Teaching Case

A 37-year-old woman with diarrhoea and abdominal cramps lasting for 15 days.

A 37-year-old female patient was admitted to our department for diarrhoea and abdominal cramps lasting for 15 days. Ultrasonography was performed showing multiple hepatic hypoechoic lesions (Fig. 1). Total body CT scan confirmed hepatic lesions that appeared hypodense in precontrast phase, hyperdense in arterial phase and hypodense in portal phase and also detected obstruction of the right main bronchus and right upper lobe atelectasis (Fig. 2). OctreoScan examination demonstrated abnormal tracer accumulation in the anatomic region of the liver and in the right lung (Fig. 3). The hepatic lesions, on biopsy, were consistent with atypical carcinoid tumour. The patient immediately underwent a lobectomy of the right upper lobe (Fig.4). Biopsy specimen confirmed the diagnosis of atypical carcinoid (well differentiated), on the basis of number of mitosis and proliferation index, instead morphology was suggestive for the typical form.
Post-surgical total body CT scan showed no regional relapse (Fig. 5). The liver metastases were treated with somatostatin analogs.

Bronchial carcinoid tumours are rare neuroendocrine tumours, accounting for up to 2.5% of all pulmonary neoplasms. They originate from the neurosecretory cells of bronchial mucosa. Two different types have been identified. The first and most common is referred to as typical carcinoid. It is a low-grade tumour, with 10-year survival rate approaching 90%. It is capable of local invasion, including invasion of local lymph-nodes, but rarely metastasizes. The second type, atypical carcinoid, is much more aggressive and carries a 5-year survival rate of 25% to 69% [1].
It metastatizes in 70% of the cases to regional lymph-nodes, liver, bone or brain. Both subtypes tend to arise from the bronchial tree and spread by local invasion. Typical carcinoid tumour is more commonly found centrally within the major bronchi, whereas the atypical carcinoid tends to arise from the peripheral and central bronchi with equal frequency. The typical carcinoid tumour shows insular and trabecular foci of uniform cells with regular central nuclei and a faintly eosinophilic cytoplasm. There is no significant cytological atypia or nuclear pleomorphism and mitoses are few. On the other hand, the atypical variant of bronchial carcinoid tumour is characterised by disorganised histological structure with increased cellularity, cellular and nuclear pleomorphism, increased mitoses and focal necrosis [2, 3]. The most frequently (58%) presenting symptoms of carcinoid are lobar obstruction, haemoptysis, dyspnoea, cough, and lobar pneumonia secondary to obstruction. Some are found on chest radiographs in asymptomatic patients. Rarely (1-3%) patients may present symptoms related to serotonin secretion (diarrhea, flushing, wheezing, and carcinoid heart disease) or other hormonally active tumor products such as Cushing's syndrome, hypercalcaemia, hypoglycemia and acromegaly and this reflects liver metastases. Typically, the carcinoid syndrome occurs when hepatic spread results in hormonally active tumor products exceeding the hepatic capacity for degradation [4]. Both types of carcinoid tumours of the lung can occur in patients of any age, without gender preference. The role that smoking plays in the development of carcinoids is still under debate. Chest radiography is the first-line imaging investigation in most patients, is abnormal in 90% of patients with bronchial carcinoids. Computed tomography (CT) is useful for detecting lesions not visible on CXR, for assessing endobronchial lesions, and for characterising and staging tumours. These findings include dense ossification, scattered calcifications, and intraluminal location [5]. Nuclear medicine studies (octreoscan) hold great promise not only for diagnosing and staging of tumours but also for predicting the potential response to somatostatin analogues and other therapeutic radioligands. Definitive diagnosis and classification is made by biopsy and pathologic examination of the tissue. Due to its predilection for arising from the luminal surface of the major bronchi, adequate tissue samples can often be obtained through endobronchial biopsy. Alternatively, tissue may be obtained through CT-guided fine-needle biopsy or thoracotomy. Carcinoid is treated surgically, with the approach dependent on the size, location, and tissue type. Chemotherapy has not been demonstrated to be successful as a treatment option but, rather, is limited to a palliative role in the management of diffuse metastatic carcinoid.