Primitive Neuroectodermal Tumour (PNET) in subcutaneous abdominal wall: a case report

Betal, Dibendu; Shaygi, Behnam; Babu, R; Jamil, Kamarul; Sainsbury, Richard

doi:10.1186/1477-7800-6-10

Cited by 9 publications

(5 citation statements)

References 21 publications

(14 reference statements)

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“…Anatomic site of extraskeletal ES in the preperitoneal fat tissue of the abdominal wall in our patient is exceptionally rare, considering that the most common locations of this highly aggressive neoplasm are the thoracic wall, paravertebral region, lower extremities, head, neck and pelvic region [1,2]. However, there are several case reports or small series of extraskeletal ES/PNET with primary location in the kidney, lung, pancreas, uterus, urinary bladder, ovary, testis, parotid gland, mesenterium, skin, and subcutaneous tissue [4,5]. After an extensive literature search on the PubMed, using keywords "Ewing sarcoma, " "primitive neuroectodermal tumor, " and "abdominal wall, " we found only three cases of neoplasms in this primary location [6,7,8].…”

Section: Discussionmentioning

confidence: 91%

“…After an extensive literature search on the PubMed, using keywords "Ewing sarcoma, " "primitive neuroectodermal tumor, " and "abdominal wall, " we found only three cases of neoplasms in this primary location [6,7,8]. Another three cases of extraskeletal ES in the abdominal wall location were reported, but all of them were described as tumors confined to the subcutaneous adipose tissue, so we consider that they should not be included in the group of abdominal wall neoplasms [4,9,10].…”

Section: Discussionmentioning

confidence: 95%

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Extraskeletal Ewing sarcoma in the anterior abdominal wall

Savić

Djuricic²,

Milickovic

et al. 2018

Srp Arh Celok Lek

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Introduction Extraskeletal Ewing sarcoma (ES) is a highly malignant neoplasm occurring most commonly in the thoracic wall and the paravertebral region. ES belongs to the group of small round cell tumors and displays pathognomonic structural abnormalities of the EWS gene. The aim of this article was to present extraskeletal ES in an extremely rare anatomic location, an unusual clinical presentation, and modified treatment strategy. Case outline A 15-year-old boy was admitted to the hospital with acute abdominal pain in the right iliac region. During urgent operation, because of suspected appendicitis with periappendicular infiltrate, partly hemorrhagic tumor tissue was discovered in the preperitoneal space. Histopathological and immunohistochemical analyses revealed a tumor resembling extraskeletal ES. A postoperative CT scan showed the tumor rest, which was completely removed in the second operation. Molecular genetic analysis confirmed extraskeletal ES by finding the EWSR1-FLI1 fusion gene. Chemotherapy and radiotherapy according to the VAC protocol were started, and the patient is free of the disease eight months after the first operation. Conclusion Our case is the fourth case of extraskeletal ES located in the abdominal wall, the second case confirmed by the molecular genetic finding, and the first case described in children at this anatomic site. Due to an extremely rare location, unusual clinical presentation, and needed genetic analysis, the tumor treatment strategy was modified with good short-term results.

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Section: Discussionmentioning

confidence: 91%

Section: Discussionmentioning

confidence: 95%

Extraskeletal Ewing sarcoma in the anterior abdominal wall

Savić

Djuricic²,

Milickovic

et al. 2018

Srp Arh Celok Lek

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“…PNET is a malignant neoplasm included in the Ewing’s sarcoma (ES) family of tumors owing to its biological similarities with osseous and extraosseous tumors arising from germinal matrix cells of the primitive neural tube [ 4 ]. Peripheral PNETs have primarily been observed in children in different organ systems, including bone [ 5 , 6 ], mandible [ 7 ], the maxillofacial area [ 8 ], eye [ 9 ], parathyroid gland [ 10 ], thymus [ 11 ], thorax [ 1 , 12 ], lung [ 13 ], urogenital tract [ 14 – 17 ], adrenal gland [ 18 ], pancreas [ 19 ], chest wall [ 20 ], and subcutaneous abdominal wall [ 21 ].…”

Section: Discussionmentioning

confidence: 99%

Peripheral primitive neuroectodermal tumor: a case report

et al. 2022

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Background Primitive neuroectodermal tumors are extremely rare and highly aggressive malignant small round cell tumors that arise from the primitive nerve cells of the nervous system or outside it. These tumors share similar histology, immunohistologic characteristics, and cytogenetics with Ewing’s sarcoma. Peripheral primitive neuroectodermal tumors of the chest wall are rare malignant tumors seen in children and young adults. Case presentation We report a rare case of peripheral primitive neuroectodermal tumor in a 4-year-old Albanian girl with a mediastinal tumor and an unusual clinical presentation. She was initially treated for acute polyradiculoneuritis (Guillain–Barré syndrome) owing to pain, weakness in the lower limbs, and walking difficulty, as well as severe irritability. During the second week of treatment, the child began to experience dry cough, chest discomfort, and worsening dyspnea. Chest radiography, chest computed tomography, and contrast-enhanced computed tomography demonstrated a large mass in the right hemithorax that was derived from the posterior mediastinum with expansive growth in all directions and that shifted the mediastinal structures in the anterolateral left direction. Consequently, histopathology and immunohistochemical examination of the markers S-100, CD99, and Ki-67 showed that the tumor cells stained positively for S-100 and CD99. The proliferative index measured by Ki-67 was approximately 20%, which suggested primitive neuroectodermal tumor. Conclusions Even though other diseases, including leukemia, lymphoma, and neuroblastoma, may be accompanied by musculoskeletal manifestations in children, other solid tumors, such as peripheral primitive neuroectodermal tumors, should be considered in the differential diagnosis in any child presenting with musculoskeletal symptoms.

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“…O caso em questão descreve um Tumor Neuroectodérmico Primitivo (PNET), tumor de partes moles de baixa incidência na população, em especial em adultos. Os locais mais acometidos, além do sistema nervoso central, são em parede torácica e partes moles de extremidades 7 , com poucos casos descritos em musculatura de parede abdominal 3,4,5,6 .…”

Section: Discussionunclassified

“…Normalmente encontrado em sistema nervoso central, também pode estar localizado em partes moles e ossos 2 . É um tumor raro, cuja incidência é maior em crianças 3 . O PNET possui sutil distinção com Sarcoma de Ewing, sendo o diagnóstico realizado a partir da imunohistoquímica, microscopia eletrônica e estudos de biologia molecular.…”

Section: Introductionunclassified