A patient with co-existing bronchial carcinoid tumour and bilateral phaeochromocytoma.

We report a 31-year-old Chinese man with bronchial carcinoid tumour and bilateral phaeochromocytoma. His sister also gave a history of bilateral carotid body paraganglioma. This case demonstrates the importance of screening for other endocrine disorders in patients with foregut carcinoid tumours.     

Multiple endocrine neoplasia type 2b: long-term follow-up of a case

Multiple endocrine neoplasia type 2b is a rare inherited syndrome which comprises the association of medullary thyroid carcinoma, phaeochromocytoma, widespread neuromatous proliferation and a characteristic body habitus. In this report we present the late clinical course and autopsy findings of the first patient with this syndrome described in Australia. At presentation she was found to have a right adrenal phaeochromocytoma and medullary thyroid carcinoma which were resected in separate operations. No clinical or biochemical evidence of residual medullary thyroid carcinoma was identified in life. However, in spite of serial vanillylmandelic acid estimations, which showed normal or only mildly elevated levels, and normal results of urinary catecholamine studies, a left adrenal phaeochromocytoma was identified in a metaiodobenzylguanidine (MIBG) study performed 14 years after presentation. Her late clinical course was dominated by progressive dysphagia, intestinal dysmotility and megacolon associated with unrelenting malnutrition. After her death due to an intracerebral haemorrhage, an autopsy confirmed the presence of a left adrenal phaeochromocytoma and revealed diffuse intestinal ganglioneuromatosis to be the cause of her intestinal dysmotility. No residual medullary thyroid carcinoma was found. This case emphasises the propensity for multiple endocrine tumours in these patients and highlights the potentially significant role of intestinal ganglioneuromatosis in the natural history of this condition.     

Adenocarcinoma of the gastro-oesophageal junction with a synchronous carcinoid of the duodenum

Duodenal carcinoids are rare tumours. There is an increased incidence of primary carcinomas, especially in the gastrointestinal tract, which occur synchronously with gastrointestinal tract carcinoids. However, the synchronous occurrence of adenocarcinoma of the gastro-oesophageal junction with a duodenal carcinoid has not been previously described. A case report is presented, with discussion of carcinoid tumours and management when occurring synchronously with non-carcinoid tumours.     

Carcinoid tumour of gall bladder--a case report

Gall bladder carcinoid tumours are rare, constituting less than 1% of all carcinoid tumours arising from different parts of the body. They usually lack specific symptoms as they typically are unassociated with the carcinoid syndrome, despite frequent hepatic spread and mostly detected after cholecystectomy. A case of gall bladder carcinoid is reported in a 35-year-old woman who underwent laparoscopic cholecystectomy for clinical features of cholelithiasis. Several comparative evaluation was attempted mainly between the carcinoid and variant groups (endocrinomas) and occasionally between the typical and atypical carcinoid series to solve the existing serious problem in categorisation of this group of tumours. While the usual criteria for judging malignancy, such as anaplasia and mitotic figures, are unreliable in these sites of carcinoid tumour, malignancy is well determined from evidence of tumour invasion into adjacent structures. This rarity and the ambiguity around it stresses for its documentation and warrants for international agreements and standards on basic criteria for such classification of these endocrine carcinomas.     

Primary tumours of the duodenum.

Six primary duodenal tumours were diagnosed in our 300 bed institute during a period of 10 years. Two patients had benign tumours (leiomyoma and carcinoid) and four had malignant tumours (adenocarcinoma). The most common manifestation was severe iron deficiency anemia. Diagnosis was usually delayed (with a mean time of 7.7 months from initial complaints), endoscopy being the most common and useful diagnostic tool. A curative procedure was performed in two patients. Patients with unexplained chronic iron deficiency anaemia should undergo thorough gastrointestinal evaluation, including the small intestine, as a curable disease may be found to be the source of the complaint.     

The impact of recent advances in diagnostic technology on the clinical presentation of phaeochromocytoma.

