Primitive neuroectodermal tumor of the kidney at the advanced stage: A case series of eight Chinese patients

Sun, Qipeng; Miao, Benchun; Lao, Xiang-Ming; Yuan, Ping; Cai, Jiarong; Zhan, Hong

doi:10.3892/mco.2018.1611

Cited by 4 publications

(8 citation statements)

References 22 publications

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“…The presenting symptoms of renal PNET are nonspecific, including flank pain, abdominal mass, hematuria, and other symptoms related to genitourinary infections ( 9 ). A retrospective study done by Sun et al showed most patients (87%) having renal PNET on the left side, but our study and case series conducted by Narayanan et al revealed it to be on the right kidney in most of the patients ( 10 , 11 ).…”

Section: Discussioncontrasting

confidence: 73%

“…In a retrospective study conducted by Sun et al, four cases received adjuvant chemotherapy. They had a significantly better overall survival of 36 months, compared with an overall survival of 10 months in the three patients without adjuvant chemotherapy ( 10 ). In the present study, one of the patients with the localized disease received chemotherapy, that is, VAC/IE of a total of 17 cycles and following up regularly with remission for the last 2 years and 4 months.…”

Section: Discussionmentioning

confidence: 99%

“…Sun et al studied the role of cytokine-induced killer (CIK) cell immunotherapy in a patient of renal PNET ( 10 ). They noted a survival advantage of 20 months with lesser adverse events with this form of therapy in comparison to chemotherapy.…”

Section: Discussionmentioning

confidence: 99%

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Do Primitive Neuroectodermal Tumors of the Kidney Have a Predilection for Inferior Vena Cava Involvement? A Case Series and Review of the Literature

Hota

Kalra

Dorairajan

et al. 2020

jkcvhl

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The primitive neuroectodermal tumor (PNET) of the kidney is an extremely rare neoplasm, the diagnosis of which mainly depends upon histopathology, immunohistochemistry (IHC), and cytogenetics. A handful of cases reported in the literature mention about aggressive features of this neoplasm. The purpose of our study was to review our experience in not only the diagnosis and management of the patients with renal PNET but also to highlight its propensity to involve inferior vena cava (IVC) and also present a rare occurrence of Ewing’s sarcoma (ES)/PNET of the renal pelvis. The clinical, operative, and histopathology records of four patients of renal PNET treated between January 2017 and December 2019 were reviewed and data analyzed concerning the available literature. Out of the four patients treated, two had level III and IV IVC thrombus, and one had dense desmoplastic adhesions with the IVC wall. One of the cases had a rare presentation of ES/PNET of the renal pelvis. All patients were managed surgically, while only one patient received adjuvant chemotherapy and following up with remission for the last 2 years and 4 months. On IHC, cluster of differentiation-99 (CD-99) was positive in all patients, and three were positive for Friend leukemia integration-1. PNET of the kidney is primarily an immunohistopathological diagnosis. This neoplasm has an increased propensity for the local invasion of surrounding structures. A multimodality approach with surgery, chemotherapy, and radiotherapy could offer better outcomes, although the prognosis of these tumors remains poor.

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Section: Discussioncontrasting

confidence: 73%

Section: Discussionmentioning

confidence: 99%

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Do Primitive Neuroectodermal Tumors of the Kidney Have a Predilection for Inferior Vena Cava Involvement? A Case Series and Review of the Literature

Hota

Kalra

Dorairajan

et al. 2020

jkcvhl

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“…Effective treatment methods include a combination of surgery, chemotherapy and radiotherapy. VDC (vincristine, doxorubicin, cyclophosphamide) alternating with the use of IE (ifosphamide, VP-16) in combination is currently the recommended treatment [ 26 ].…”

Section: Discussionmentioning

confidence: 99%

Non-Wilms' renal tumors in children: experience with 139 cases treated at a single center

