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(searched for: Neuroendocrine Small Cell Carcinoma of the Bladder: Rare and Aggressive Entity)
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Valerio Olivieri, Valentina Fortunati, Luca Bellei, Massimo Massarelli, Gabriele Ruggiero, Danilo Abate, Nicoletta Serra, Daniele Griffa, Flavio Forte, Sciprofile linkEmanuele Corongiu
Archivio Italiano di Urologia e Andrologia, Volume 92; doi:10.4081/aiua.2020.3.211

Abstract:
Background: Neuroendocrine tumours (NET) are extremely rare and aggressive. Although they commonly affect intestine, many organs may be involved such as pancreas, lung or urinary tract. Bladder is rarely involved. Actually, two main forms of bladder NET have been described: small-cell and large-cell. The first one is considered highly agressive since it shows poor oncologic outcomes being mainly diagnosed at advanced stage: the second one is extremely rare and equally aggressive. Case report: A 78-years-old Caucasian male presented to our facility for lower urinary tract symptoms and gross hematuria recently occurred. He was a strong smoker since many years. No familiarity for urothelial cancer was referred nor previous episodes of hematuria until that time. Citology was negative; outpatient ultrasound of the bladder revealed a 3 cm bladder thickening highly suspicious for bladder cancer; patient underwent TC scan that confirmed the bladder lesion. A transurethral resection of the bladder (TURB) was performed. After 3 months total body TC showed multiple visceral metastases also involving brain and lymph nodes. Best supportive care was offered but the patient died 6 months later. Results: Pathology revealed a mixed bladder tumor: 30% of the specimen resulted as an high-grade urothelial cancer (G3) and 70% as small-cell neuroendocrine variant.Microscopic muscle involvement was excluded.Conclusions: Neuroendocrine tumors are uncommon entities which origin from cells of neuro-endocrine system and may potentially involve all human tissues. Neuroendocrine smallcell carcinoma of the bladder is a non-urothelial histotype: it is highly aggressive and diagnosed mainly at advanced stages. Whenever considering the high risk of metastatic spread and the poor prognosis, a multimodal approach is highly suggested. TURB alone is uneffective in disease control due to its aggressive nature. Unless metastatic, radical cystectomy and adjuvant chemotherapy represent the gold standard.
Misbah Azmath, Ashley Dunbar, Imran Siddiqui
Journal of the Endocrine Society, Volume 4; doi:10.1210/jendso/bvaa046.1472

Abstract:
Background: Pure large cell neuroendocrine carcinoma of the gall bladder (LCNEC-GB) is an extremely rare entity, with only 12 such cases reported in literature to date. None has been reported in a patient with genetic disorders. We describe the case of a patient with pure LCNEC-GB in the presence of Down syndrome and prior biliary atresia. Case: A 49-year-old female with Down Syndrome and history of neonatal surgery for congenital duodenal atresia presented with fever, vague abdominal discomfort and 6 month history of 15 pound weight loss. CT abdomen revealed a 5 X 4.2 cm exophytic, heterogeneously enhancing mass in the gall bladder fossa extending into segment 4B-5 of liver with mild intrahepatic biliary dilation, along with a 9 cm cystic lesion in continuity with the duodenum which was confirmed to a dilated duodenal anastomosis (from prior biliary surgery) on endoscopy. There was high suspicion for malignancy (gall bladder carcinoma versus intrahepatic cholangiocarcinoma) and subsequent metastatic workup including tumor markers, staging CT chest, MRI of the abdomen and diagnostic laparoscopy was negative. The patient underwent robotic converted to open en bloc resection of the gallbladder mass with segment 4B-5 liver resection and adherent loops of small bowel as well as resection of the dilated duodenal anastomosis followed by reconstruction with a gastrojejunostomy and Roux-en-Y/small bowel entero-enterostomy and closure of duodenotomy. Pathology demonstrated poorly differentiated “pure” large cell neuroendocrine carcinoma (G3), 6 cm in greatest dimension, with invasion of liver, duodenum and stomach, negative liver and gastric/small bowel margins. Lymphovascular invasion (LVI) was present with no perineural invasion (PNI), and 3/15 lymph nodes involved. Staging was determined to be pT4pN1. Patient was considered for adjuvant chemotherapy based on few case reports (platinum/etoposide) but unfortunately developed systemic complaints of fevers, fatigue, pain in bones and joints 2 months post operatively. Workup revealed metastatic disease which was confirmed on biopsy. Several small satellite liver lesions were also identified which was consistent with metastatic hepatic disease. Patient and family elected to proceed with hospice. Patient died within 4 months of surgery. Conclusion: Pure LCNEC-GB is an extremely rare and aggressive tumor with a poor prognosis as seen in our patient. This is the first reported case of pure LCNEC-GB in a patient with a genetic syndrome, although it is unknown if it had any causal relationship with the tumor. Prior biliary atresia/post -surgical inflammation may have also contributed to its pathogenesis by plausible development of metaplasia and expression of neuroendocrine cells which are normally absent in the gall bladder. Our case might help shed some light into pathogenesis and genetic basis if any of this rare entity.
M. Rkik, M. Modeste, A. Ettanji, A. Kbiro, M. Dakir, R. Aboutaib, A. Debbaghi
Archives of Nephrology and Urology, Volume 3, pp 1-4; doi:10.26502/anu.2644-2833014