OBJECTIVE: To examine the impact of recent advances in diagnostic technology on the spectrum of clinical and biochemical features of patients presenting with a new diagnosis of phaeochromocytoma. DESIGN: A retrospective review of the clinical and biochemical features of patients diagnosed by our laboratory as having phaeochromocytoma within a 27-month period up to December, 1990. Noradrenaline, adrenaline and dihydroxyphenylglycol were assayed in 24-hour urine specimens (19 patients) or plasma (1 anuric patient) by gas chromatography/mass spectrometry. SETTING: A tertiary level chemical pathology department. PATIENTS: Twenty patients with a new diagnosis of phaeochromocytoma. RESULTS: The classic, episodic adrenergic symptoms traditionally associated with phaeochromocytoma were absent in 9 of the 20 patients (45%). "Atypical" phaeochromocytoma presented as a mass on computed tomography imaging (6 patients, 30%), "phaeochromocytoma crisis" (4 patients, 20%) or family screening (1 patient, 5%). Excessive adrenaline production was found in 11 patients (55%) and six (30%) had predominantly adrenaline-secreting tumours. The urinary noradrenaline:dihydroxyphenylglycol ratio was raised in all nine patients with predominantly noradrenaline-secreting tumours but was not raised in nine out of ten patients with adrenaline-secreting phaeochromocytoma. Adrenaline excretion was significantly correlated with tumour size (r = 0.8; P less than 0.05). CONCLUSIONS: Advances in diagnostic technology, particularly specific adrenaline assays and computed tomography, have made possible the early diagnosis of patients with phaeochromocytoma presenting in ways previously thought to be uncommon. All patients with adrenal masses noted incidentally on CT scan should be investigated for phaeochromocytoma. Adrenaline-secreting tumours are common and both noradrenaline and adrenaline should be assayed in all patients investigated for phaeochromocytoma.     

Surgical treatment of endocrine hypertension experience in India

Between 1980 and 1994, 162 cases of endocrine based hypertension were diagnosed and treated surgically. Seventy-nine cases (48.7%) of phaeochromocytoma, 63 cases (38.8%) of Cushing's syndrome, and 20 cases (12.3%) of Conn's syndrome were diagnosed. In phaeochromocytoma 75% of the tumours arose from the adrenal glands and 25% arose from the extra-adrenal sites. Cushing's syndrome was caused by adenoma (45%), diffuse bilateral adrenal hyperplasia (36%), pigmented macronodular hyperplasia (9%), and adrenal carcinoma (10%). The most common cause of Conn's syndrome was adenoma (95%) which arose mainly from the left adrenal gland (60%). In the present series the success rate of surgical treatment was 100% for phaeochromocytoma, 90% for Cushing's syndrome and 96% for Conn's syndrome. Trucut biopsy of the kidneys of these patients showed hypertensive changes, the moderate hypertension could be due to renal damage.     

Role of 131I-metaiodobenzylguanidine in the localization of suspected phaeochromocytomas

Iodine-131-metaiodobenzylguanidine is a recently-developed radiopharmaceutical agent for adrenal medullary scintigraphy. Twenty-one scans with 131I-metaiodobenzylguanidine were performed in 20 adults with suspected phaeochromocytomas over a four-year period. All patients previously had undergone computed tomographic scans of the abdomen and pelvis. The computed-tomographic scans were abnormal in 14 patients (16 tumours), eight (nine tumours) of whose 131I-metaiodobenzylguanidine scans gave positive results. Both types of scan gave negative results in the remaining six patients. Among 12 patients who underwent surgery or postmortem examination, the 131I-metaiodobenzylguanidine scan correctly showed eight phaeochromocytomas; six tumours that were found on computed-tomographic scans but not on 131I-metaiodobenzylguanidine scans proved not to be phaeochromocytoma. The 131I-metaiodobenzylguanidine scan probably gave a true-positive result in an additional case (surgical confirmation was not available). None of the remaining seven patients in whom the 131I-metaiodobenzylguanidine scan gave negative results has been shown to harbour a phaeochromocytoma on extended follow-up (2.5 to four years). Iodine-131-metaiodobenzylguanidine is a highly sensitive and specific agent for the localization of phaeochromocytomas. In patients with suspected phaeochromocytomas and abnormal computed-tomographic findings, 131I-metaiodobenzylguanidine permits a non-invasive, functional evaluation of the morphological abnormalities to be made. The importance of making a biochemical diagnosis of a phaeochromocytoma before attempting localization studies is emphasized.     

Carcinoid tumours.

