Dagmar Adamkova Krakorova1, Jana Halamkova2, Stepan Tucek3, Ondrej Bilek3, Jan Kristek4, Tomas Kazda5, Iva Staniczkova Zambo6, Regina Demlova7, Igor Kiss8. 1. Department of Cancer Comprehensive Care Masaryk Memorial Cancer Institute, Zluty kopec 7. 2. Department of Cancer Comprehensive Care Masaryk Memorial Cancer Institute, Faculty of Medicine Masaryk University Zluty kopec 7, 656 53 Brno, Czech Republic And Department of Medical Ethics, Faculty of Medicine Masaryk University, Kamenice 25. 3. Department of Cancer Comprehensive Care Masaryk Memorial Cancer Institute, Faculty of Medicine Masaryk University Zluty kopec 7. 4. Department of Radiology, Masaryk Memorial Cancer Institute Zluty kopec 7. 5. Assoc. Prof. Department of Radiation Oncology Masaryk Memorial Cancer Institute Faculty of Medicine Masaryk University Zluty kopec 7. 6. Department of Pathology, St. Anne's University Hospital, Faculty of Medicine Masaryk University Pekarska 53. 7. Assoc. Prof. Clinical Trial Unit, Masaryk Memorial Cancer Institute Zluty kopec 7, 656 53 Brno, Czech Republic And Department of Pharmacology, Faculty of Medicine Masaryk University, Kamenice 25. 8. Assoc. Prof. Department of Cancer Comprehensive Care Masaryk Memorial Cancer Institute Faculty of Medicine Masaryk University Zluty kopec 7, Brno, Czech Republic.
Abstract
RATIONALE: The Ewing sarcoma family of malignant tumors is a group of tumors characterized by morphologically similar round-cell neoplasms and by the presence of a common chromosomal translocation; Ewing sarcoma family of tumors typically occur in children and young adults between 4 to 15 years of age. The primary tumor usually originates in the bone, extraskeletal localization is rare. PATIENT CONCERN: We present a case report concerning a 32-year-old male patient with a primary involvement of the penis. DIAGNOSIS: The histopathology from the first penile biopsy showed a small-cell neuroendocrine carcinoma; however, that result was based on a sample obtained at a different facility than the Sarcoma Center, where the investigating pathologist did not have the adequate expertise. The patient then underwent a radical penectomy and a second reading of the histology was demanded after a radical penile amputation when Ewing sarcoma with R1 resection was confirmed. INTERVENTIONS: The patient was referred to the national Sarcoma Center, where - using a multidisciplinary approach - the treatment was started with curative intent. However, it was preceded by a non-standard initiation of the therapy due to the poor primary diagnosis. OUTCOMES: The non-standard therapy at the onset of the disease caused a poor prognosis of an otherwise curable diagnosis. Despite all that, the patient survived for a relatively long time. LESSONS: The treatment of sarcomas with atypical localizations should be conducted by an experienced multidisciplinary team in a center with experience in sarcoma treatment.
RATIONALE: The Ewing sarcoma family of malignant tumors is a group of tumors characterized by morphologically similar round-cell neoplasms and by the presence of a common chromosomal translocation; Ewing sarcoma family of tumors typically occur in children and young adults between 4 to 15 years of age. The primary tumor usually originates in the bone, extraskeletal localization is rare. PATIENT CONCERN: We present a case report concerning a 32-year-old male patient with a primary involvement of the penis. DIAGNOSIS: The histopathology from the first penile biopsy showed a small-cell neuroendocrine carcinoma; however, that result was based on a sample obtained at a different facility than the Sarcoma Center, where the investigating pathologist did not have the adequate expertise. The patient then underwent a radical penectomy and a second reading of the histology was demanded after a radical penile amputation when Ewing sarcoma with R1 resection was confirmed. INTERVENTIONS: The patient was referred to the national Sarcoma Center, where - using a multidisciplinary approach - the treatment was started with curative intent. However, it was preceded by a non-standard initiation of the therapy due to the poor primary diagnosis. OUTCOMES: The non-standard therapy at the onset of the disease caused a poor prognosis of an otherwise curable diagnosis. Despite all that, the patient survived for a relatively long time. LESSONS: The treatment of sarcomas with atypical localizations should be conducted by an experienced multidisciplinary team in a center with experience in sarcoma treatment.
Authors: Mark L Bernstein; Meenakshi Devidas; Dominique Lafreniere; Abdul-Kader Souid; Paul A Meyers; Mark Gebhardt; Kimo Stine; Richard Nicholas; Elizabeth J Perlman; Ronald Dubowy; Irving W Wainer; Paul S Dickman; Michael P Link; Allen Goorin; Holcombe E Grier Journal: J Clin Oncol Date: 2006-01-01 Impact factor: 44.544
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Authors: A Zoubek; C Pfleiderer; M Salzer-Kuntschik; G Amann; R Windhager; F M Fink; E Koscielniak; O Delattre; S Strehl; P F Ambros Journal: Br J Cancer Date: 1994-11 Impact factor: 7.640