scholarly journals A long-term survivor of clear cell sarcoma-like tumor of the gastrointestinal tract with liver metastasis: a case report

2020 ◽  
Vol 6 (1) ◽  
Author(s):  
Takuhisa Okada ◽  
Yasumitsu Hirano ◽  
Shintaro Ishikawa ◽  
Hiroka Kondo ◽  
Toshimasa Ishii ◽  
...  
Keyword(s):  
Small Intestine ◽  
Lymph Node ◽  
Gastrointestinal Tract ◽  
Liver Metastasis ◽  
Surgical Resection ◽  
Clear Cell ◽  
Clear Cell Sarcoma ◽  
Cell Sarcoma

Abstract Background Clear cell sarcoma-like tumor of the gastrointestinal tract (CCSLTGT) is extremely rare. It is a mesenchymal neoplasm that usually forms in the small intestine of adolescents and young adults, is prone to local recurrence and metastasis, and has a high mortality rate. We report a patient with CCSLTGT with lymph node- and liver metastases, who continues to survive 6 years after initial surgical resection. Case presentation A 38-year-old woman presented with lightheadedness. Laboratory analysis revealed anemia (hemoglobin, 6.7 g/dL), and enhanced computed tomography (CT) demonstrated a mass in the small intestine, about 6 cm in diameter, with swelling of 2 regional lymph nodes. Double-balloon small intestine endoscopic examination revealed a tumor accompanied by an ulcer; the biopsy findings suggested a primary cancer of the small intestine. She was admitted, and we then performed a laparotomy for partial resection of the small intestine with lymph node dissection. Pathologic examination revealed CCSLTGT with regional lymph node metastases. About 3 years later, follow-up CT revealed a single liver metastasis. Consequently, she underwent a laparoscopic partial liver resection. Histopathologic examination confirmed that the liver metastasis was consistent with CCSLTGT. It has now been 3 years without a recurrence. Conclusion Repeated radical surgical resection with close follow-up may be the only way to achieve long-term survival in patients with CCLSTGT.

Imprint cytology of clear cell sarcoma-like tumor of the gastrointestinal tract in the small intestine: A case report

2017 ◽  
Vol 45 (12) ◽  
pp. 1137-1141 ◽  
Author(s):  
Takashi Kato ◽  
Shin Ichihara ◽  
Hiroko Gotoda ◽  
Shunji Muraoka ◽  
Terufumi Kubo ◽  
...  
Keyword(s):  
Small Intestine ◽  
Case Report ◽  
Gastrointestinal Tract ◽  
Clear Cell ◽  
Clear Cell Sarcoma ◽  
Imprint Cytology ◽  
Cell Sarcoma

scholarly journals Long-term Follow-up of Metastatic Clear Cell Sarcoma of Soft Tissue: A Case Report

2012 ◽  
Vol 61 (3) ◽  
pp. 600-603
Author(s):  
Shinichiro Tanaka ◽  
Kiyoshi Oka ◽  
Toshitake Yakushiji ◽  
Hiroo Sato ◽  
and Hiroshi Mizuta
Keyword(s):  
Case Report ◽  
Soft Tissue ◽  
Clear Cell ◽  
Clear Cell Sarcoma ◽  
Cell Sarcoma ◽  
Long Term Follow Up

Clear cell sarcoma of the kidney: A clinicopathologic study of 21 patients with long-term follow-up evaluation

1985 ◽  
Vol 16 (12) ◽  
pp. 1219-1230 ◽  
Author(s):  
C. Sotelo-Avila ◽  
F. Gonzalez-Crussi ◽  
S. Sadowinski ◽  
W.M. Gooch ◽  
R. Pena
Keyword(s):  
Clear Cell ◽  
Clear Cell Sarcoma ◽  
Cell Sarcoma ◽  
Clinicopathologic Study ◽  
Long Term Follow Up

scholarly journals Is clear cell sarcoma a malignant form of psammomatous melanotic schwannoma?

2006 ◽  
Vol 21 (6) ◽  
pp. 1-4 ◽  
Author(s):  
Ahmet Şengöz ◽  
Erol Taşdemiroğlu ◽  
Halit Togay
Keyword(s):  
Malignant Melanoma ◽  
Nerve Root ◽  
Clear Cell ◽  
Clear Cell Sarcoma ◽  
Histopathological Diagnosis ◽  
Total Resection ◽  
Cell Sarcoma ◽  
Melanotic Schwannoma ◽  
Psammomatous Melanotic Schwannoma

✓The authors present a case of clear cell sarcoma (CCS) in which the tumor originated in the S-1 nerve root and had been previously diagnosed as psammomatous melanotic schwannoma (PMS). This is the third case of a spinal nerve root origin for CCS reported in the English-language literature. The similar histogenesis of CCS and malignant melanoma supports the hypothesis that biological agents or immunotherapy are potentially important areas of investigation. The patient underwent S1–3 laminectomy and gross-total resection of the mass lesion. The border of the resection was extended 1 cm distal to the tumor margin. The postoperative period was uneventful. The new histopathological diagnosis was CCS (malignant melanoma of soft tissue). Despite total resection, the patient returned with disseminated disease at the 18-month follow-up visit. His follow-up magnetic resonance image of the lumbar spine revealed sacral L5–S3 involvement of the vertebral bodies along with disseminated cauda equina seeding. A CCS originating from peripheral nerves is quite rare. The histopathological and immunohistochemical appearance of CCSs resembles those of PMSs. Surgery should be the first choice of treatment.

