Primary hepatic carcinoid tumor in children

Primary carcinoid tumors of the liver are rare, with fewer than 60 cases currently reported in the English literature. We present the evaluation and management of a solid hepatic tumor in a 14-year-old boy. Intraoperative biopsy was indeterminant for malignant potential, and the patient underwent complete resection by left hepatic lobectomy. Final histopathologic evaluation of the mass revealed a carcinoid tumor.Extensive endoscopic and radiologic workup revealed no other primary source. The patient recovered well from surgery and is currently free of disease 32 months after initial resection. Review of the literature suggests that primary hepatic carcinoid tumors are particularly rare in children. As the liver is frequently a site for carcinoid metastasis from the gastrointestinal tract, any patient with a suspected primary hepatic carcinoid tumor must undergo an extensive search for an extrahepatic primary site. These tumors are typically indolent but may metastasize. In addition, medical therapy is of limited benefit in reducing tumor bulk. The mainstay for treatment of primary hepatic carcinoid tumors is surgical resection, and these tumors carry a more favorable prognosis than other primary hepatic malignancies and metastatic carcinoid. Follow-up is long-term, as these tumors can recur many years after initial resection.     

A half century of experience with carcinoid tumors in children

: To investigate the frequency, presentation, clinical management, and prognosis of appendiceal carcinoid tumors in children. : A review of our institution's experience over 50 years. : 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. : 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.     

Carcinoid Tumor of the Spleen: Report of a Case

Carcinoid tumors are uncommon tumors of the neuroendocrine system. They grow slowly and may remain silent for years before presenting with carcinoid syndrome. A diagnosis of asymptomatic carcinoid tumor is difficult. Wide resection of the primary tumor and metastatic lesions is the first choice of treatment. Primary carcinoid is sometimes distributed through-out the entire body, but it is rare in the spleen. We herein present a rare case of a symptomless carcinoid tumor that predominantly invaded the spleen with liver metastasis. Received: February 8, 2001 / Accepted: July 17, 2001     

Primary renal carcinoid tumor with liver metastases detected with somatostatin receptor imaging

If the final pathologic examination reveals a carcinoid tumor of the kidney, additional evaluation to rule out another occult primary tumor site is necessary. If the primary origin of the tumor is confirmed to be from the kidney, additional evaluation is required for the detection of metastasis. Renal carcinoid tumors are extremely rare; however, both primary and metastatic renal carcinoid tumors have been reported. We report a case of a 40-year-old woman with primary carcinoid of the kidney and metastatic disease in the liver not evident by computed tomography and magnetic resonance imaging but identified by somatostatin receptor scintigraphy.     

Carcinoid tumors in the breast

BACKGROUND: Carcinoid tumors in the breast are rare. Most represent metastases from other primary sites, but commonly are mistaken for primary breast lesions. METHODS: A literature search of the English language found 59 cases of carcinoid tumors in the breast, 21 (36%) of which were metastases. RESULTS: We present an additional 3 cases of carcinoid tumors metastatic to the breast and discuss the clinical, radiologic, and pathologic manifestations. CONCLUSIONS: It is important to differentiate between primary breast carcinoid tumor and metastatic disease to the breast because of differences in treatment. All palpable breast masses and mammographically detected lesions should undergo a biopsy examination. In those patients with a known history of carcinoid tumor, pertinent clinical history, and previous surgical specimens should be reviewed to avoid an unnecessary mastectomy. If there is no history of a prior carcinoid tumor, a thorough work-up to look for an occult primary tumor elsewhere should be performed.     

