Fistula Formation Secondary to Mucinous Appendiceal Adenocarcinoma May Be Related to a Favorable Prognosis: A Case Report and Literature Review.

A 90-year-old man was referred to our hospital because of a positive fecal occult blood test. Colonoscopy revealed a lesion with multiple nodules covered with abundant mucus at the hepatic flexure. Computed tomography showed a dilated appendix attached distally to the hepatic flexure. Right hemicolectomy was performed, and the pathological examination revealed a mucinous appendiceal adenocarcinoma infiltrating the hepatic flexure without pseudomyxoma peritonei. The patient is doing well without recurrence 12 months postoperatively. Extraperitoneal drainage of the malignant ascites caused by the fistula may allow for an early diagnosis, while also making it possible to successfully resect the lesion, thus resulting in a favorable outcome.

Molecular Diagnostics in the Neoplasms of Small Intestine and Appendix: 2018 Update.

Neoplasms of the small intestine are rare in comparison with colorectal tumors. The most common tumor types arising in the small intestine are adenocarcinomas, well-differentiated neuroendocrine tumors, gastrointestinal stromal tumors, and lymphoma. Primary appendiceal neoplasms are rare and found in less than 2% of appendectomy specimens with an incidence of approximately 1.2 cases per 100,000 people per year in the United States. This article explores molecular diagnostics in the neoplasms of small intestine and appendix.

Overinterpretation is common in pathological diagnosis of appendix cancer during patient referral for oncologic care.

CONTEXT: Low-grade appendiceal mucinous neoplasm (LAMN) and appendiceal adenocarcinoma are known to cause the majority of pseudomyxoma peritonei (PMP, i.e. mucinous ascites); however, recognition and proper classification of these neoplasms can be difficult despite established diagnostic criteria. OBJECTIVE: To determine the pathological diagnostic concordance for appendix neoplasia and related lesions during patient referral to an academic medical center specialized in treating patients with PMP. DESIGN: The anatomic pathology laboratory information system was searched to identify cases over a two-year period containing appendix specimens with mucinous neoplasia evaluated by an outside pathology group and by in-house slide review at a single large academic medical center during patient referral. RESULTS: 161 cases containing appendix specimens were identified over this period. Forty-six of 161 cases (28.6%) contained appendiceal primary neoplasia or lesions. Of these, the originating pathologist diagnosed 23 cases (50%) as adenocarcinoma and 23 cases (50%) as LAMN; however, the reference pathologist diagnosed 29 cases (63.0%) as LAMN, 13 cases (28.3%) as adenocarcinoma, and 4 cases (8.7%) as ruptured simple mucocele. Importantly, for cases in which the originating pathologist rendered a diagnosis of adenocarcinoma, the reference pathologist rendered a diagnosis of adenocarcinoma (56.5%, 13 of 23), LAMN (39.1%, 9 of 23), or simple mucocele (4.3%, 1 of 23). The overall diagnostic concordance rate for these major classifications was 71.7% (33 of 46) with an unweighted observed kappa value of 0.48 (95% CI, 0.27-0.69), consistent with moderate interobserver agreement. All of the observed discordance (28.3%) for major classifications could be attributed to over-interpretation. In addition, the majority of LAMN cases (65.5%) had potential diagnostic deficiencies including over-interpretation as adenocarcinoma and lacking or discordant risk stratification (i.e. documentation of extra-appendiceal neoplastic epithelium). CONCLUSIONS: Appendiceal mucinous lesions remain a difficult area for appropriate pathological classification with substantial discordance due to over-interpretation in this study. The findings highlight the critical need for recognition and application of diagnostic criteria regarding these tumors. Recently published consensus guidelines and a checklist provided herein may help facilitate improvement of diagnostic concordance and thereby reduce over-interpretation and potential overtreatment. Further studies are needed to determine the extent of this phenomenon and its potential clinical impact.

Primary adenocarcinoma of the appendix - a case report.

