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Carcinoid tumors.

Carcinoid tumors can present a difficult diagnostic and therapeutic dilemma. Despite their reputation as indolent tumors, they frequently metastasize and can cause significant symptomatology. The only curative therapy remains surgical resection. The prognosis and treatment of carcinoids vary based on location and histology, and therapy must be tailored to each patient.

Antineoplastic Agents, Hormonal↗

Appendiceal carcinoma masquerading as recurrent urinary tract infections: case report and review of literature.

We report on a female patient who presented with recurrent urinary tract infections. The investigations revealed an adenocarcinoma of the appendix fistulating into the bladder and causing irritative symptoms. After right hemicolectomy and partial cystectomy, she was well at 3 months without any need for adjuvant therapy. This unusual situation reminds us that the finding of bladder adenocarcinoma is rare, and it would be prudent to examine the lower gastrointestinal tract thoroughly to look for a bowel source, before recommending any ablative surgery.

Aged↗

Incidence of metastasis to the ovaries from nongenital tract primary tumors.

OBJECTIVES: The purpose of this study was to evaluate the characteristics of metastatic tumors to the ovaries in nongenital tract primaries and to determine the route of dissemination. METHODS: An IRB-approved study retrospectively reviewed patient records from January 1992 to January 2003. A tumor registry and pathology database search identified women with metastatic disease to the ovaries that had undergone surgery for the presence of an adnexal mass. The charts were reviewed for age at diagnosis, presenting symptoms, size of ovarian metastasis, laterality of metastasis, and primary tumor site. Pathology reports and specimen slides were reviewed to confirm the diagnosis and evaluate the tumors for various pathological features. RESULTS: A total of 59 cases of metastasis to the ovary were identified. The median age of the study group was 55 years old (range: 27-78). Primary colon cancer was identified in 19 (32.2%) cases; appendix 12 (20.3%); breast 5 (8.4%); small bowel and gastric each contributed 4 (6.8%) cases. Pancreatic cancer added 3 (5.1%), while gallbladder and urinary bladder each contributed 1 (1.7%) case. Tumors of unknown primary contributed 10 (18.5%) of the cases. Stromal invasion was seen in 56 (95%) of the cases and surface involvement in 9 (15%) cases. Bilateral metastasis was found in 39 (66.1%) patients and unilateral metastasis in 20 (33.9%) patients. CONCLUSIONS: Metastatic lesions to the ovary are more commonly seen from primary colon cancer, appendiceal, and breast carcinomas. The mechanism of metastasis is through hematogenous pathways as opposed to a transserosal route.

Adult↗

Clinical challenges presented by three simultaneous solid tumors.

BACKGROUND: Simultaneous tumors are rare, and their management can be challenging. The simultaneous presentation of cervical carcinoma, renal cell carcinoma, and appendiceal carcinoma has not been previously described. CASE: A 57-year-old woman presented with cervical cancer. During her workup, she was diagnosed with mucinous appendiceal carcinoma and clear cell carcinoma of the kidney. One year following surgery, she remains without evidence of disease and with continually improving nutritional status. CONCLUSION: When simultaneous tumors are diagnosed, optimal care requires the creative expertise of a multidisciplinary team. Standard sequential therapies may be problematic in patients undergoing major surgery to treat another primary tumor, and sequential treatment delays rather than combining therapies can jeopardize cure. Treatment planning should utilize a coordinated multidisciplinary approach.

Adenocarcinoma, Clear Cell↗

Primary appendiceal cancer: gynecologic manifestations and treatment options.

