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Carcinoid Syndrome Due to a Malignant Somatostatinoma

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Carcinoid Syndrome Due to a Malignant Somatostatinoma Giant retroperitoneal hemangiopericytoma 695 10 Kanffman SL, Stout AP. Hemangiopericytoma in children. 21 Jimenez-Avala M, Diez-Nau MD, Larrad A, et al. Heman- Cancer 1960; 13: 695-710. giopericytoma in a male breast. Report of a case with 11 Tulenko JF. Congenital hemangiopericytoma: case report. cytologic, histologic and immunochemical studies. Acta 234-8. Plast Reconstr Surg 1968; 41: 276-7. Cytol 1991; 35: Postgrad Med J: first published as 10.1136/pgmj.71.841.695 on 1 November 1995. Downloaded from 12 Kent KH. Hemangiopericytoma. AJR 1957; 77: 347-56. 22 Smullens SN, Scatti DJ, Osterholm JL. Preoperative 13 Mujahed Z, Vasilas A, Evans JA. Hemangiopericytoma: a embolization of retroperitoneal hemangiopericytomas as an report offour cases with a review ofthe literature. AIR 1959; aid in their removal. Cancer 1982; 50: 1870-5. 82: 658-66. 23 Smith RB, Machleder HI, Rand RW, Bentson J, Tarbas P. 14 Sutton D, Pratt AE. Angiography of hemangiopericytoma. Preoperative vascular embolization as an adjunct to success- Clin Radiol 1967; 18: 324-9. ful resection of large retroperitoneal hemangiopericytoma. J 15 Yaghmai I. Angiographic manifestations of soft tissue and Urol 1976; 115: 206-8. osseous hemangiopericytomas. Radiology 1978; 126: 653-9. 24 Staples JJ, Robinson RA, Wen BC, Hussey DH. Heman- 16 Stephens DH, Sheedy PF, II, Hattery RR, Williamson B, Jr. giopericytoma - the role ofradiotherapy. Int J Radiat Oncol Diagnosis and evaluation of retroperitoneal tumor by com- Biol Phys 1990; 19: 445-51. puted tomography. AJR 1977; 129: 395-402. 25 Craven JP, Quigley TM, Bolen JW, Raker EJ. Current 17 Pinson CW, Remine SG, Fletcher WS, Braasch JW. Long management and clinical outcome of hemangiopericytomas. term results with primary retroperitoneal tumors. Arch Surg AmJ Surg 1992; 163: 490-3. 1989; 124: 1168-73. 26 Munoz AK, Berek JS, Fu YS, Heintz PA. Pelvic heman- 18 Lorigan JG, David CL, Evans HL, Wallace S. The clinical giopericytomas: a report of five cases and literature revew. and radiologic manifestation of hemangiopericytoma. AJR Gynecol Oncol 1990; 36: 380-2. 1989; 153: 345-9. 27 Wong PP, Yagoda A. Chemotherapy of malignant heman- 19 Alpern MB, Thorsen MK, Kellman GM, Pojunas K, giopericytoma. Cancer 1978; 41: 1256-60. Lawson TL. CT appearance of hemangiopericytoma. J Comput Assist Tomogr 1986; 10: 264-7. 20 Imachi M, Tsukamoto N, Tsukimori K, et al. Malignant hemangiopericytoma of the omentum presenting as an ovarian tumor. Gynecol Oncol 1990; 39: 208-13. Carcinoid syndrome due to a malignant somatostatinoma Omer Ozbakir, Fahrettin Kelestimur, Figen Ozturk, Erdogan Szfier, Ali Unal, Tahir E Patirolu, Kadri Guven Summary Metastatic spread is usually to the liver, with Somatostatinoma is one of the rarest involvement of lymph nodes and contiguous tumours of the endocrine pancreas. Car- spread also being common. It has been sug- dinal manifestations of a somatosta- gested that the expression of the classic triad of tinoma include gallstones, mild diabetes symptoms may be more common when liver mellitus, steatorrhoea, diarrhoea and metastases are present.3 Total tumour resection dyspepsia. Like any other pancreatic islet is the first line of therapy in patients with cell carcinoma, a somatostatinoma may pancreatic somatostatinoma, while chemo- http://pmj.bmj.com/ also produce several different hormones therapy is also frequently used either as the such as adrenocorticotropic hormone, primary mode of therapy in disseminated calcitonin, vasoactive intestinal polypep- disease or as adjunctive therapy after surgery.' tide, pancreatic polypeptide, gastrin, Carcinoid syndrome is a clinical entity which insulin, and glucagon. In many cases, the is usually caused by the humoral secretions of clinical picture is dominated by the effect carcinoid tumours that originate in the midgut. of these other hormones. We present a Lesions other than carcinoid tumours some- on September 26, 2021 by guest. Protected copyright. Erciyes University patient with somatostatinoma in which times secrete serotonin and present with symp- School ofMedicine, an immunocytochemical study of the toms of the carcinoid syndrome (see box 1). In Kayseri, Turkey specimens from pancreas and liver its most complete form, the carcinoid synd- Department of showed a weak positive reaction for gas- rome involves several different organ systems Internal Medicine for such as the vasomotor, cardiopulmonary and 0 Ozbakir trin besides a strong positive reaction F Keletimur somatostatin. Interestingly, this patient gastrointestinal systems. The cardinal manifes- A tnal also showed the signs of carcinoid syn- tations of this syndrome consist of K Guven drome which was successfully treated