Epithelioid Sarcoma—From Genetics to Clinical Practice

Epithelioid sarcoma is a mesenchymal soft tissue sarcoma often arising in the extremities, usually in young adults with a pick of incidence at 35 years of age. Epithelioid sarcoma (ES) is characterized by the loss of SMARCB1/INI1 (integrase interactor 1) or other proteins of the SWI/SNF complex. Two distinct types, proximal and distal, with varying biology and treatment outcomes, are distinguished. ES is known for aggressive behavior, including a high recurrence rate and regional lymph node metastases. An optimal long-term management strategy is still to be defined. The best treatment of localized ES is wide surgical resection. Neo-adjuvant or adjuvant radiotherapy may be recommended, as it reduces the local recurrence rate. Sentinel lymph node biopsy should be considered in ES patients. Patients with metastatic ES have a poor prognosis with an expected median overall survival of about a year. Doxorubicin-based regimens are recommended for advanced ES. Tazemetostat, an EZH2 methyltransferase, has shown promising results in ES patients. Novel therapies, including immunotherapy, are still needed.

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Long‐term resolution of invasive extramammary Paget’s disease with multiple regional lymph node metastases solely with regional lymph node dissection

The Journal of Dermatology ◽

10.1111/1346-8138.16007 ◽

2021 ◽

Author(s):

Yu Kawahara ◽

Yoshiyasu Umeda ◽

Buntaro Yamaguchi ◽

Anna Kamimura ◽

Yukiko Teramoto ◽

...

Keyword(s):

Lymph Node ◽

Lymph Node Dissection ◽

Lymph Node Metastases ◽

Regional Lymph Node ◽

Paget’S Disease ◽

Node Dissection ◽

Regional Lymph Node Dissection ◽

Node Metastases ◽

Extramammary Paget's Disease

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The risk of regional lymph node metastases in patients with melanoma less than 1.0 mm thick: recommendations for sentinel lymph node biopsy

Journal of the American College of Surgeons ◽

10.1016/s1072-7515(03)00432-0 ◽

2003 ◽

Vol 197 (3) ◽

pp. 403-407 ◽

Cited By ~ 25

Author(s):

John L Zapas ◽

H Christopher Coley ◽

Sandra L Beam ◽

Sally D Brown ◽

Kathleen A Jablonski ◽

...

Keyword(s):

Lymph Node ◽

Sentinel Lymph Node ◽

Sentinel Lymph Node Biopsy ◽

Lymph Node Metastases ◽

Regional Lymph Node ◽

Lymph Node Biopsy ◽

Node Biopsy ◽

Node Metastases

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Long-Term Recurrence-Free Survival After Metachronous Surgery of the Stomach and Liver for Gastric Adenocarcinoma and Multiple, Synchronous Liver Metastases: A Case Report and Review of Literature

International Surgery ◽

10.9738/intsurg-d-12-00015.1 ◽

2013 ◽

Vol 98 (3) ◽

pp. 241-246 ◽

Cited By ~ 1

Author(s):

Yoshito Kiyasu

Keyword(s):

Lymph Node ◽

Liver Metastases ◽

Gastric Adenocarcinoma ◽

Lymph Node Metastases ◽

Regional Lymph Node ◽

Term Survival ◽

Severe Diarrhea ◽

Regional Lymph Node Dissection ◽

Node Metastases

Abstract This report describes a 58-year-old woman with gastric adenocarcinoma and liver metastases, who survives for more than 18 years after diagnosis. At diagnosis and first surgery, a moderately differentiated gastric adenocarcinoma with subserosal invasion was detected, along with 2 regional lymph node metastases and 2 liver metastases. She underwent gastrectomy and regional lymph node dissection but did not undergo liver operation then. After gastrectomy, she received adjuvant chemotherapy for 1 month but discontinued it due to severe diarrhea. Another metastasis in another area of the liver was detected, for which she underwent excision of the right lobe of the liver (subsegments 5, 6, and 7) about 30 months later. No signs of recurrence have been detected for 18 years (as of March 2012). This patient represents a rare case of long-term survival of gastric adenocarcinoma without recurrence after surgical treatment, despite multiple, synchronous, liver and regional lymph node metastases.

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