Abstract:
Neuroendocrine small cell carcinoma of the bladder is a rare cancer, rapidly aggressive and metastatic with a very pejorative behavior. The diagnosis is based on histological analysis coupled with immunohistochemical study. Because it is rare, there is no consensus to date. Its management should be multidisciplinary, involving surgery, chemotherapy, and radiotherapy. We report two new cases of neuroendocrine small cell carcinoma of the bladder, one is pure and the other associated with urothelial carcinoma. The poor outcome illustrates the quick evolution of this tumor. A review of the literature on this topic is also presented.
Marwan Sleiman, Abbassi Ziad, Amanda Seipel, Minoa Jung, Christian Toso, Thibaud Koessler, Stefan Monig
Journal of Cancer Science and Clinical Therapeutics, Volume 4, pp 222-228; doi:10.26502/jcsct.5079066

Abstract:
Introduction: Small cell carcinoma is most frequently found in the lung. Only 4% of small cell carcinomas are present in the urinary bladder, prostate, oesophagus, stomach, colon, rectum, gallbladder, larynx, salivary glands, cervix and skin. Primary gastric small cell carcinoma (GSCC) is a very rare and poorly differentiated neuroendocrine tumour. We present a case of primary gastric small cell carcinoma and a systematic review of literature. Case Presentation: A 58 year-old man presented with epigastric pain, nausea and melena. Several exams showed an antro-pyloric poorly differentiated grade III gastric small cell carcinoma classified as cT4 cN+ cM0. The tumour board recommended neoadjuvant treatment with six cycles of cisplatin-etoposide, with the last two cycles associated with radiotherapy. Re-staging showed a good partial response and no spread of the disease, therefore we completed treatment with a radical gastrectomy with D2 lymph node dissection. The surgery was performed in August 2019 and the patient was discharged after ten days. Pathology report showed a mix large and small cells neuroendocrine carcinoma . Due to the neoadjuvant treatment, the small cell component seen in a preoperative biopsy had largely disappeared and the case was diagnosed as a mixed large and small cell neuroendocrine carcinoma ypT4a N3a (9/28) G3 L1 V1 Pn1 R0. Discussion: Clinical manifestations of gastric small cell carcinoma (GSCC) are similar to classic gastric adenocarcinomas. However, gastric small cell carcinoma can secrete ectopic hormones like parathyroid hormone, antidiuretic hormone, calcitonin or serotonin. Upper gastrointestinal endoscopy is the method of choice for the diagnosis, supplemented with an immunohistochemical examination with a positive reaction to neuro-specific enolase (NSE), chromographin A, synaptophysin, CD56 and Grimelius. The treatment strategy for GSCC is unclear. There is no difference in the pathological features, response to chemotherapy, incidence of progression and survival between the different anatomical locations of these tumours. A review of the literature limited to GSCC recommended multi-modal approach combining surgery, radiotherapy and platinum-based adjuvant chemotherapy. Ten months after surgery, the patient is still disease free. Conclusion: GSCC is a rare and aggressive entity with low survival rate. The rarity of this entity makes treatment challenging. Unlike small cell lung cancer, surgery remains part of the multi-modality treatment of GSCC.
Shilpy Jha, Suvradeep Mitra, Sciprofile linkAmit K. Adhya, Prasant Nayak
Published: 18 January 2019
by BMJ
Case Reports, Volume 12; doi:10.1136/bcr-2018-228017