Abstract Primary adenocarcinoma of the vermiform appendix is a rare clinical condition. It usually presents as an acute abdominal process or as a mass in the right lower quadrant. The gross pathologic and microscopic characteristics of this disease are the same as for colon carcinoma and carry a serious prognosis. We report a case of a 64-year old male patient presenting symptoms of acute appendicitis. The patient underwent laparoscopic appendectomy that revealed a severely inflamed appendix. Histopathological examination has shown that it was a poorly differentiated, high grade adenocarcinoma invading the periappendicular fat. According to the histopathological findings, a right hemicolectomy was further advised and performed. Right hemicolectomy is the generally recommended method of treatment for invasive adenocarcinoma of the appendix.

Intussusception of the appendix induced by sessile serrated adenoma: a case report.

Intussusception of the appendix vermiformis is a rare condition. It occurs mainly in infants and children. Here, we report an intussusception case that occurred in a 65-year-old male presenting with repeated periumbilical pain, nausea, vomiting and febrile sensation. The appendix was seen to be intussuscepted at laparoscopy. The invaginated segment was reducted and simple appendicectomy was carried out. Histopathologic examination revealed a sessile serrated adenoma at the wall of the appendix, suggesting it as the cause of the intussusception.

Ectopic ACTH-syndrome due to a neuroendocrine tumour of the appendix.

Ectopic ACTH production causes 10% of Cushing's syndromes. The diagnostic workup is difficult, can last more than 6 months (> 50% of cases), and the underlying tumour is still frequently not located (12%). Carcinoid tumours of the appendix are frequent and are revealed in 0.3% of patients undergoing routine appendectomy. However, neuroendocrine tumours of the appendix with ACTH production are an extremely rare entity. Here we report the case of a female patient with clinically overt Cushing's syndrome due to ectopic ACTH-production from a carcinoid tumour of the appendix. During the diagnostic workup, repeated endocrine tests, multiple different imaging modalities and frequent and lengthy hospitalisations were necessary. Wrongly, even a neurosurgical pituitary exploration was performed. After 12 months from the initial admission, the tumour was finally detected by an ¹8F-fluoro-L-dihydroxyphenylalanine (¹8FDOPA PET) and an appendectomy followed by right hemicolectomy were performed. The patient recovered rapidly and the symptoms from the hypercortisolism were no more present.In this case, we discuss the multitude of problems, which may delay the diagnosis and the pitfalls, that should be avoided in order to locate the tumour and to initiate adequate therapy as early as possible. Furthermore, our case demonstrates the complexity of diagnostic procedures, which demand most of the times a multidisciplinary approach. In this setting, regular follow-ups in short time intervals and the use of novel imaging techniques can finally cut the diagnostic "Gordian knot".

PET/CT appearance of appendiceal adenocarcinoma.

Appendiceal cancer is rare and is associated with a poor prognosis because it is usually found at an advanced stage. We report the case of a 58-year-old man with appendiceal adenocarcinoma, who complained of right lower abdominal malaise for a couple of months. The tumor was detected early on contrast-enhanced computed tomography and F-18 FDG PET/CT. It appeared as an obvious enhancement and intense abnormal tracer uptake focus in front of the right psoas major, which was easily misinterpreted as an enlarged lymph node.

Adenocarcinoma of the appendix presenting as bilateral ureteric obstruction.

BACKGROUND: Adenocarcinoma of the vermiform appendix is a rare neoplasm of the gastrointestinal tract. Presentation mimics acute appendicitis, but right iliac fossa mass and intestinal obstruction have also been reported. These presentations reflect various stages of a locally expanding tumour causing luminal obstruction of appendix. The investigation and subsequent management with a review of the literature is presented. CASE PRESENTATION: We report a case of appendicular adenocarcinoma found unexpectedly in a 43 year old male who presented with urinary symptoms. Cystoscopy and uretero-renoscopy showed normal bladder but external compression of the ureters and therefore bilateral stents were inserted. CT scan showed a caecal mass. After colonoscopy, that showed external compression, and diagnostic laparoscopy the patient underwent right hemicolectomy. Histopathology revealed well differentiated adenocarcinoma with signet ring morphology with multiple lymph node involvement. The patient was referred for chemotherapy where he received infusional 5 fluorouracil but died 7 months after surgery. CONCLUSION: Patients with atypical manifestations related to right lower abdominal quadrant should be thoroughly investigated with an open mind. Every attempt should be made to make a precise diagnosis through all the available means to direct the treatment along correct lines.