Solitary versus multiple carcinoid tumors of the ileum: a clinical and pathologic review of 68 cases

It is well known that small intestinal carcinoid tumors may occur as solitary or multiple lesions. However, the biologic significance of multiple carcinoid tumors has not been clearly defined. The purpose of this study was to compare the clinical and pathologic features and prognosis of patients with solitary versus multiple carcinoid tumors of the ileum. Sixty-eight patients, including 50 with solitary and 18 with multiple carcinoid tumors, were included in the study. Hematoxylin and eosin-stained slides from routinely processed tumor resection specimens of the ileum were evaluated for a variety of histologic features such as tumor size, depth of invasion, tumor stage, and venous, perineural, and lymphovascular invasion. Follow-up and clinical data, such as patient age, gender, presenting complaints, presence of synchronous or metachronous malignancies, and presence of the carcinoid syndrome, were obtained and the results were compared between the two patient groups. Fifty patients with solitary carcinoid tumors (male/female ratio, 27:23) and 18 patients with multiple tumors (male/female ratio, 7:11) were identified. Patients with multiple carcinoid tumors were significantly younger than patients with solitary tumors at the time of diagnosis (55 years vs 63 years, p = 0.006). There was a high association between multiple carcinoid tumors and the carcinoid syndrome (4 of 18 vs 1 of 50, p = 0.004) as compared with patients with solitary carcinoid tumors. There was also an association between tumor multiplicity and venous invasion, but this relationship was not statistically significant (p = 0.07). The follow-up period was similar for both groups (mean 36 months, median 26 months, range 1-139 months). A significantly higher proportion of patients with multiple carcinoid tumors were either alive with disease or died of disease (56%) compared with those with solitary carcinoid tumors (18%, p = 0.002), and this relationship persisted in multivariate analysis (p = 0.02). Overall, no significant differences were observed between these two patient groups with respect to other clinicopathologic features such as tumor size, depth of invasion, presence of distant metastases, lymphatic or perineural invasion, or presence of an associated malignancy (p >0.05). In conclusion, we found that patients with multiple carcinoid tumors are younger, have a significantly greater risk of developing the carcinoid syndrome, and have a poorer prognosis than patients with solitary tumors.     

Current management of gastrointestinal carcinoid tumors

Gastrointestinal carcinoid tumors are rare neuroendocrine tumors arising from the embryologic primitive gut. Depending on the location in the gastrointestinal tract, these tumors may secrete a variety of hormonally active substances. However, many of these tumors are found incidentally, or the diagnosis is made postoperatively. Also, there is a significant incidence of multicentric carcinoid tumors and synchronous noncarcinoid malignancies in these patients. Treatment is usually based on the size of the tumor. Surgical resection remains the cornerstone of therapy. For advanced metastatic disease, somatostatin analog therapy and surgical debulking provide the best symptomatic relief and may improve survival. Recent studies have demonstrated a benefit from radiolabeled somatostatin analogs for carcinoid tumor localization. In contrast, radiolabeled somatostatin analogs have shown little therapeutic benefit. Future directions include somatostatin receptor profiling of carcinoid tumors, with somatostatin analog therapy targeting the specific receptors.     

Metastatic renal carcinoid: case report and review of the literature

Primary renal carcinoid tumor is a rare tumor of the kidney. Metastatic renal carcinoid tumor has not been described in the medical literature. We report a case of renal metastasis of a primary bronchial carcinoid tumor and review the literature about renal carcinoid tumors.     

Case report: primary ovarian carcinoid: a rare tumor causing unexpected manifestations in a previously undiagnosed woman

Although carcinoid tumors arising from midgut structures are rare, the existence of these tumors is well documented. However, carcinoid tumors arising from the reproductive organs, such as primary ovarian carcinoids, are rare and sparsely documented. Because of this rarity, a small percentage of women may present with ovarian masses that are not properly diagnosed as carcinoid tumor creating unexpected hemodynamic changes under general anesthesia. However, prior knowledge of the clinical manifestations of this rare entity may improve the sensitivity of the preoperative evaluation and allow the anesthesiologist to take precautions as a result of the suspicion of ovarian carcinoid.     

