Anorectal small cell carcinoma

PURPOSE: Poorly differentiated neuroendocrine carcinoma (PDNEC) of the rectum and anus is a rare disease exhibiting aggressive biological behaviour, even if diagnosed early. Currently, there are no agreed standard treatment approaches and management of locally advanced (LA) and metastatic PDNEC usually follows treatments used in pulmonary neuroendocrine carcinomas because of the similarities with small cell lung cancer. The role of surgery in PDNEC is still debated and the benefit of chemoradiotherapy (CRT) is unknown...

Poorly differentiated neuroendocrine carcinomas (NECs) are rare malignant neoplasms with aggressive behavior. The diagnosis remains challenging due to ever-changing terminologies and morphologic overlaps with other disease entities. Herein, we seek to better define anorectal NECs by high-risk human papillomavirus (HPV) status and molecular profiling. Fourteen cases, including 3 men and 11 women with a median age of 63 years, were included. High-risk HPV RNA in situ hybridization was diffusely positive (+) in 7 cases, focal rarely positive (+/-) in 2 cases, and completely negative (-) in 5 cases...

BACKGROUND: Basal cell carcinoma of the perianal region is a rare anorectal disease. This condition is not related to exposure to ultra-violet radiation. Due to the low prevalence and poor detection, there is a paucity of data relating to this condition in literature. Perianal basal cell carcinoma present different surgical challenges from other anatomic locations and may not share the same prevalence or natural history. Here, we describe the largest series to date on the surgical management of perianal basal cell carcinoma...

INTRODUCTION: Bladder urothelial carcinoma rarely spreads to the gastrointestinal tract, and its presentation in the rectum varies. We report a case of a patient who presented with an annular constriction of the rectum. PRESENTATION OF CASE: A 60-year-old man was referred to our hospital with chief complaints of anal stricture and partial obstruction for about 1 month. Computed tomography and magnetic resonance imaging revealed diffuse wall thickening of the rectum, possible high cellularity in the lower portion of urinary bladder, and lesions in the visible pelvic bony structure...

BACKGROUND: Anorectal cancer arising in IBD can be challenging to manage. There is a paucity of reports describing locally advanced and recurrent anorectal cancer in this setting. OBJECTIVE: This study aimed to describe patients who underwent extended radical pelvic resection for locally advanced and recurrent IBD-associated anorectal cancer. DESIGN: This is a retrospective review of a prospectively maintained database of extended radical pelvic resection...

The incidence of anal cancer has increased during the second half of the 20th century, with an incidence rate over 2.9% greater than in the decade of 1992-2001. Yet, it still constitutes a small percentage, about 4%, of all anorectal tumours. Its risk factors are human papillomavirus infection, a history of sexually transmitted diseases, a history of vulvar or cervical carcinoma, immunosuppression related to human immunodeficiency virus infection or after organ transplantation, haematological or immunological disorders, and smoking...

BACKGROUND: Paraneoplastic limbic encephalitis (PLE) is a rare autoimmune neurological syndrome observed in cancer patients. PLE is difficult to diagnose and presents a variable response to treatment, depending on the characteristics of the tumor and neuronal autoantibodies. CASE PRESENTATION: A 64-year-old, Caucasian, non-smoker man presented with a rapidly developing cognitive impairment, personality change, spatial disorientation, and short-term memory loss associated with anorexia and cervical and inguinal lymph nodes...

AIM: To determine the incidence, prognosis, and immunophenotypes (CK7, CK20, CDX2, and GCDFP-15) of primary or secondary perianal Paget's diseases (PPDs). METHODS: Twenty-three PPD patients were recruited, including 10 primary and 13 secondary PPDs. Immunophenotypes of PPD were analyzed. RESULTS: In 23 PPD patients, 14 (60.9%) were male and the median age was 75 years. Three (13.0%, 2 primary and 1 secondary PPDs) had recurrence and two (8...

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BACKGROUND: Colorectal carcinoma in the pediatric age group is rare and tends to be very aggressive and present late, due to which it has a very poor prognosis. It may present with distant metastasis; however, metastasis to the testes is very rare and signifies an advanced stage of the disease. Surgery is the only effective modality to cure patients with localized colorectal carcinomas. However, statistics show a higher incidence of unresectable disease and a higher metastasis rate in childhood colorectal carcinomas...

BACKGROUND: Cancer arising from perianal fistulas in patients with Crohn's disease is rare. There are only a small series of articles that describe sporadic cases of perianal cancer in Crohn's disease fistulas. Therefore, there are no clear guidelines on how to appropriately screen patients at risk and choose proper management. OBJECTIVE: The purpose of this study was to describe patients diagnosed with cancer in perianal fistulas in the setting of Crohn's disease...