Primary T-cell non-Hodgkin's malignant lymphoma of the appendix.

A case of primary T-cell lymphoma of the appendix in an 84-year-old female was reported. Appendectomy was performed as a result of the clinical diagnosis of acute appendicitis, due to the rebound tenderness of McBurney's point and thickness of the appendix wall as determined from ultra echo sonograph. Grossly, the surgical resected appendix did not have a dominant inflammatory appearance, therefore a tumor was suspected. Microscopic examination showed diffused proliferation of large and medium size lymphoma cells. Immunohistochemical examination further revealed that the lymphoma cells were positive for T-cell markers. To ensure this was a T-cell lymphoma, molecular examination was performed using paraffin-embedded tissue sections, since T-cell lymphoma of the appendix is extremely rare. Polymerase chain reaction (PCR) single-strand conformation polymorphism (SSCP) analysis demonstrated monoclonal T-cell receptor gene rearrangement. T-cell-rich B-cell lymphoma was excluded. To our knowledge, this is the first reported case of primary T-cell lymphoma of the appendix. PCR-SSCP analysis in paraffin-embedded tissue section was very useful in the diagnosis of lymphoma cell monoclonality.

Tumors of the appendix and colon.

This collective review includes all available case reports of smooth muscle (stromal) tumors of the appendix and large intestine in the world literature. When compiling this review, we endeavored to examine cumulative as well as recently collected data on both benign and malignant smooth muscle tumors spanning the period 1875 to 1996. In total, there were reports of 331 leiomyomas (LMs) and 263 leiomyosarcomas (LMSs). The peak age of incidence of LM was 30 to 39 years, and the peak age of incidence of LMSs was 50 to 59 years. The female/male ratio was slightly higher for LM, and the male/female ratio was higher for LMS. The descending colon and sigmoid colon were the most common sites of both benign and malignant smooth muscle tumors. The growth of LMs most often occurred extraluminally, whereas LMSs tended to grow within the lumen of the colon. With both tumor types pain was the most frequent presenting complaint, followed less commonly by complaints of a palpable mass or gastrointestinal bleeding. LMSs tended to be larger at diagnosis than LMs, though the duration of symptoms for both types of tumor was most often reported to be between 1 month and 1 year. Finally, LMSs were found to metastasize mo

Tumores carcinóides do apêndice cecal / Appendiceal carcinoid tumors

O comportamento biológico distinto dos demais carcinóides do intestino e as dúvidas existentes no tratamento cirúrgico dos carcinóides apendiculares motivaram a realizaçäo deste estudo retrospectivo. De 20 pacientes operados no HSPE-FMO entre 1970 e 1990, 14 (setenta por cento) eram mulheres e 6 (trinta por cento) homens, com média de idade de 26,8 anos. Quatorze (setenta por cento) foram operados com diagnóstico de apendicite aguda, confirmada pelo exame histopatológico em todos, após apendicectomia clássica. Três (quinze por cento) tiveram seus carcinóides descobertos acidentalmente por ocasiäo de outras cirurgias e outros 3 (quinze por cento) apresentaram dor, febre, massa palpável e quadro séptico, sendo submetidos à colectomia direita. Esses 3 doentes tinham tumores > 2cm, todos apresentando invasäo neoplásica do meso apendicular e um tinha linfonodo comprometido. O tumor era < 1cm em 14 (setenta por cento) doentes e media entre 1 e 2 cm em outros três (quinze por cento). O local preferencial foi a extremidade distal (sessenta por cento) e em setenta por cento dos doentes os carcinóides estavam confinados à túnica muscular. Houve 1 (cinco por cento) óbito hospitalar motivado pela doença e um segundo, dois anos após por infarto miocárdio. Os demais doentes (noventa por cento) estavam vivos cinco a dezoito anos após, sem sinais de recidiva. Os autores acreditam que tumores < 1 cm säo adequadamente tratados pela simples retirada do apêndice, assim como a grande maioria daqueles com 1 a 1,5 cm. Doentes com tumores > 2cm e jovens com tumores entre 1,5 e 2,0 cm, com achados histopatológicos de agressividade, devem ser submetidos à colectomia direita