Treatment of rectal carcinoid tumors

We investigated the treatment of 24 rectal carcinoid tumors from both the clinicopathologic and prognostic viewpoints. All tumors less than 2 cm in diameter had neither muscle layer invasion nor lymph node metastasis, except for an atypical carcinoid tumor that had both lymphatic permeation and intramural metastasis. One typical carcinoid tumor larger than 2 cm had both several lymphatic permeations and urinary bladder invasion. All cases had a good prognosis with no recurrence and no new metastases. For rectal carcinoid tumors less than 2 cm in diameter, local resection is sufficient, whereas radical operation is required for tumors larger than 2 cm in diameter. For atypical rectal carcinoid tumors, radical operation should be considered even if the diameter is less than 2 cm.     

Childhood carcinoid tumors: the St Jude Children's Research Hospital experience

BACKGROUND/PURPOSE: To better characterize childhood carcinoid tumors, the authors reviewed the clinical presentation, treatment, and outcomes of pediatric patients with these rare tumors. METHODS: A retrospective review was conducted of medical records and pathologic materials of all children with carcinoid tumors treated at St Jude Children's Research Hospital between December 1977 and March 1999. RESULTS: Eight patients (median age, 12.7 years) were identified; 2 were boys, and 7 were white. Primary tumor sites were the appendix (n = 5), small intestine (n = 1), bronchus (n = 1), and 1 unknown site. In 7 cases, carcinoid tumor was not suspected at the time the tumor was identified. Seven patients had localized disease; 5 remain disease-free after complete resection, and 2, whose carcinoid tumors were identified incidentally, died of metastatic mucinous adenocarcinoma of the colon. One patient who presented with symptoms of carcinoid syndrome had metastatic disease that responded poorly to cytotoxic chemotherapy and remains alive with active disease. CONCLUSIONS: Although most pediatric carcinoid tumors arise in the appendix, these tumors also occur in other primary sites. Clinical awareness and early diagnosis are important factors in preventing morbidity and mortality. Outcomes are excellent for patients with localized disease that is completely resected, but those with metastatic disease fare poorly. New therapeutic strategies are needed for these patients.     

Laparoscopic resection of posterior duodenal bulb carcinoid tumor

Duodenal carcinoid tumors are rare neuroendocrine foregut tumors that, in contrast to midgut carcinoid tumors, have a low metastatic potential. The majority can be resected endoscopically. This case details a 67-year old man presenting with a biopsy-proven carcinoid tumor located at the posterior superior aspect of the duodenal bulb which was not amenable to endoscopic resection. The patient underwent a laparoscopic resection of the tumor after precise localization using simultaneous laparoscopy and duodenoscopy, with reconstruction of the duodenotomy defect using intracorporeal suturing techniques. The authors present a case report and details of an operative technique that may be utilized on other operations of the duodenum.     

Comparative analysis of alternative and traditional immunohistochemical markers for the distinction of ovarian sertoli cell tumor from endometrioid tumors and carcinoid tumor: A study of 160 cases

