Pure Small Cell Neuroendocrine Carcinoma of Urinary Bladder: a Rare Entity
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CASE REPORT
Pure Small Cell Neuroendocrine Carcinoma of Urinary Bladder: a Rare Entity Shashikant Singh 1 & Arvind Ahuja 1
&
Rohan Sardana 2 & Anurag Singla 3
Received: 28 April 2020 / Accepted: 19 August 2020 # Indian Association of Surgical Oncology 2020
Introduction
Case Report
Neuroendocrine carcinoma of bladder is a rare malignancy of urinary bladder accounting for 0.35–0.70% of all the bladder carcinoma [1]. These carcinomas develop from epithelium rich in enterochromaffin cells. According to the 2016 World Health Organization Classification of Tumours of Urinary System and Male Genital Organ, neuroendocrine tumors are divided into four subtypes, namely, small cell neuroendocrine carcinoma, large cell neuroendocrine carcinoma, welldifferentiated neuroendocrine tumor, and paragangliomas [2]. Small cell carcinoma of the bladder (SCCB) is a rare, poorly differentiated neuroendocrine epithelial tumor. It is associated with a more aggressive behavior and poorer outcome as compared with urothelial cell carcinoma of bladder. Here, we report a case of pure small cell neuroendocrine carcinoma of bladder in a male patient who underwent first TURBT followed by radical cystoprostatectomy.
A 52-year-old male presented to the hospital with complaints of burning micturition and painless gross hematuria associated with clots since 7 months. Patient did not have any history of loss of appetite, weight loss, fever, or vomiting. Patient was a chronic bidi smoker for 20 years. There was no significant past medical or surgical history. Complete blood count and biochemical investigations were within normal limits except for mild anemia. Ultrasound abdomen and pelvis showed a heterogenous echo poor mass of size 6.6 × 4.5 cm with irregular margin in the urinary bladder. Contrast enhanced computed tomography (CECT) of abdomen and pelvis showed an intraluminal polypoidal soft tissue mass of size 5 × 4.6 × 4.6 cm along the left posterolateral wall of urinary bladder which appear to involve the seminal vesicles and prostate suggestive of malignant tumor [clinical stage cT4N0M0] (Fig. 1). Chest X-ray performed was normal. Patient underwent incomplete transurethral resection of the bladder tumor (TURBT) with deep muscle biopsy. Histopathological examination revealed a tumor composed of nests of small round malignant cells with pyknotic round to oval nuclei and evenly dispersed salt and pepper chromatin and scant amount of cytoplasm. Few foci of necrosis and increased mitosis were also noted. Tumor was focally infiltrating into lamina propria and deep muscle (Fig. 2). Immunohistochemical (IHC) studies showed positivity for CD56 and synaptophysin. However, tumor was negative for chromogranin. Based on morphology and immunohistochemistry, a diagnosis of small cell carcinoma was given. In view of histological diagnosis, the patient was given 3 cycles of neoadjuvant chemotherapy (etoposide and cisplatin). A repeat CECT abdomen was done post neoadjuvant therapy; however, no response was seen. Thereafter, patient underwent radical cystop
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