft-tissue sarcomas. It was previously termed “malignant melanoma of soft parts” due to its resemblance clinically and morphologically. Normally CCS is seen in patients aged 20–40 years. A rare case of CCS of paraspinal ligament in a 5-year-old boy is being reported. Histopathology and immunohistochemistry markers confirmed the diagnosis. The present case is unique since the entity itself is rare and also due to its occurrence in a child.

Current status and progress of treatment for clear cell sarcoma / 中国肿瘤临床

Clear cell sarcoma (CCS) is a rare, aggressive soft tissue sarcoma that primarily affects adolescents and young adults and typically involves tendons and aponeuroses. Currently, systematic treatment based on surgery is mainly adopted for CCS; the out-comes of chemotherapy and radiotherapy are poor due to the high resistance. With the progression in research regarding CCS patho-genesis, targeted therapy and immunotherapy are becoming increasingly important in the treatment of CCS. However, CCS has a high rate of local recurrence and distant metastasis, so it is urgent to identify comprehensive treatment regimens. This article reviews the current status and progress of treatment for CCS to provide information for clinical practice.

Clinical analysis of clear cell sarcoma of the kidney in children / 中国肿瘤临床

Objective:To summarize the clinical features of clear cell sarcoma of the kidney (CCSK) in children and to evaluate the effica-cy and safety of the WT-2009 chemotherapy protocol. Methods:Clinical data of children with CCSK recorded between January 2009 and December 2014 were retrospectively analyzed. Follow-ups were conducted to monitor the postoperative conditions of the pa-tients. Results:Nine cases of CCSK were recruited, and another three cases were excluded for the analysis because of incomplete clini-cal data. All patients achieved complete remission when the treatment was finished. The estimated 3-year overall survival rate was 100％, and the estimated 3-year event-free survival rate was 83.3％. No report is available on the significant side effects associated with this treatment. Conclusion:CCSK is rare in children and is easily misdiagnosed. The chemotherapy based on the WT-2009 proto-col can produce a favorable prognosis and a high tolerance for patients with CCSK. However, the treatment for high-risk patient needs to be further explored, and follow-ups must be intensified.

Multidisciplinary diagnosis, treatment, and follow-up of children with clear cell sarcoma of kidney / 上海交通大学学报（医学版）

Objective · To investigate the clinical characteristics and multidisciplinary treatment of children with clear cell sarcoma of kidney (CCSK). Methods · Data of seven children with CCSK treated at Xinhua Hospital, Shanghai Jiao Tong University School of Medicine between Jan 2011 and Jan 2016 were collected. The retrospective analysis of clinical manifestations, features of imaging and pathology, treatment, and follow-up was performed. Results · Of 7 children with CCSK, 6 were male and 1 was female with the median age of 28 months (4-59 months), and 3 were at stageⅠ, 2 at stage Ⅲ, and 2 at stage Ⅳ. All cases were discussed and evaluated by multidisciplinary teams, including pediatric hematology/oncology, pediatric surgery, pathology, radiology and radiotherapy. CCCG-WT-2009 protocol was adopted to treat these patients. The median follow-up period was 22 months (8-56 months). Six children survived and one died. Conclusion · The multidisciplinary treatment mode can effectively improve the prognosis of CCSK. CCCG-WT-2009 protocol has good therapeutic effect and high cure rate for children with early stage CCSK, but the treatment of advanced stage CCSK needs to be further explored and perfected.

Renal Tumors in Children / 임상소아혈액종양

Pediatric renal tumors represent a diverse group, which include Wilms' tumor (WT), renal cell carcinoma (RCC), clear cell sarcoma of the kidney (CCSK), congenital mesoblastic nephroma, malignant rhabdoid tumor of the kidney (MRTK) and primitive neuroectodermal tumor. WT (85%) and RCC (8%) are the most prevalent types. WT predominates among the 1- to 10-year age group, but RCC exceeds WT in children over age 10 years. Pediatric renal tumors are genetically, histologically and clinically heterogeneous. The overall survival for children with localized WT is currently more than 90%, whereas poorer survival rates are observed for anaplastic WT, metastatic WT, metastatic CCSK, MRTK, metastatic RCC and relapsed WT. Therefore risk-stratified treatment is important to minimize treatment morbidity while preserving survival. This review focuses on distinct characteristics of each tumor type and optimal stratified treatment.

