Resection and reconstruction of a giant primitive neuroectodermal tumour of the abdominal wall with an ultra-long lateral circumflex femoral artery musculocutaneous flap: a case report

Background Primitive neuroectodermal tumours are clinically rare. Here, we report a case of a large peripheral primitive neuroectodermal tumour of the abdominal wall. The defect was reconstructed with the longest lateral circumflex femoral artery musculocutaneous flap reported to date. Case presentation A 15-year-old male suffered rupture and bleeding of an abdominal wall mass with a volume of approximately 23*18*10 cm3, involving the whole layer of the abdominal wall. Pathological examination revealed a peripheral primitive neuroectodermal tumour. The tumour was removed via oncologic resection, and the abdominal wall was reconstructed with a bilateral 44*8 cm2 lateral circumflex femoral artery musculocutaneous flap combined with a titanium polypropylene patch. The patient had smooth recovery postoperative, and the functions of the donor and recipient areas of the flap were not significantly affected. Conclusion In this case report, we describe a rare primitive neuroectodermal tumour of the abdominal wall, which invaded almost the entire abdominal wall due to delay of treatment. After thoroughly removing the tumour, we immediately reconstructed the abdominal wall with an ultra-long lateral circumflex femoral artery musculocutaneous flap and achieved better appearance and function after the operation. This case suggests that we should adopt an integrated scheme of surgery combined with radiotherapy and chemotherapy in the treatment of peripheral primitive neuroectodermal tumours. Under the premise of determining the blood supply, the lateral circumflex femoral artery musculocutaneous flap can be cut to a sufficient length.

Primitive neuroectodermal tumour (PNET) of the renal capsule mimicking solid adrenal tumour

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.

Primitive Neuroectodermal Tumour Invading the Inferior Vena Cava

In the present report, we describe our experience with a 44-year-old male with abnormal retroperitoneal primitive neuroectodermal tumours (PNETs) in our hospital, who was operated on with a spindle cell neoplasm diagnosis.

Primitive neuroectodermal tumour of the kidney presenting as diplopia secondary to skull metastasis

A 20-year-old man presented to the department of neurology with diplopia, occipital headache and right flank pain for 1-week duration. CT of the brain revealed skull metastasis with heterogeneously enhancing dural-based mass lesion at the occipital region. Positron emission tomography revealed tracer avid soft tissue mass involving the upper pole of the right kidney with loss of fat planes with the inferior surface of the liver. Multidisciplinary team approach was discussed. He underwent palliative nephrectomy with lymph nodal mass excision. Biopsy from the renal mass was suggestive of primitive neuroectodermal tumour. He developed progressive liver metastases in spite of adjuvant chemotherapy denoting very aggressive disease.