et al. 2022

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Background Pediatric non-Wilms renal tumors (NWRTs), which comprise a small proportion of renal tumors, are a heterogeneous group of neoplasms with variable malignant potential, mortality, and response to treatment. We performed this study to determine the clinical characteristics, management and prognosis of children with Pediatric NWRTs. Methods Medical records of all patients (n = 139) treated for NWRTs over a 12-year period (2008.01–2019.10) at a single center were reviewed retrospectively. Results The histopathological groups of NWRTs included malignant rhabdoid tumor of the kidney (MRTK) (n: 30, 21.6%), renal cell cancer (RCC) (n: 26,18.7%), clear cell sarcoma of the kidney (CCSK) (n: 24,17.3%), congenital mesoblastic nephroma (CMN) (n: 21,15.1%), cystic nephroma (CN) (n: 16,11.5%), metanephric tumors (n: 12, 8.6%), renal angiomyoliporma (RAML) (n: 3, 2.2%), renal primitive neuroectodermal tumor (n: 2, 1.4%), renal hemangioma (n: 2, 1.4%), inflammatory myofibroblastic tumor (n: 2, 1.4%), ossifying renal tumor of infancy (ORTI) (n: 1, 0.7%). The distribution of all malignant NWRTs, including MRTK, CCSK, RCC and PNET, according to stage was as follows: stages I (n = 26), II (n = 16), III (n = 29), and IV (n = 11). The summary table shows the treatment offered to children with NWRTs. A total of 123 children were followed up for an average of 42 months. Sixteen children were lost to follow-up. Tumor-free survival was observed in 94 children. One patient who suffered from RCC is currently receiving targeted therapy and survives with the tumor. Twenty-eight children (22.8%) died. Conclusions Pediatric NWRTs comprise 19.1% of all renal tumors in our single center. Most NWRTs can readily be distinguished using a range of immunohistochemical markers. Molecular genetic profiling has allowed much progress in the understanding of this group of tumors, making diagnosis and classification less difficult. The mainstay treatment of malignant NWRTs, including MRTK, CCSK, RCC and PNET, is comprehensive treatment. The mainstay treatment of benign NWRTs, including RAML, CN, ORTI, CMN, metanephric tumors, and renal hemangioma, is surgical resection alone and when the tumor diameter is smaller than 7 cm and the tumor locates in one pole, NSS can be performed.

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“…In the EFT-2001 protocol, 1800 mg/m 2 of ifosfamide per day for 5 days and 100 mg/m 2 of etoposide per day for the same period are added to the VAC regimen (20). Sun et al studied the role of cytokine-induced killer (CIK) cell immunotherapy in a patient of renal PNET (10). They noted a survival advantage of 20 months with lesser adverse events with this form of therapy in comparison to chemotherapy.…”

Section: Discussionmentioning

confidence: 99%

Do Primitive Neuroectodermal Tumors of the Kidney Have a Predilection for Inferior Vena Cava Involvement?

Hota¹,

Kalra²,

Narayanan³

et al. 2020

jkcvhl

View full text Add to dashboard Cite

The primitive neuroectodermal tumor (PNET) of the kidney is an extremely rare neoplasm, the diagnosis of which mainly depends upon his-topathology, immunohistochemistry (IHC), and cytogenetics. A handful of cases reported in the literature mention about aggressive features of this neoplasm. The purpose of our study was to review our experience in not only the diagnosis and management of the patients with renal PNET but also to highlight its propensity to involve inferior vena cava (IVC) and also present a rare occurrence of Ewing’s sarcoma (ES)/PNET of the renal pelvis.The clinical, operative, and histopathology records of four patients of renal PNET treated between January 2017 and December 2019 were reviewed and data analyzed concerning the available literature. Out of the four patients treated, two had level III and IV IVC thrombus, and one had dense desmoplastic adhesions with the IVC wall. One of the cases had a rare presentation of ES/PNET of the renal pelvis. All patients were managed surgically, while only one patient received adjuvant chemotherapy and following up with remission for the last 2 years and 4 months. On IHC, cluster of differentiation-99 (CD-99) was positive in all patients, and three were positive for Friend leukemia integration-1. PNET of the kidney is primarily an immunohistopathological diagnosis. This neoplasm has an increased propensity for the local invasion of surrounding structures. A multimodality approach with surgery, chemotherapy, and radiotherapy could offer better outcomes, although the prognosis of these tumors remains poor.

show abstract

Primitive neuroectodermal tumor of the kidney at the advanced stage: A case series of eight Chinese patients

Cited by 4 publications

References 22 publications

Do Primitive Neuroectodermal Tumors of the Kidney Have a Predilection for Inferior Vena Cava Involvement? A Case Series and Review of the Literature

Do Primitive Neuroectodermal Tumors of the Kidney Have a Predilection for Inferior Vena Cava Involvement? A Case Series and Review of the Literature

Non-Wilms' renal tumors in children: experience with 139 cases treated at a single center

Do Primitive Neuroectodermal Tumors of the Kidney Have a Predilection for Inferior Vena Cava Involvement?

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