An unusual site for Carcinoid
A Abbara, R Cottle , K Baynes, R Bell, Department of Endocrinology, Ealing Hospital
R Sharma (Department of Cardiology, Ealing Hospital)
M Naughton (Department of Rheumatology, Ealing Hospital)
A Farthing (Department of Gynaecology, Hammersmith hospital),
T Zaman (Department of Orthopaedics, Ealing hospital)
A 64 year old psychiatric nurse, with a history of rheumatoid arthritis, attended a pre-assesment clinic for a right shoulder repair. On examination, she was incidentally noted to have a large pelvic mass and a pan-systolic murmur. A pelvic ultrasound demonstrated a 25x12x18cm right adnexal mass, which was predominantly solid but also contained multiple cystic areas. A CT chest, abdomen and pelvis confirmed the presence of a pelvic mass and did not reveal any other intra-abdominal lesions. An echocardiogram showed a dilated right atrium and ventricle with severe tricuspid regurgitation.
This raised the possibility of carcinoid syndrome and two 24hr urine collections for 5HIAA (urinary 5-hydroxyindoleacetic acid) were found to be markedly raised at 2128 and 2083 (NR 0-100 umol/24hr). A 24hr urine catecholamine collection, contained normal levels of adrenaline, noradrenaline and dopamine, but raised metanephrines (which was felt to be due to cross-reactivity of the assay with 5HIAA). She had a raised chromogranin A at 930pmol/l (NR 0-60), but other gut hormones and tumour markers were normal.
An octreotide scan demonstrated tracer uptake within the pelvic mass and increased joint activity (thought to be due to rheumatoid arthritis). On further questioning, she reported lethargy, weight loss of 1.5 stone, ankle swelling, flushing of the face and sclera over the last year, but denied diarrhoea or wheeze.
She underwent an open total abdominal hysterectomy, bilateral salpingo-oophorectomy and omentectomy, under the cover of a 48 hour octreotide infusion to ensure a haemodynamically stable peri-operative period.
Histology revealed carcinoid tumour of the left ovary measuring 20x18x9cm, not breaching the capsule (pT1a) and a mature cystic teratoma of the right ovary (dermoid cyst). Left ovarian microscopic histology showed atypical cells with a ‘salt and pepper’ appearance, arranged in trabeculae. The tumour cells strongly expressed CAM5.2, chromogranin, CD56, synaptophysin and NSE. Omental samples showed no evidence of metastatic disease, consistent with a carcinoid tumour not breaching the capsule.
Post-operatively her flushing and her ankle oedema remitted and her 24hr urinary 5HIAA came down to 14 (0-100 umol/24hr). A repeat octreotide scan showed no residual disease and a repeat echocardiogram showed a marked improvement in her tricuspid regurgitation.
Ovarian carcinoid tumours are rare constituting less than 0.1% of ovarian tumours and ~0.5-1% of carcinoid tumours. Primary ovarian carcinoid tumours are commonly unilateral, large and occur more commonly in post-menopausal women. There are distinct histological subtypes including insular, trabecular, stromal, mucinous and mixed. An insular pattern, similar to midgut carcinoid, is the most common pattern and carries low malignant potential and is associated with carcinoid syndrome. As the venous drainage from the ovary connects directly into the systemic circulation, ovarian carcinoid may be associated with carcinoid syndrome even in the absence of liver metastases. The trabecular subtype is the rarest subtype and is usually not associated with carcinoid syndrome and carries a good prognosis.
Up to one third of patients with carcinoid tumours may have ‘carcinoid syndrome’ (episodic flushing of the skin, sweating, diarrhoea, abdominal cramping and wheezing). ~60% of patients with carcinoid syndrome develop valvular heart disease, which may significantly impact upon prognosis. In fact cardiac involvement may reduce life expectancy by more than 50%. In cases such as this, in which the tumour is confined to the ovary, prognosis is favourable with a 5 year survival of 95% with surgery alone.
Due to the vigilance of an orthopaedic FY1 doctor during pre-assessment and successful collaboration of cardiologists, endocrinologists, cardiologists, anaesthetists and gynaecology surgeons, this rare tumour was diagnosed and optimally managed.