The use of long-acting and potent somatostatin analogues is a major advance in the management of carcinoid tumours. In addition to providing effective symptom relief in malignant carcinoid syndrome, octreotide can also be used for diagnostic purposes. Despite its expense, octreotide is the current agent of choice for the treatment of this condition while analogues with different receptor specificities and pharmacokinetics hold promise for the future. Gastric carcinoids have aroused interest because of their experimental association with chronic hypergastrinaemia, a condition now commonplace because of the widespread use of H2-blockers and proton-pump inhibitors. This subject is reviewed. The slow evolution of many tumours demands prolonged follow-up and the active use of a variety of palliative interventions. These include measures such as hepatic and cardiac surgery, which might be deemed inappropriate for patients with other types of metastatic malignancy. Interferons may have a role when first-line treatments have failed. Chemotherapy is, generally, of limited value.     

Cutaneous scleroderma in association with carcinoid syndrome.

A case of scleroderma in a woman with carcinoid syndrome is described and the similarities between our case and those in the literature are reviewed. The carcinoid tumours were all of midgut origin and liver metastases were present. All subsequently developed fibrotic heart disease and none had clinical features or autoantibodies suggestive of systemic sclerosis. The association between carcinoid syndrome and particular features of scleroderma is likely to be more than fortuitous.     

Recurrence of Phaeochromocytoma and Abdominal Paraganglioma After Initial Surgical Intervention

BackgroundClinical and biochemical follow up after surgery for phaeochromocytoma is essential with long term studies demonstrating recurrence frequencies between 6% and 23%.AimTo examine the characteristics and frequency of tumour recurrence in a regional endocrine referral centre, in patients with surgical resection of phaeochromocytoma (P) and abdominal paraganglioma (AP).MethodsWe identified a cohort of 52 consecutive patients who attended our Regional Endocrinology & Diabetes Centre and retrospectively reviewed their clinical, biochemical and radiological data (between 2002 and 2013). After confirmation of early post-operative remission by negative biochemical testing, tumour recurrence was defined by demonstration of catecholamine excess with confirmatory imaging.ResultsPhaeochromocytoma was confirmed histologically in all cases (43:P, 9:AP, mean-age:53years). Open adrenalectomy was performed in 20 cases and laparoscopically in 32. Hereditary phaeochromocytoma was confirmed by genetic analysis in 12 (23%) patients. Median follow up time from initial surgery was 47 months, (range: 12 - 296 months), 49 patients had no evidence of tumour recurrence at latest follow-up. Three patients (6%) demonstrated tumour development, one in a patient with VHL which occurred in a contralateral adrenal gland, one sporadic case had local recurrence, and an adrenal tumour occurred in a patient with a SDHB gene mutation who had a previous bladder tumour. After initial surgery, the tumours occurred at 8.6, 12.0 and 17.7 years respectively.ConclusionIn this study tumour development occurred in 6% of patients. Although tumour rates were low, careful and sustained clinical and biochemical follow up is advocated, as new tumour development or recurrence may occur long after the initial surgery is performed.     

Synchronous carcinoid tumour of the small intestine and appendix in the same patient

Carcinoid tumours have been reported in a wide range of organs but most frequently involve the gastrointestinal tract. Many of these carcinoid tumours are associated with metachronous and synchronous lesions of another histological type. Primary carcinoid tumours of the different organ in the same patient is rare. In this paper, the authors present a case with synchronous carcinoid tumour of the small intestine and appendix in the same patient.     

Role of venous sampling in locating a phaeochromocytoma

Selective venous sampling was performed in 31 patients in whom the diagnosis of phaeochromocytoma was suspected on clinical and biochemical grounds. Data from samples assayed for their adrenaline and noradrenaline content using a radioenzymatic technique were used to confirm or refute the suspected diagnosis. In 19 of the 31 patients a phaeochromocytoma was subsequently removed surgically, and the remaining 12 patients are now thought not to have tumours (mean follow up period: four years). Analysis of the assay data shows that selective venous sampling correctly identified the presence of a tumour in all 19 patients, and correctly excluded the diagnosis in 11 of the 12 remaining patients--an overall success rate of 97%. Success rates of 88% for arteriography and 84% for computed tomography were recorded, though these investigations were not performed in all patients. Ultrasound and intravenous urography were much less accurate. On the basis of this study a sequence of investigation is proposed for patients with a suspected phaeochromocytoma. Computed tomography occupies a central role in this sequence with venous sampling (and occasionally other techniques) being used only as complementary investigations when specific indications for their use exist.     