The main neoplasms in the differential diagnosis for primary ovarian tumors with a tubule-rich pattern are pure Sertoli cell tumor, endometrioid tumors (including borderline tumor, well-differentiated carcinoma, and the sertoliform variant of endometrioid carcinoma), and carcinoid tumor. Because traditional immunohistochemical markers [pan-cytokeratin (pan-CK), low molecular weight cytokeratin (CK8/18), epithelial membrane antigen (EMA), inhibin, calretinin, CD99, chromogranin, and synaptophysin] can occasionally have diagnostic limitations, the goal of this study was to determine whether or not any alternative markers [cytokeratin 7 (CK7), estrogen receptor (ER), progesterone receptor (PR), CD10, and CD56] have better diagnostic utility when compared with traditional markers for this differential diagnosis. Immunohistochemical stains for alternative, as well as traditional, markers were performed on the following primary ovarian tumors: pure Sertoli cell tumor (n = 40), endometrioid borderline tumor (n = 38), sertoliform endometrioid carcinoma (n = 13), well-differentiated endometrioid carcinoma (n = 27), and carcinoid tumor (n = 42). Extent and intensity of immunostaining were semiquantitatively scored. In addition, immunohistochemical composite scores (ICSs) in positive cases were calculated on the basis of the combination of extent and intensity scores. Cytokeratin 7 (CK7) was positive in 97% of endometrioid tumors, 13% of Sertoli cell tumors, and 24% of carcinoid tumors. The differences in the mean ICSs for endometrioid tumors versus Sertoli cell tumor or carcinoid tumor were statistically significant (P values ranging from <0.001 to 0.018). ER and PR were positive in 87% and 86% of endometrioid tumors, 8% and 13% of Sertoli cell tumors, and 2% each of carcinoid tumors, respectively. The differences in the mean ICSs for endometrioid tumors versus Sertoli cell tumor were statistically significant (P values ranging from <0.001 to 0.012). Among the epithelial markers, EMA seemed to be the most discriminatory but only slightly better than CK7, ER, or PR. Pan-CK and CK8/18 were not helpful. CD10 showed overlapping patterns of expression in all categories of tumors. Among the sex cord markers, CD10 was markedly less useful than inhibin or calretinin; CD99 was not discriminatory. CD56 showed overlapping patterns of expression in all categories of tumors. Among the neuroendocrine markers, CD56 was less useful than chromogranin or synaptophysin. When traditional immunohistochemical markers are problematic for the differential diagnosis of ovarian Sertoli cell tumor versus endometrioid tumors versus carcinoid tumor, adding CK7, ER, and/or PR to a panel of markers can be helpful. Endometrioid tumors more frequently express CK7, ER, and PR and show a greater extent of immunostaining in contrast to Sertoli cell tumor and carcinoid tumor. Compared with traditional epithelial markers, CK7, ER, and PR are nearly as advantageous as EMA. Inhibin is the most discriminatory sex cord marker, and CD10 is not helpful in the differential diagnosis. Chromogranin and synaptophysin are excellent discriminatory markers for carcinoid tumor, and CD56 is neither sufficiently sensitive nor specific enough for this differential diagnosis to warrant its use in routine practice.     

术前化疗对胰腺癌细胞凋亡和组织结构的影响

探讨术前选择性动脉化疗对胰腺癌细胞生长的影响，为临床制定合理的胰腺癌化疗方案提供理论依据。方法运用ＤＮＡ末端标记法对３０例胰腺癌患者术前化疗和未化疗和术后病理切片进行肿瘤细胞凋亡的检测，同时对比观察它们ＨＥ染色的组织结构变化 。     

Testicular carcinoid tumor: A case report and literature review

We present a case of a 46-year-old man with an incidental finding of a right testicular tumor on magnetic resonance imaging. He received radical orchiectomy, and the pathological report showed a testicular carcinoid tumor. He has remained in a stable condition 3 years after the operation. Carcinoid tumors of the testis are extremely rare and account for less than 1% of testicular tumors and carcinoids. A painless mass or prominent testis enlargement is the most distinctive presentation. Less than 1–3% of patients demonstrate carcinoid syndrome. Nuclear scintigraphy scan and computed tomography scan have a diagnostic value in excluding carcinoid from other sites. Radical orchiectomy is the treatment of choice, and the prognosis is good for localized carcinoid tumors after resection. Adjuvant chemotherapy or radiotherapy for advanced disease shows only minimal benefits.     

