Rarest of the Rare
A Pathologic Surprise in the form of
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.
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 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
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.
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.