hepatomegaly, cutaneous flushing, facial telan- Department of with octreotide. giectasia, hypotension, diarrhoea, endocardial Pathology F Ozturk syndrome, oct- TE Patirolu Keywords: somatostatinoma, carcinoid Department of reotide Surgery Carcinoid syndrome: causes E Sozuer Gut endocrine tumours have a low incidence of ofthese * carcinoid tumours about 1 in 200 000 population and 60% ofthe thyroid Correspondence to are carcinoids.' Somatostatinomas appear to be * medullary carcinomas Doq Dr Fahrettin * oat-cell carcinomas ofthe lung Kelestimur, Erciyes one of the rarest gut endocrine tumours, and * pancreatic islet cell cancers 1lniversitesi Tip Fakultesi Iq the yearly incidence is estimated to be as low as * neuroblastomas Hastaliklari Anabilim Dali, 1 in 40 million people.2 In the majority of the * other chromaffin tumours 38039 Kayseri, Turkey patients, metastatic spread is evident at the Accepted 26 April 1995 time of presentation or shortly thereafter. Box 1 696 Ozbakir, Kelegtimur, Ozturk, et al lesions, bronchoconstriction and oedema. A total gastrectomy + splenectomy + distal Since the liver metabolises most of the pancreatectomy + roux-en-Y oesophagoje- liver serotonin to which it is exposed, the carcinoid junostomy procedure was performed. A Postgrad Med J: first published as 10.1136/pgmj.71.841.695 on 1 November 1995. Downloaded from syndrome does not often occur from a gast- biopsy from one of the nodules was also rointestinal primary lesion until massive liver obtained. Histopathological examination replacement by the tumour has occurred.4 revealed a malignant islet cell carcinoma of We have been unable to find a report in the pancreas with liver metastases. An literature of a patient with pancreatic somato- immunocytochemical study of specimens from statinoma leading to a well-documented car- both the pancreas and liver was performed cinoid syndrome, although some patients with which showed a strong positive reaction for flushing have been reported5'6 and somato- somatostatin (figures 1 and 2), a weak positive statinoma is known to secrete a variety of reaction for gastrin (figures 3 and 4) and hormones and peptides which may affect the negative reactions for insulin, glucagon, clinical presentation. adrenocorticotropic hormone and pancreatic polypeptide. Two weeks after surgery plasma Case report and serum levels of the following hormones were within the normal range: gastrin 64 ng/l, A 50-year-old woman was first admitted to the cortisol 358 nmol/l, insulin 21 pmol/l, C- department of surgery in December 1993 peptide 0.93 nmol/l, growth hormone 0.61 jig/l because of severe upper gastrointestinal and prolactin 21.44 jtg/l. Though the level of bleeding with the signs ofhypovolaemic shock. gastrin was within normal limits, this finding In her past medical history, there was nothing supported the existence of a gastrin-secreting but an appendectomy, 21 years earlier. A tumour as the entire stomach had been significant site of bleeding could not be removed. She was put on a once a week detected though an upper gastrointestinal 5-fluorouracil regimen and discharged. endoscopy was performed. An abdominal ult- The patient was re-admitted to the Depart- rasonogram showed multiple metastatic ment of Internal Medicine in September 1994 nodules in the liver with splenomegaly. Her with recurrent watery diarrhoea, cutaneous gastrointestinal bleeding could not be cont- flushing of the head and neck, nausea, and rolled by medical measures, and she underwent vomiting. Physical examination revealed a surgery. During the surgical procedure, a cutaneous flushing of the head and neck and tumoural enlargement at the tail ofthe pancreas increased bowel movements. The serum and multiple metastatic nodules in the liver biochemical values were: fasting blood sugar were observed, along with splenomegaly. 7.33 mmol/l, sodium 136 mmol/l, potassium http://pmj.bmj.com/ ) on September 26, 2021 by guest. Protected copyright. Figure 1 Pancreatic islets and tumour cells giving a Figure 3 Pancreatic islets and tumour cells giving a positive reaction for somatostatin (immunoperoxidase, weak positive reaction for gastrin (immunoperoxidase, x 100) x 100) Figure 2 Metastatic tumour cells in the liver giving a Figure 4 Metastatic tumour cells in the liver giving a positive reaction for somatostatin (immunoperoxidase, weak positive reaction for gastrin (immunoperoxidase, x 200) x 200) Carcinoid syndrome 697 3.4 mmol/l, chloride 99 mmol/l, total proteins 68 g/l, albumin 35 g/l, calcium 2.45 mmol/l Somatostatinoma: clinical features and phosphorus 0.65 mmol/l. Liver and kidney Postgrad Med J: first published as 10.1136/pgmj.71.841.695 on 1 November 1995. Downloaded from * gallstones function tests were normal. The 24-hour urine * mild diabetes mellitus 5-hydroxyindoleacetic acid (5-HIAA) level * steatorrhoea was 272
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