Malignant carcinoid tumor in the papilla of Vater: case report and review of 25 cases reported in Japan

Carcinoid tumors in the papilla of Vater are rare. We describe a 48-year-old male who was diagnosed with a tumor consisting of atypical cells in the papilla of Vater. He underwent curative resection of the tumor by pancreatico-duodenectomy (PD), with dissection of the regional lymph nodes. Microscopic examination revealed a metastatic lymph node in the pancreatic region. Characteristic findings on histopathological and electron microscopic studies led to the final diagnosis of malignant carcinoid tumor. It is clear from the literature that carcinoid tumors in the papilla of Vater metastasize to the regional lymph nodes and the liver. However, it has been difficult to preoperatively diagnose carcinoid tumor in the papilla of Vater. Therefore, if we observe atypical cells in the papilla of Vater, malignant carcinoid tumor should be considered. If this tumor is suspected, PD or pylorus-preserving PD, together with dissection of the regional lymph nodes, at least along the hepatoduodenal ligament, should be considered as the treatment of first choice.     

Carcinoid syndrome from gastrointestinal carcinoids without liver metastasis.

Although patients with bronchial and ovarian carcinoid tumors can develop the carcinoid syndrome (diarrhea and/or flushing) in the absence of hepatic metastasis, it is believed that development of the carcinoid syndrome in patients with carcinoid tumors of gastrointestinal origin occurs only after the patient has hepatic metastasis. This is explained by hepatic inactivation of most of the serotonin in the portal circulation or by the fact that hepatic metastases are larger than the primary tumor in the gastrointestinal tract. Three patients with ileal and jejunal carcinoid tumors who developed the carcinoid syndrome without obvious hepatic metastasis are described. Two of the patients had intra-abdominal, but extrahepatic, metastasis that probably drained directly into the systemic circulation. The third patient had an ileal carcinoid with clinical involvement limited to adjacent mesenteric lymph nodes. Following resection of her tumor, her urinary 5-HIAA excretion and platelet serotonin level returned to normal, and her attacks of carcinoid flushing virtually ceased. She has occasional spells of "blushing" that are thought to be benign; however, further close follow-up study will be needed to be certain that she is free of disease. It is suggested that each patient with the carcinoid syndrome be evaluated with CT and technetium-99 pertechnetate liver scans. If there is no liver involvement detected with these studies, one should consider hepatic arteriogram or laparotomy to determine if the patient's tumor might be totally resectable.     

Testicular metastasis 10 years after resection of appendiceal carcinoid

Testicular carcinoids are very rare and account for less than 1% of all testicular neoplasms. They may present as primary testicular tumors or secondary tumors from extratesticular sources. We report a case of a secondary testicular carcinoid occurring 10 years after surgical treatment of an appendiceal carcinoid. The patient underwent radical orchiectomy and at 24 months of follow-up had no evidence of metastases elsewhere. Because secondary carcinoids have a worse clinical course and prognosis than primary tumors, metastatic disease should be excluded before the tumor is identified as a primary. Long-term follow-up is necessary for patients with carcinoid tumor owing to its indolent course and the risk of metastasis several years after treatment of the primary.     

Coexisting thymic carcinoid tumor and thymoma

Thymic carcinoid tumors are unusual neoplasms that are different from thymomas. We report a case of coexisting thymic carcinoid tumor and thymoma associated with myasthenia gravis. The clinicopathological findings are discussed with a review of the literature.     

Primary carcinoid of the testis associated with carcinoid syndrome

Testicular carcinoid is a rare disease accounting for less than 1% of all testicular neoplasms. It rarely manifests symptoms of carcinoid syndrome. Recent reports have noted that only 1.1-3.1% of testicular carcinoid tumors are complicated by carcinoid syndrome. In general, large tumor size and the presence of carcinoid syndrome are features associated with a malignant course. In the present case, pathological findings revealed pure carcinoid of the testis without metastasis. Moreover, watery diarrhea due to carcinoid syndrome disappeared and the serum serotonin level normalized following orchiectomy. The patient was followed up for 12 months with whole body computed tomography scan and assessment of serotonin levels. To date, there is no evidence of tumor recurrence. These findings suggest that monitoring serum serotonin levels may be useful as a marker during follow up of this type of tumor.