No abstract

The identification of BBS is crucial to avoid serious complications including sepsis and peritonitis. The diagnosis can be made clinically or from endoscopy. The exact site of the migrated PEG internal bumper can be demonstrated on imaging study.

This is a case of a 62-year-old Indian man who was diagnosed with a rare type of lung neuroendocrine tumour (NET) of atypical carcinoid (AC) subtype which comprise only 0.1%–0.2% of pulmonary neoplasms. He initially presented to a private hospital in May 2018 with a 6-month history of chronic productive cough and haemoptysis. Chest X-Ray (CXR), CT scan, bronchoscopy, biopsy and broncho-alveolar lavage were conducted. At this stage, imaging and histopathological investigations were negative for malignancy. Diagnosis of bronchiectasis was made and he was treated with antibiotic and tranexamic acid. Due to financial difficulties, his care was transferred to a university respiratory clinic in June 2018. His condition was monitored with CXR at every visit and treatment with tranexamic acid was continued for 6 months. However, due to persistent haemoptysis, he presented to the university primary care clinic in Dec 2018. Investigations were repeated in January 2019 where his CXR showed increased opacity of the left retrocardiac region and CT scan revealed a left lower lobe endobronchial mass causing collapse with mediastinal lymphadenopathy suggestive of malignancy. Bronchoscopy, biopsy and histopathology confirmed the presence of NET. Although the Ki-67 index was low, the mitotic count, presence of necrosis and evidence of liver metastases favoured the diagnosis of AC. A positron emission tomography Ga-68 DONATOC scan showed evidence of somatostatin receptor avid known primary malignancy in the lungs with suspicions of liver metastasis. He was subsequently referred to the oncology team and chemotherapy was initiated. This case highlights the challenge in diagnosis and management of patients with AC. Physicians ought to be vigilant and have a high index of suspicion in patients who present with persistent symptoms on multiple visits. Early diagnosis of NET would prevent metastasis and provide better prognosis. Continuous follow-up shared care between primary care and secondary care physicians is also essential to provide ongoing psychosocial support for patients with NET, especially those with metastatic disease.

Objective: To report a diagnostic challenge of primary sphenoid adenoid cystic carcinoma. Case summary: A 60-year-old premorbid healthy lady presented with progressive deterioration of visual acuity of the left eye associated with diplopia, left epiphora and left ear blockage for two weeks duration. Examination revealed a 'non perception of light' (NPL) of the left eye with left abducent nerve palsy. The nasoendoscopic findings were unremarkable. Initial computed tomography of the brain and paranasal sinus showed a large clivus tumor with intracranial extension while subsequent magnetic resonance detected the epicenter of the tumour appeared to be in the sphenoid sinus with extension to the surrounding structures. A transeptal transsphenoidal biopsy was done reported as mixed pattern adenoid cystic adenocarcinoma. Discussion: Primary sphenoid adenoid cystic carcinoma is an extremely rare slow growing malignancy with non-specific clinical symptoms. The neuro-ophthalmology symptoms are the main presentation. Combined computed tomography and magnetic resonance images are essential in establishing differential diagnosis and to delineate the extent of this disease. Combined modality of surgery and postoperative radiation for adenoid cystic carcinoma has proven better results. Conclusions: Primary sphenoid adenoid cystic carcinoma poses a diagnostic challenge clinically. Combined radiological characteristic from computed tomography and magnetic resonance images are essential to aid the diagnosis.