Radiologic Findings of Peripheral Primitive Neuroectodermal Tumor Arising in the Retroperitoneum

Kim, Mi Sung; Kim, Bohyun; Park, Chan Sup; Song, Soon Young; Lee, Eun Ja; Park, Noh Hyuck; Kim, Hye-Seong; Kim, Seung Hyup; Cho, Kyoung Sik

doi:10.2214/ajr.04.1688

Cited by 54 publications

(50 citation statements)

References 14 publications

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“…A similar presentation of renal PNET with pulmonary embolism was reported in a 30-year-old male and 21-year-old female patient, with tumor resection and thrombectomy done in both cases and later confirmed on histopathology. A good chemotherapeutic response with over a 1 year disease free post operative period was mentioned in the female patient [4] . Renal PNET mostly presents with nonspecific symptoms such as abdominal pain, palpable masses or hematuria.…”

Section: Discussionmentioning

confidence: 87%

“…Skin, soft tissue and viscera (kidney, lungs, adrenal) and the retroperitoneum are less commonly affected [3] . ES/PNET of the kidney with pulmonary tumor embolism is very unusual, with only two cases reported to date [4,5] . A similar presentation of renal PNET with pulmonary embolism was reported in a 30-year-old male and 21-year-old female patient, with tumor resection and thrombectomy done in both cases and later confirmed on histopathology.…”

Section: Discussionmentioning

confidence: 99%

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Peripheral primitive neuroectodermal tumor of the kidney presenting with pulmonary tumor embolism: A case report

Chinnaa

Das

Sharma

et al. 2014

WJR

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Peripheral primitive neuroectodermal tumor (PNET) of the kidney is a rare, aggressive tumor known for its recurrence and metastatic potential. Despite the frequency of venous extension to the renal veins and inferior vena cava, pulmonary tumor embolism at the initial presentation is not common. We report a case of 22-year-old female with PNET of the kidney who presented with tumor embolism in the inferior vena cava (IVC) and bilateral pulmonary artery. The patient underwent surgical resection and histopathological analysis confirmed the presence of tumor within the IVC and pulmonary arteries. The patient received adjuvant chemotherapy and is currently doing well on follow-up. Core tip: Traditionally, an aggressive renal mass with pulmonary tumor embolism is an entity well described in renal cell carcinomas. However, renal primitive neuroectodermal tumor (PNET), a rare and aggressive tumor, can have a similar presentation. The severity of the clinical manifestations of pulmonary tumor embolism is highly variable and less predictable. Bland thrombus often mimics tumor emboli in imaging and histopathology is often confirmatory. Tumoral extension into the pulmonary artery is not necessarily associated with pulmonary metastasis. Surgical removal of the thrombus along with the primary tumor followed by adjuvant chemotherapy may prolong the survival in these patients.Chinnaa S, Das CJ, Sharma S, Singh P, Seth A, Purkait S, Mathur SR. Peripheral primitive neuroectodermal tumor of the kidney presenting with pulmonary tumor embolism: A case report. World

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Section: Discussionmentioning

confidence: 87%

Section: Discussionmentioning

confidence: 99%

Peripheral primitive neuroectodermal tumor of the kidney presenting with pulmonary tumor embolism: A case report

Chinnaa

Das

Sharma

et al. 2014

WJR

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“…Although once viewed as distinct entities, Ewing's sarcoma, Askin's tumor, and PNET are now considered together as members of the Ewing family of tumors [9]. pPNET is a rare malignancy that comprises only around 1 % of all the sarcomas [10] with a 2 year survival rate of 65 % [11]. The poor prognosis is secondary to the high rate of metastasis of 14-50 % at the time of diagnosis of primary tumor [12].…”

Section: Discussionmentioning

confidence: 99%

Mandibular Peripheral Primitive Neuroectodermal Tumor: A Rare Case Report with Review of Literature

Lakshminarayana

Pendem

Chatni³

et al. 2013

J. Maxillofac. Oral Surg.

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Primitive neuroectodermal tumor (PNET) is a high grade malignant neoplasm of small round cell tumor family, commonly affecting children and young adults. Peripheral primitive neuroectodermal tumor (pPNET) is a predominately neural, nonepithelial malignancy seen outside the nervous system that can arise in any place throughout the body including the diverse tissues of the head and neck. The diagnosis of PNET is confounded by its clinical and histopathological similarity to Ewing's sarcoma of the bone and has seldom been reported in the literature. The paucity of literature pertaining to the successful diagnosis and management of this lesion mandates its documentation and discussion. This article describes a case of an 11-year-old boy with an aggressive pPNET of the mandible. The clinical and radiographic presentations of this rare entity along with a detailed review on the current management modalities have been discussed.

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“…Peripheral PNET is uncommon, accounting for only about 1% of all sarcomas. 2 The incidence of peripheral PNET/EWS in the abdomen and pelvis, including the retroperitoneum, is about 14% of all peripheral PNET/EWSs. It has rarely been described in the genitourinary system, including kidney, bladder, prostate, testis, ovary, and uterus.…”

Section: Introductionmentioning

confidence: 99%

“…It has rarely been described in the genitourinary system, including kidney, bladder, prostate, testis, ovary, and uterus. 2 The peak incidence of PNET/EWS is in adolescence and young adulthood, with most reported patients under 35. 3 Peripheral PNET/EWS arises outside the central and sympathetic nervous systems and differs from central PNET in that peripheral PNET/EWS typically expresses high amounts of the MIC2 antigen (CD99) and exhibits highly characteristic chromosomal translocation.…”

Section: Introductionmentioning

confidence: 99%

Peripheral neuroectodermal tumour of the kidney (Ewing’s sarcoma): Restaging with 18F-fluorodeoxyglucose (FDG)-PET/CT

Öztürk¹

2015

CUAJ

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Primitive peripheral neuroectodermal tumour and Ewing's sarcoma (PNET/EWS) were originally described as two distinct pathologic entities, although both share common stem-cell precursor and unique chromosomal abnormality. Although its incidence has increased recently, its share in all sarcomas is 1%. It is usually seen in men and women in their twenties. We present a case of a 38-year-old woman with a left renal mass detected incidentally. Magnetic resonance imaging revealed a centrally located hypervascular renal mass with diameter of 6 cm with non-homogenous contrast enhancement containing necrotic and calcific areas. The patient was diagnosed as having PNET/EWS by histopathological examination following radical nephrectomy. Para-aortic lymph node metastasis was found on imaging by

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Radiologic Findings of Peripheral Primitive Neuroectodermal Tumor Arising in the Retroperitoneum

Cited by 54 publications

References 14 publications

Peripheral primitive neuroectodermal tumor of the kidney presenting with pulmonary tumor embolism: A case report

Peripheral primitive neuroectodermal tumor of the kidney presenting with pulmonary tumor embolism: A case report

Mandibular Peripheral Primitive Neuroectodermal Tumor: A Rare Case Report with Review of Literature

Peripheral neuroectodermal tumour of the kidney (Ewing’s sarcoma): Restaging with 18F-fluorodeoxyglucose (FDG)-PET/CT

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