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Sciprofile linkDharma Ram, Sciprofile linkS.K. Rajappa, Venkata Pradeep Babu Koyyala, Smaranjeet Chatterjee, Yogendra Singh Bhakuni, Himanshu Shukla, Amitabh Singh, Sudhir Rawal
Indian Journal of Medical and Paediatric Oncology, Volume 39; doi:10.4103/ijmpo.ijmpo_30_17

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Sciprofile linkG. Velilla, C.J. Carrión, J.A. Portillo, D. Truán, A. Azueta, J. Fuentes, E. Herrero, L. Gala
Published: 1 April 2016
Actas Urológicas Españolas, Volume 40, pp 195-200; doi:10.1016/j.acuro.2015.11.008

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Yu Yu Thar, Poras Patel, Tiangui Huang, Elizabeth Guevara
Case Reports in Oncological Medicine, Volume 2016, pp 1-5; doi:10.1155/2016/1496916

Abstract:
Small cell neuroendocrine carcinoma (SNEC) is a rare form of malignancy. It mainly presents as bronchogenic neoplasm, and the extrapulmonary form accounts for only 0.1% to 0.4% of all cancers. These extrapulmonary tumors have been described most frequently in the urinary bladder, prostate, esophagus, stomach, colon and rectum, gall bladder, head and neck, cervix, and skin. Primary SNEC of the sinonasal tract is extremely rare with only less than 100 cases reported in the literature. Because of extreme rarity and aggressiveness of the tumor, the management for this entity varies considerably mandating multimodality approach. In this paper, we report a patient presented with left-sided facial swelling, and the histopathologic examination confirmed primary SNEC of left sinonasal tract. The tumor involved multiple paranasal sinuses with invasion into the left orbit and left infratemporal fossa and metastasized to cervical lymph nodes and bone. The patient encountered devastating outcome in spite of optimal medical management and treatment with palliative chemotherapy highlighting the necessity for further research of primary SNEC of head and neck.
Sciprofile linkMustapha Ahsaini, Omar Riyach, Mohammed Fadl Tazi, Mohammed Jamal El Fassi, My Hassan Farih, Hind Elfatmi, Afaf Amarti
Published: 18 August 2013
Case Reports in Urology, Volume 2013, pp 1-4; doi:10.1155/2013/598325

Abstract:
Introduction. Small cell neuroendocrine carcinomas of the urinary tract is an extremely rare entity and very few cases have been reported in the literature. Small cell neuroendocrine carcinoma of the urinary tract (SCC-UT) is the association between bladder and urinary upper tract-small cell carcinoma (UUT-SCC). It characterized by an aggressive clinical course. The prognosis is poor due to local or distant metastases, and usually the muscle of the bladder is invaded. Case Presentation. We report a rare case of a 54-year-old Arab male native of moroccan; he is a smoker and was referred to our institution for intermittent hematuria. Following a diagnosis of small cell neuroendocrine carcinomas of the ureter and the bladder, thoracoabdominal-pelvic CT was done, and the staging of the tumor was done in the bladder (T2N0M0) and (T1N0M0) in the ureter. Neoadjuvant alternating doublet chemotherapy with ifosfamide/doxorubicin and etoposide/cisplatin was realized, and nephroureterectomy associated to a cystoprostatectomy was carried out. After 24 months of followup, no local or distant metastasis was detected. Conclusion. The purpose of this review is to present a rare case of pure small cell neuroendocrine carcinoma of the urinary tract and review the literature about the place of neoadjuvant chemotherapy in this rare tumors.
Nora I. Schneider, Cord Langner, Richard Zigeuner
The American Journal of the Medical Sciences, Volume 345, pp 155-157; doi:10.1097/maj.0b013e3182648759

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