Superadded aspergillosis on carcinoid bronchial adenoma leading to delayed diagnosis

A review of aspergillosis in neoplastic disease revealed that most cases occur in association with malignant disorders. Amongst solid tumours it is mostly adenocarcinomas which are invaded by the fungus due to necrotic cavity formation. We here record the first reported association of aspergillosis with a benign carcinoid bronchial adenoma which was hidden by the fungal growth with a delay in diagnosis.     

Malignant phaeochromocytoma metastasising to the breast

Background Nearly one-quarter of metastatic tumours in the breast are from an occult extramammary tumour, usually a lung carcinoma. Aim To report on a patient with a history of metastatic malignant phaeochromocytoma and a breast mass. Result A 54-year-old female presented with a right breast mass. At the age of 32, she had presented with a phaeochromocytoma. The staining of the breast mass was comparable with that of her original adrenal tumour. Conclusion This is the first published case of a phaeochromocytoma metastasising to the breast, and demonstrates the challenge that extramammary tumours in the breast can pose for the pathologist.     

Carcinoid tumour arising in a Meckel's diverticulum

A patient with a carcinoid tumour arising in a Meckel's diverticulum and presenting with acute small intestinal obstruction is described. Review of previously reported cases indicates that the initial clinical presentation of carcinoid tumours occurring in a Meckel's diverticulum is usually similar to that of appendiceal carcinoids. While most carcinoids in Meckel's diverticula present as incidental findings at post-mortem examination or laparotomy, their metastatic potential and subsequent behaviour resembles that of other small intestinal carcinoid tumours.     

前白蛋白诊断支气管肺类癌的研究

本研究检测了前白蛋白作为诊断支气管肺类癌的可靠性以及前白蛋白在其它类型肺癌的分布。结果表明：类癌前白蛋白阳性率为５０％，其中典型类癌１００％，不典型类癌２５％。小细胞癌尚有１１．８％的阳性率。１４例鳞癌，６例腺癌均为阴性。因而前白蛋白是诊断肺支气管类癌有用的标记，其价格便宜，稳定可靠，适合常规开展。     

Goodpasture's syndrome in a patient with two endocrine tumours

We describe the occurrence of two anatomically separate and histologically distinct endocrine tumours of bronchus and pancreas in a 64 year old woman with biopsy and serology proven Goodpasture's syndrome. The association between anti-glomerular basement membrane antibody disease and multiple endocrine tumours has not been reported previously; possible pathogenetic mechanisms are discussed.     

Carcinoid syndrome due to a malignant somatostatinoma.

Somatostatinoma is one of the rarest tumours of the endocrine pancreas. Cardinal manifestations of a somatostatinoma include gallstones, mild diabetes mellitus, steatorrhoea, diarrhoea and dyspepsia. Like any other pancreatic islet cell carcinoma, a somatostatinoma may also produce several different hormones such as adrenocorticotropic hormone, calcitonin, vasoactive intestinal polypeptide, pancreatic polypeptide, gastrin, insulin, and glucagon. In many cases, the clinical picture is dominated by the effect of these other hormones. We present a patient with somatostatinoma in which an immunocytochemical study of the specimens from pancreas and liver showed a weak positive reaction for gastrin besides a strong positive reaction for somatostatin. Interestingly, this patient also showed the signs of carcinoid syndrome which was successfully treated with octreotide.     

钛夹防治胃肠道出血的疗效观察

目的探讨钛夹在预防和治疗胃肠道出血中的疗效和安全性。方法在胃镜、肠镜下应用钛夹预防和治疗胃肠道疾病出血27例,其中十二指肠球部溃疡12例,腺瘤6例,息肉4例,胃溃疡3例,胃错构瘤1例,十二指肠降部类癌1例。喷血或涌血3例,渗血15例,血管裸露4例,有潜在出血(有不牢固的新鲜血栓形成、病灶电凝切后有鲜红色创面裸露)5例。结果使用钛夹后出血停止或有效地预防了出血26例,有效率96·30%。27例共打钛夹86枚,成功80枚,打钛夹成功率93·02%。未发现任何并发症。结论钛夹预防和治疗胃肠道出血安全、有效。