Tumors and tumorlike conditions of the anus and perianal region originate from the anal canal and anal margin or result from direct extension of tumors from adjacent organs. The anatomy of the anal canal is complex, and its different histologic characteristics can lead to diverse pathologic conditions. The anal canal extends from the anorectal junction to the anal verge. The World Health Organization classification of anal canal tumors includes (a) anal intraepithelial neoplasia, the precursor of squamous cell carcinoma (SCC), and (b) invasive tumors...

BACKGROUND: Colorectal high-grade neuroendocrine carcinomas (HGNEC) are a rare but aggressive group of malignancies without standard management recommendations. METHODS: We retrospectively reviewed the records of 100 consecutive patients with histologically confirmed colorectal HGNEC diagnosed at MD Anderson Cancer Center between 1991 and 2013. RESULTS: In our cohort, most tumors (89%) were small cell carcinoma, and most (60%) involved the sigmoid or the anorectal regions...

Human papilloma virus (HPV) infection causes cancers and their precursors (high-grade squamous intraepithelial lesions) near cervical and anal squamocolumnar junctions. Recently described cervical squamocolumnar junction cells are putative residual embryonic cells near the cervical transformation zone. These cells appear multipotential and share an identical immunophenotype (strongly CK7-positive) with over 90% of high-grade squamous intraepithelial lesions and cervical carcinomas. However, because the number of new cervical cancers discovered yearly world wide is 17-fold that of anal cancer, we posed the hypothesis that this difference in cancer risk reflects differences in the transition zones at the two sites...

The standard treatment of locally advanced (stage II and III) squamous cell carcinoma of the anal canal consists of concurrent chemoradiotherapy (two cycles of 5-fluoro-uracil, mitomycin C, on a 28-day cycle), with a dose of 45 Gy in 1.8 Gy per fraction in the prophylactic planning target volume and additional 14 to 20 Gy in the boost planning target volume (5 days per week) with a possibility of 15 days gap period between the two sequences. While conformal irradiation may only yield suboptimal tumor coverage using complex photon/electron field junctions (especially on nodal areas), intensity modulated radiation therapy techniques (segmented static, dynamic, volumetric modulated arc therapy and helical tomotherapy) allow better tumour coverage while sparing organs at risk from intermediate/high doses (small intestine, perineum/genitalia, bladder, pelvic bone, etc...

Vismodegib (Erivedge) is a first-in-class small-molecule hedgehog pathway inhibitor for the treatment of adults with advanced basal-cell carcinoma. Because this pathway is known to play key roles in patterning and growth during vertebrate development, vismodegib was anticipated to be embryotoxic. To support marketing applications, an embryofetal development study was completed in which a limited number of pregnant rats (n = 6/group) was administered vismodegib by oral gavage on gestation days 6 to 17. When vismodegib was administered at ≥60 mg/kg/day, doses associated with evidence of pharmacologic activity in previous rat toxicity studies, all conceptuses were resorbed at an early embryonic stage in the absence of significant maternal toxicity...

Neurofibromatosis type-1 (NF1), also known as Von Recklinghausen disease, is an autosomal dominant disorder with incidence of one per 4000. Neurofibromas are benign, heterogeneous, peripheral nerve sheath tumors coming up from the connective tissue of peripheral nerve sheaths, particularly the endoneurium. Visceral involvement in disseminated neurofibromatosis is considered rare. Neurofibroma occurs most frequently in the stomach and jejunum, but colon and anorectal canal may also be involved. Gastrointestinal neurofibromas may lead to bleeding, obstruction, intussusception, protein-losing enteropathy and bowel perforation...

We describe the treatment of a 46-year-old Saudi man with advanced stage liver metastatic neuroendocrine rectal cancer. The patient presented with a large liver lesion and rectal bleeding. He was cachectic, with a firm tender mass 20 mm above the anal verge. Computed tomography (CT) showed a mass 9.5 × 13 cm in size in the right hemi-liver, abutting the middle hepatic vein. The patient refused treatment, and consulted another hospital. After 3 months, he presented with the same symptoms in addition to delirium...

OBJECTIVE: The aim of this study was to examine the brain activity manifested while non-small cell lung cancer (NSCLC) patients with and without anorexia were exposed to visual food stimuli. METHODS: We included 26 treatment-naïve patients who had been recently diagnosed with advanced NSCLC. Patients with brain metastasis were excluded. The patients were classified into anorectic and non-anorectic groups. Data from functional magnetic resonance imaging based on blood oxygen level-dependent (BOLD) signals were analyzed while the patients perceived pleasant and unpleasant food pictures...

INTRODUCTION: Gluteal fold flaps (GFFs) have been extensively reported for vulvo-vaginal reconstruction but there are no published series of their use for perineal reconstruction following anorectal cancer excision. In this context, abdominal myocutaneous flaps remain the method of choice but may be unavailable because of pre-existing abdominal scars, or need for a colostomy/urostomy. In addition, their abdominal wall morbidity makes them less acceptable, especially given the increasing use of laparoscopic techniques for the extirpative surgery...