Tumor carcinóide de apêndice / Carcinoid tumor of appendix

O tumor carcinóide de apêndice é uma patologia rara e na maioria das vezes assintomática. Apresenta quadro clínico indistinguível de apendicite aguda em mais de 50 por cento dos casos, sendo achado ocasional em laparatomias por abdome agudo. Tem excelente prognóstico quando a lesão é pequena e desfecho mais reservado quando o tumor é maior que dois centímetros. Seu tratamento ainda é controverso, embora na maioria dos casos observe-se boa sobrevida com a simples ressecção do apêndice. O presente estudo visa explanar a ocorrência incomum, dificuldades diagnósticas, bem como a necessidade de medidas terapêuticas definidas, através da revisão da literatura segundo suas diferentes publicações mundiais

Tumor carcinoide del apéndice cecal: informe de 12 casos / Carcinoid tumor of the cecal appendix: report of 12 cases

El tumor carcinoide del apéndice cecal es la neoplasia maligna más frecuente de este órgano y, si bien es de baja frecuencia, puede ocasionar metástasis e incluso la muerte. Se realizó un revisión de los estudios realizados a las piezas quirúrgicas enviadas al Departamento de Anatomía Patológica del Hospital General Regional No. 25 para conocer su frecuencia, el sexo y la edad de presentación el tamaño tumoral, los signos de presentación y el tratamiento. Se revisaron 88.817 informes, 20.5 por ciento fueron apéndices y 0.06 por ciento se diagnosticaron como carcinoides, la edad promedio fue de 19.25 años y la relación entre uno y otro sexo fue de 1:1. La indicación de cirugía en la mayoría fue apendicitis aguda. La Localización del tumor principalmente fue en la punta y sólo en un caso, debido al tamaño tumoral, se realizó hemicolectomía derecha

Carcinoid tumor of the appendix in the first two decades of life.

Carcinoid tumor of the appendix is the most common neoplasm of the gastrointestinal tract in childhood and adolescence. Sufficient long-term follow-up data after surgical treatment are not currently available for patients diagnosed during the first two decades of life. From 1936 to 1988, 23 patients were observed at this institution with histologically confirmed carcinoid tumors involving the vermiform appendix. In contrast to the adult experience, in which the tumor is most commonly encountered as the result of an incidental appendectomy, 18 of these patients presented with signs and symptoms of an acute abdomen directing the surgeon to the appendix. In the other five cases, surgery was performed for other reasons. Median age at presentation was 13.0 years (range, 6 to 20 years). Fourteen patients were female, nine were male. Simple appendectomy was the initial procedure for all patients. Tumor size ranged from "microscopic" to 2.5 cm in largest dimension. Three patients subsequently underwent right hemicolectomy, and one patient had removal of a residual appendiceal stump, but no residual or metastatic tumor tissue was found in any of the resected specimens. Nineteen patients underwent simple appendectomy alone. Eighteen available specimens were reviewed at the time of this study for confirmation of histology and degree of invasion. The tumor invaded to the serosa in nine of 23 (39%). The mesoappendix or periappendiceal fat was involved in seven of 23 (30%). Vessel invasion was not noted in any specimen. Our median follow-up time was very long, being 26 years (range, 9 months to 51 years). No patient has had evidence of recurrent or metastatic disease.(ABSTRACT TRUNCATED AT 250 WORDS)