OBJECTIVE: To determine the presenting symptoms, gynecologic manifestations, and optimal intraoperative management of women with primary appendiceal cancer. METHODS: A multi-institutional investigation was performed to identify female patients with primary appendiceal cancer who were treated from 1990 to present. RESULTS: Forty-eight women with primary appendiceal cancer were identified from the tumor registries of participating institutions. The most common symptoms were abdominal pain (40%) and bloating (23%), but only 8% experienced rectal bleeding. Serum CEA was elevated (>2.5 U/ml) in 67% of patients, and serum Ca-125 was elevated (>35 U/ml) in 50% of patients. Thirty-one patients (65%) presented with a right adnexal or right lower quadrant mass and were operated on initially by a gynecologic oncologist. Ovarian involvement by metastatic appendiceal cancer was documented in 18 patients (38%). All of these patients underwent total abdominal hysterectomy, bilateral salpingo-oophorectomy, and staging, but only 8 had a right hemicolectomy at the time of initial surgery. Forty-one patients (85%) presented with advanced stage appendiceal cancer (Stage III or IV) and 19 patients (46%) received postoperative chemotherapy, most commonly with a combination of 5-FU/Leukovorin. Following surgery, 22 patients (46%) experienced disease progression or recurrence, and 14 have died of disease. The most common sites of recurrence were abdominal or pelvic peritoneum (18), colon (2), and ovary (2). Patient survival was 70% at 2 years, and 60% at 5 years. CONCLUSION: Women with primary appendiceal cancer frequently present with ovarian metastases, and initial surgical intervention is often performed by a gynecologic oncologist. All patients with mucinous epithelial ovarian cancer should undergo appendectomy at the time of surgical staging. The appendix should be examined intraoperatively, and if appendiceal carcinoma is identified, a right hemicolectomy and appropriate surgical staging should be considered.

Adult↗

Molecular genetic evidence supporting the clonality and appendiceal origin of Pseudomyxoma peritonei in women.

Pseudomyxoma peritonei (PMP) is a poorly understood condition characterized by mucinous ascites and multifocal peritoneal mucinous tumors. Women with PMP often have mucinous tumors involving both the appendix and the ovaries. Several previous histopathological and immunohistochemical studies of PMP have suggested that most, if not all, cases of PMP in women are derived from mucinous adenomas of the appendix rather than from primary ovarian tumors. A few studies of the molecular genetics of PMP have been recently reported. However, these studies analyzed only a small number of cases and some included a heterogeneous group of mucinous tumors, including both benign and malignant appendiceal and ovarian tumors. We analyzed K-ras mutations and allelic losses of chromosomes 18q, 17p, 5q, and 6q in a substantial number of morphologically uniform cases of PMP with synchronous ovarian and appendiceal tumors as well as in appendiceal mucinous adenomas (MAs) and ovarian mucinous tumors of low malignant potential (MLMPs) unassociated with PMP. Each of the 16 PMP cases (100%) analyzed demonstrated identical K-ras mutations in the appendiceal adenoma and corresponding synchronous ovarian tumor. K-ras mutations were identified in 11 of 16 (69%) appendiceal MAs unassociated with PMP and in 12 of 16 (75%) ovarian MLMPs unassociated with PMP. Two PMP cases showed identical allelic losses in the matched ovarian and appendiceal tumors. A discordant pattern of allelic loss between the ovarian and appendiceal tumors at one or two of the loci tested was observed in six PMP cases. In all but one instance, LOH was observed in the ovarian tumor, whereas both alleles were retained in the matched appendiceal lesion, suggesting tumor progression in a secondary (metastatic) site. Our findings strongly support the conclusion that mucinous tumors involving the appendix and ovaries in women with PMP are clonal and derived from a single site, most likely the appendix.

Adenoma↗

Goblet cell carcinoid of the appendix.

BACKGROUND: Over the past 15 years, a variant of appendiceal carcinoid has been recognized which is not widely known to the general surgeon. This tumor, variously labeled adenocarcinoid or goblet cell carcinoid, has a more aggressive natural history than classic appendiceal carcinoids and requires a different surgical approach. PATIENTS AND METHODS: A survey of the tumor registries of 10 hospitals identified 7 female and 2 male patients with goblet cell carcinoid of the appendix between 1983 and 1993. The clinical data of these patients were reviewed. RESULTS: Median age was 58 years (range 31 to 73). Clinical presentation followed two distinct patterns: acute onset suggestive of appendicitis or chronic symptoms associated with a pelvic mass. In no case was the diagnosis suspected preoperatively or established intraoperatively. Four patients with discrete distal appendiceal tumors treated with appendectomy are alive without evidence of disease 2 to 11 years since their diagnoses. The 5 patients with diffuse appendiceal involvement all had intra-abdominal metastases. Four are dead of disease at intervals from 5 months to 2 years postdiagnosis. CONCLUSIONS: Goblet cell carcinoids are a distinct clinical and pathologic entity with variable malignant potential. Patients with diffuse appendiceal involvement require aggressive surgery as part of multimodal therapy.

