img
Dr. Pankaj Kumar Pande

Dr. Pankaj Kumar Pande
Sr. Consultant
Surgical Oncology
BLK Cancer Centre
BLK Super Speciality
Hospital, New Delhi












Rarest of the Rare

A Pathologic Surprise in the form of
Neuroendocrine Tumour

Primary Hepatic Neuroendocrine Tumours are extremely rare, with only about 150 reported cases in the world. Most primary Hepatic Neuroendocrine Tumours occur mostly in middle-aged females. Effective treatment methods for cure includes, Surgical Resection, Liver Transplantation, Somatostatin Analogues and Chemotherapy.

THE CASE

A 58 year old lady from Afghanistan came to BLK Super Speciality Hospital, New Delhi with complaints of pain over her right upper abdomen for the past 4 months with intermittent non-bilious vomiting. On examination she had a palpable lump in the epigastium of about 5 x 4 cms in size. Triphasic whole body PET CT scan revealed a large mass lesion with no abnormal FDG uptake in left lobe of liver measuring 70 x 70 x 72 mm3. Few cystic enhancing areas were seen within. Non FDG avid large heterogeneous mass lesion with associated necrosis, fat, areas of enhancement and calcifi cation were seen in the left side pelvic cavity of 74 x 71 mm2 dimension - which is a likely ovarian dermoid cyst. No other metabolic activity elsewhere were noticed in the body.

THE PROCEDURE

The patient was planned for surgery, i.e. Exploratory Laparotomy. Excision of left ovarian mass with frozen section was done, which revealed a dermoid cyst. Type 1 Hysterectomy and right Salpingoophorectomy along with left Lobectomy liver with excision of hepatic node was performed. Histoptholgy revealed Neuroendocrine Tumour grade 2 and positive Hepatic Node and Cystic Teratoma of ovary.

THE RESULT

The patient withstood the procedure well and had a steady recovery post-operatively. She was discharged on seventh post-operative day. She was advised to undergo a DOTA-PET on her next follow-up to rule out Metastatic Neuroendocrine Tumour.

DISCUSSION

Mature Cystic Teratoma of the ovary is almost always benign, but in approximately 0.2-2% of cases, it may undergo malignant transformation. Surgery offers the best chance of cure. Primary Hepatic Neuroendocrine Carcinoma should be considered as a possible differential diagnosis in the management of Hepatic Tumours. The liver can be the primary origin of Neuroendocrine Tumours and if the tumours are diagnosed as primary Hepatic Neuroendocrine Tumours, surgical resection must be considered for curative treatment.