Renal Tumors in Children / 임상소아혈액종양

Pediatric renal tumors represent a diverse group, which include Wilms' tumor (WT), renal cell carcinoma (RCC), clear cell sarcoma of the kidney (CCSK), congenital mesoblastic nephroma, malignant rhabdoid tumor of the kidney (MRTK) and primitive neuroectodermal tumor. WT (85%) and RCC (8%) are the most prevalent types. WT predominates among the 1- to 10-year age group, but RCC exceeds WT in children over age 10 years. Pediatric renal tumors are genetically, histologically and clinically heterogeneous. The overall survival for children with localized WT is currently more than 90%, whereas poorer survival rates are observed for anaplastic WT, metastatic WT, metastatic CCSK, MRTK, metastatic RCC and relapsed WT. Therefore risk-stratified treatment is important to minimize treatment morbidity while preserving survival. This review focuses on distinct characteristics of each tumor type and optimal stratified treatment.

Clear Cell Sarcoma of the Wrist: MRI Findings with Diffusion-Weighted Image and Histopathologic Correlation

Clear cell sarcoma is rare and difficult to diagnose. Herein, we present a case of clear cell sarcoma in the dorsum of the wrist with MRI findings, including diffusion-weighted imaging, and histopathologic correlation, which was initially diagnosed as giant cell tumor of tendon sheath.

Clear cell sarcoma of gastrointestinal tract：clinicopathologic analyses and review of litera-tures / 临床与实验病理学杂志

Purpose To study the clinicopathologic and genetic features of clear cell sarcoma of the gastrointestinal tract. Methods One case of clear cell sarcoma of the gastrointestinal tract was analyzed by histology, immunohistochemistry, fluorescence in situ hy-bridization ( FISH) , with review of relevant literatures. Results A female patient was admitted to the hospital with a history of 1-week abdominal pain. Computed tomography revealed a mass in right hemicolon. A grey-white ulcerated mass was observed in the colon, in-volving the entire thickness of the colon wall extending into the subserosa. Microscopically, medium-size oval or round cells were ar-ranged in sheets and scattered osteoclast-like multinucleated giant cells were also presented. Immunohistochemically, the tumor cells were strongly positive for S-100 protein, but negative for HMB-45, Melan-A, CD117, CD1a and PCK. FISH showed the rearrangement of EWSR1 in 74% of the neoplasm cells. Conclusions Clear cell sarcoma of the gastrointestinal tract is an extraordinarily rare gastro-intestinal neoplasm that shows peculiar histopathological, immunohistochemical, ultrastructural, and genetic features. Whether clear cell sarcoma-like gastrointestinal tumor represents a distinct entity awaits larger series, including cytogenetic or molecular biological in-vestigation.

18F-FDG PET/CT image analysis of clear cell sarcoma of soft tissue / 第二军医大学学报

Objective To investigate the 18F-FDG PET/CT features of clear cell sarcoma (CCS) of soft tissue. Methods Ten patients with pathologically confirmed CCS were enrolled in the present study. All patients underwent preoperative 18F-FDG PET/CT scanning, and the image findings and clinical characteristics of the tumors were analyzed. Results The mean maximum standard uptake value (SUVmax) of the primary lesions was 6.52(range, 5-9.6) in the patients. PET/CT revealed lung metastasis in 7 patients, soft tissue metastases in 5, and regional lymph node metastases in 3, with no brain/bone/liver metastases. CT findings showed lung metastases in 6 cases and soft tissue metastasis in one. Conclusion CCS 18F-FDG PET/CT image lacks specificity, but it can better demonstrate the extent and metastases of CCS, helping the accurate, timely, and more targeted therapy.