Malignant Gastrointestinal Neuroectodermal Tumor: a case report and a review of the literature

2021 ◽  
Vol 156 (Supplement_1) ◽  
pp. S66-S67
Author(s):  
B Youssef ◽  
D Asberry ◽  
R Mohamed
Keyword(s):  
Case Report ◽  
Gastrointestinal Tract ◽  
Clear Cell ◽  
Muscularis Propria ◽  
Clear Cell Sarcoma ◽  
Neuroectodermal Tumor ◽  
High Rate ◽  
Cell Sarcoma ◽  
Molecular Features ◽  
Eosinophilic Cytoplasm

Abstract Introduction/Objective Malignant gastrointestinal neuroectodermal tumor (GNET) is a rare soft tissue tumor arising in the wall of the gastrointestinal tract. The GNET was first described as an osteoclast rich tumor of the gastrointestinal tract with features resembling clear cell sarcoma, with only few cases reported in the literature. Methods/Case Report We report a case of a 71-year-old man with a past medical history of hypertension, hyperlipidemia, and benign prostatic hyperplasia presented with complaints of dyspnea, dizziness, fatigue, black stools, and a recent syncopal episode. Laboratory testing revealed anemia (HB 5.4 g/dL). Esophagogastroduodenoscopy demonstrated a submucosal gastric mass. An abdominal CT scan confirmed a 7.8 cm mass along the gastric cardia and fundus. Results (if a Case Study enter NA) Biopsy rendered a gastrointestinal neuroectodermal tumor (GNET). Microscopically, the tumor cells were spindle with eosinophilic cytoplasm and arranged in fascicules. They were positive for CD56, CD99, and Fli-1, synaptophysin, and negative for chromogranin, TTF-1, SMA, desmin, CD34, EMA, pan-cytokeratin, and lymphoid markers. Two months later, further imaging confirmed metastasis to the liver and spleen. GNETs typically arise within the muscularis propria of the gastrointestinal tract and often extend into the submucosa and subserosa. Conclusion The most important differential of GNET is the clear cell sarcoma of the gastrointestinal tract (CCS-GI). Both share similar morphological as well as molecular features and show S100 positivity; however, the lack of melanocytic differentiation in GNET distinguishes it from CCS-GI. Both typically show rearrangements of the EWSR1 gene, with t(12;22) (q13;q12) EWSR1-ATF1 or t(2;22)(q34;q12) EWSR1-CREB1 fusions. Pathologists should be aware of GNET diagnostic entity due to its aggressive behavior and high rate of recurrence and mortality even after complete resection.

Lymphotropic Sarcomas (Epithelioid Sarcoma and Clear Cell Sarcoma)

2019 ◽  
Author(s):  
Alexandra G Lopez-Aguiar ◽  
Kenneth Cardona
Keyword(s):  
Lymph Node ◽  
Sentinel Lymph Node ◽  
Sentinel Lymph Node Biopsy ◽  
Soft Tissue ◽  
Clear Cell ◽  
Epithelioid Sarcoma ◽  
Clear Cell Sarcoma ◽  
Lymph Node Biopsy ◽  
Cell Sarcoma ◽  
Node Biopsy

Lymphotropic sarcomas encompass a rare subset of soft tissue sarcoma histotypes known for their distinct ability to metastasize to locoregional lymph nodes. Epithelioid and clear cell sarcomas are the predominant lymphotropic subtypes and the focus of this review. The utility of lymph node assessment, whether clinical, radiographic, and/or surgical, in patients with these sarcomas is presented. The role of sentinel lymph node biopsy as well as completion lymphadenectomy in patients with these lymphotropic sarcoma histotypes is discussed. Surgery remains the only potential curative option for these tumors, and advancement in histology-specific chemotherapeutic regimens and biologic therapy options is necessary.  This review contains 6 tables and 50 references. Key words: clear cell sarcoma, completion lymphadenectomy, epithelioid sarcoma, EWS-ATF1, limb-sparing surgery, lymph node metastasis, sentinel lymph node biopsy, soft tissue sarcoma

Hyperthermic Antiblastic Perfusion in the Treatment of Cancer of the Extremities

1985 ◽  
Vol 71 (4) ◽  
pp. 355-359 ◽  
Author(s):  
Maurizio Vaglini ◽  
Salvatore Andreola ◽  
Angelo Attili ◽  
Filiberto Belli ◽  
Raffaele Marolda ◽  
...  
Keyword(s):  
Overall Survival ◽  
Squamous Cell Carcinoma ◽  
Clear Cell ◽  
Remission Rate ◽  
Osteogenic Sarcoma ◽  
Major Complication ◽  
Clear Cell Sarcoma ◽  
Clinical Results ◽  
Cell Sarcoma

From February 1982 to December 1983, 42 patients affected by neoplasms of the limbs were treated at the Istituto Nazionale Tumori of Milan by hyperthermic antiblastic perfusion in extracorporeal circulation at the temperature of 40-41 °C for 1 h. Thirty-two were affected by melanoma, 4 by osteogenic sarcoma, 2 by squamous-cell carcinoma, 1 by liposarcoma, 1 by hemangiopericytoma, 1 by clear-cell sarcoma and 1 by Kaposis's sarcoma. As regards the immediate response, a complete plus partial remission rate of 88 % without any major complication was obtained. The follow-up period is too short for any considerations about overall survival. However, because of these good clinical results we consider this method able to locally control the evolution of neoplasms of the extremities, allowing in many cases a limb salvage.

scholarly journals Clear cell sarcoma-like tumor of the gastrointestinal tract presenting as a symptomatic metastasis to the humerus

2018 ◽  
Vol 11 ◽  
pp. 73-75
Author(s):  
Howard J. Lee ◽  
Emily McCracken ◽  
Diana M. Cardona ◽  
Dan G. Blazer
Keyword(s):  
Gastrointestinal Tract ◽  
Clear Cell ◽  
Clear Cell Sarcoma ◽  
Cell Sarcoma
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