Adult↗

Management of carcinoma discovered unexpectedly at operation for acute appendicitis.

This review of 900 patients who underwent colon resection for carcinoma identified 33 patients who were explored with the preoperative diagnosis of appendicitis. In 19 patients, the appendix was acutely inflamed secondary to obstruction of the appendiceal lumen. The other 14 patients had perforating or obstructing cancer of the ascending colon or cecum. The clinical clues of a prolonged symptom history, weight loss, anemia, and a palpable mass were frequently, but not invariably, present. Results of contrast enema, computerized axial tomography, and colonoscopy in this select group were frequently misleading. The diagnosis is best made in the operating room by the thoughtful appendectomist. I recommend prompt resection and an appropriate cancer procedure in these patients. Carcinoma masquerading as appendicitis occurs more often than is generally realized and will be seen more frequently as our aging population increases.

Adult↗

A half century of experience with carcinoid tumors in children.

PURPOSE: To investigate the frequency, presentation, clinical management, and prognosis of appendiceal carcinoid tumors in children. METHOD: A review of our institution's experience over 50 years. RESULTS: Twenty-two patients below the age of 20 presented with appendiceal carcinoid tumor. The mean age at presentation was 14.6 years. Twelve patients presented with symptoms of appendicitis. No tumor was > 2.0 cm in size. Only 2 patients underwent resection beyond appendectomy. No patient had recurrent or metastatic carcinoid tumor, and all but 1 patient (who died of ovarian choriocarcinoma) are alive without evidence of carcinoid tumors 1.5 to 30 years after diagnosis. CONCLUSIONS: Appendiceal carcinoid tumors in children are rarely life-threatening and the incidence of large tumors (> 2.0 cm) is very low. The role of right hemicolectomy in large (> 2.0 cm) tumors is questionable in this age group.

Adolescent↗

[Crohn's disease of the appendix].

A giant appendix is an extremely rare and improbable finding during surgery in suspected cases of acute appendicitis. Although this condition is primarily suggestive of neoplasia, it is usually due to an inflammatory or infectious disease. We report a case of Crohn's disease limited to the appendix, which was diagnosed after a short right ileocolectomy. Only 156 similar diagnoses have been reported in the literature to date. This disease appears to have a benign course and therefore differs from classical Crohn's ileocolitis. For this reason, these patients do not require any specific investigation or follow-up.

Adult↗

Pulmonary metastases in pseudomyxoma peritonei syndrome.

BACKGROUND: Pseudomyxoma peritonei (PMP) is a rare disease arising from a mucinous cystadenoma of appendiceal origin. The syndrome has been characterized by progressive growth of mucinous tumors, tense mucinous ascites, and ultimately death. Abdominal and pelvic recurrence after resection of intraperitoneal disease occurs in all patients unless adjunctive measures are taken. Local spread of PMP by direct extension to the pleural or pericardial space is uncommon but has been reported in the literature. Here we report development of pulmonary parenchymal metastases after treatment for PMP. METHODS: The charts of 3 patients were retrospectively reviewed for the presentation and management of metastatic PMP. RESULTS: Three patients underwent resection for pulmonary parenchymal metastases of PMP. All patients recovered uneventfully. The continue to do well after 2 to 8 years of follow-up. CONCLUSIONS: Pulmonary metastasectomy for PMP is safe and effective after treatment of intraperitoneal disease.

Adult↗