Clear Cell Sarcoma of the Knee. A Report of Two Rare Cases

Clear cell sarcoma of soft tissue is a rare type of soft tissue sarcoma. It is derived from melanoblast like cells located within subcutaneous tissue, tendon and aponeuroses. The tumour is also known as malignant melanoma of soft parts because it has similar morphology to malignant melanoma. Unlike malignant melanoma, however, it is not associated with a cutaneous lesion. We report here two cases of this tumour occurring in young adults.

Clear Cell Sarcoma of the Upper Thoracic Back Muscle

Clear cell sarcoma (CCS), also called malignant melanoma of soft parts, is a rare malignant soft tissue tumor and is often associated with tendons or aponeuroses. Most of CCS involve extremities, especially lower extremities, but a tumor occurring in the trunk is rare. We report an extremely rare case of CCS originated in the upper thoracic back muscle. To our knowledge, this case is the second report of CCS of the back muscle.

A Case of Clear Cell Sarcoma Occurring on the Abdomen

Clear cell sarcoma is a rare malignant tumor representing about 1% of soft tissue tumors. It usually presents in the distal extremities of young adults, frequently attached to tendons or aponeuroses. This slowly progressive tumor tends to recur and results in eventual development of metastatic growth. Early recognition of the disease and prompt wide excision of tumor are essential to get a favorable outcome. We report a rare case of clear cell sarcoma in a 57 year-old female who presented with an erythematous hard nodule on her abdomen.

A Case of Clear Cell Sarcoma showing t(12;22)(q13;q12) in Chromosomal Study / 대한피부과학회지

Clear cell sarcoma is a melanoma of soft parts and a rare dermal nodule of tissue with unknown origin. It mainly affects young adults with a predominance in women, and the predilection sites are extremities, especially foot and ankle. The cytogenic hallmark of clear cell sarcoma is the presence of the t(12;22)(q13;q12). This translocation has been described in the majority of reported clear cell sarcoma cases, however not in other malignancies. And this method has not been popular in dermatology, so we present a case of clear cell sarcoma which was identified the presence of translocation.

A Case of Clear Cell Sarcoma with Inguinal Lymph Node Metastasis / 대한피부과학회지

Clear cell sarcoma is a rare malignant soft tissue tumor, a well accepted clinicopathological entity of malignant melanoma of soft parts. It mainly affects young adults with a predominance in women. The predilection sites are extremities, especially foot and ankle. We report a case of clear cell sarcoma with inguinal lymph node metastasis in a 26-year-old woman. She had a deep seated nodule on her left anterior thigh for 4 years along with lymphadenopathy for 2 months at the initial presentation. Histopathologic findings displayed a uniform pattern of compact nests or fascicles of round or fusiform cells showing clear cytoplasm and vesicular nuclei. Total removal of the original mass, lymphadenectomy, adjuvant radiation and chemotherapy with dacarbazine were performed. To date, no recurrence has been observed during a 1 year- follow up period.

A Case of Clear Cell Sarcoma Developing Early Systemic Metastasis / 대한피부과학회지

Clear cell sarcoma is a rare malignant soft tissue neoplasm with unknown oringin. It is slow growing tumor , but uncommonly, it shows rapidly progressive course. In Korea, there has been rare case of clear cell sarcoma, especially with systemic metastasis. We herein present a case of clear cell sarcoma rapidly progressing with systemic metastasis. She had a deep seated nodule on left heel and inguinal and abdominal lymphadenopathy at the initial presentation. While chemotherapy, acute renal failure occurred and it was suspicious from abdominal ultrasono that both kidneys were invaded with clear cell sarcoma. She died with repiratory failure.

Clear Cell Sarcoma of the Kidney: A case report

Clear Cell Sarcoma of the Kidney (CCSK) is a rare malignant childhood tumor with frequent metastasis to the bone. We report a case of right sided in a 5 month-old girl. A radical nephrectomy was performed. It was clinical stage III with renal capsular invasion and lymph node metastasis by the classification of NWTS-5. Histologic examination revealed the classic pattern of CCSK. Postoperative adjuvant chemotherapy with doxorubicin and radiotheraphy were applied.