Primitive neuroectodermal tumour of the Cervix: A rare entity

Primitive neuroectodermal tumour (PNET) of renal capsule is a rare entity. We report a case of a 17-year-old girl, who presented with symptoms of epigastric and right hypochondrium pain since 1 year. She was afebrile and physical examination revealed a soft, non-tender, firm, bimanually palpable and ballotable mass along right flank. Ultrasound abdomen showed a large heteroechoic mass in right suprarenal region with indistinct planes with upper pole of right kidney. On CT, a large right suprarenal mass was noted with origin likely from right adrenal gland. Surgery was done and intraoperatively, the large mass in right suprarenal region showed involvement of the upper pole of the right kidney. The right adrenal gland was small in size, compressed and displaced by the lesion. Histopathology revealed the mass to be PNET of kidney. We report the relevant imaging findings of the case with review of literature of this entity.

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Renal primitive neuroectodermal tumour in childhood: Case report and review of literature

Canadian Urological Association Journal ◽

10.5489/cuaj.969 ◽

2013 ◽

Vol 4 (6) ◽

pp. 158 ◽

Cited By ~ 7

Author(s):

Maeed Asiri ◽

Ahmed Al-Sayyad

Keyword(s):

Axial Skeleton ◽

Pathology Report ◽

Combined Modality Treatment ◽

Rare Entity ◽

Review Of Literature ◽

Rare Tumour ◽

Primitive Neuroectodermal Tumour ◽

Neuroectodermal Tumour ◽

Local Tumour ◽

Renal Pnet

Primitive neuroectodermal tumour (PNET) is presumed to be ofneural crest origin, mostly presenting as bone or soft tissue masses.It usually occurs in the trunk or axial skeleton; while renal PNET isconsidered an extremely rare tumour. We report a case of 11-yearoldmale who presented with right flank pain and gross hematuriaafter suffering blunt trauma. During investigations, he was found tohave a large renal mass on computed tomography. He underwenta right radical nephrectomy where the pathology report showedPNET of the kidney. The patient received chemotherapy afterwards.Despite the chemotherapy, he had a local tumour recurrence 3months after and continued to deteriorate and developed distantmetastasis. Primitive neuroectodermal tumour of the kidney is adistinct and rare entity. It is very aggressive, with a poor survivaldespite combined modality treatment.

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Cervical intraspinal extradural primitive neuroectodermal tumour in an adult

Forum of Clinical Oncology ◽

10.2478/fco-2014-0001 ◽

2014 ◽

Vol 5 (1) ◽

pp. 1-5

Author(s):

Pinelopi V Gogou ◽

Myrsini J Balafouta ◽

Constantinos S Gennatas ◽

Agatha Kondi-Paphiti ◽

Xenophon N. Papacharalampous ◽

...

Keyword(s):

Pain Relief ◽

Therapeutic Approach ◽

Poor Prognosis ◽

Recent Literature ◽

Craniospinal Irradiation ◽

Rare Entity ◽

Primitive Neuroectodermal Tumour ◽

Literature Research ◽

Neuroectodermal Tumour ◽

Progressive Course

Abstract Intraspinal extradural primitive neuroectodermal tumours (PNET) represent a rare entity that almost always carry a poor prognosis. A recent literature research revealed only four cases reported in adults. The tumours have mainly thoracic and lumbar location. We report the only case with cervical location and with the longest survival. Urgent laminectomy was performed resulting in a rapid pain relief for the patient. The patient was followed up with craniospinal irradiation and several cycles of chemotherapy. The patient died due to dissemination of the disease 31 months after the diagnosis. Intraspinal PNET tumours have a rapidly progressive course, and there is no current consensus on the optimal therapeutic approach.

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