An autopsy case of von Recklinghausen's disease (vRD) associated with malignant pheochromocytoma is reported. 2007 Jan;30(1):59-64. doi: 10.1007/BF03347397. April 2009; Indian Journal of Critical Care Medicine 13(2):94-5 Although pheochromocytoma occurs in 1% of patients with von Recklinghausen's disease, composite tumors in this syndrome are much rarer, with isolated case reports in the literature. Erem C, Onder Ersöz H, Ukinç K, Hacihasanoglu A, Alhan E, Cobanoğlu U, Koçak M, Erdöl H. J Endocrinol Invest. The association between von Recklinghausen's disease and pheochromocytoma is present about 10% of cases. Prevention and treatment information (HHS). Abdominal neoplastic manifestations of neurofibromatosis type 1. A 48-year-old man with neurofibromatosis type 1 presented with chest pain, paroxysmal hypertension, tachycardia, and progressive respiratory insufficiency. Of 325 articles 118 are included in this review. Von Recklinghausen’s disease (VRD) is a genetic disorder characterized by the growth of tumors on the nerves. 3Department of pathology and INsERM U 916, Institut Bergonié, Bordeaux, France. Key words: gastrointestinal stromal tumors (GIST), pheochromocytoma, von Recklinghausen’s disease. HUSTON JR, STEWART WR. Pheochromocytoma is observed with higher frequency in patients with von Recklinghausen neurofibromatosis. We reviewed the literature on pheochromocytoma associated with von Recklinghausen's disease. The latter is most often asymptomatic. Herein, we present a case with von Recklinghausen's disease, right adrenal pheochromocytoma and multiple GISTs. Epub 2020 Sep 14. (Von Recklinghausen’s disease) for many years, which also affected his two children and his brother. Of the 148 patients 84% had solitary adrenal tumors, 9.6% bilateral adrenal disease and 6.1% ectopic pheochromocytomas. Background. The association between von Recklinghausen's disease and pheochromocytoma is present about 10% of cases. 2020 Oct 13;12(10):2965. doi: 10.3390/cancers12102965. A case of von Recklinghausen's disease with bilateral pheochromocytoma-malignant peripheral nerve sheath tumors of the adrenal and gastrointestinal autonomic nerve tumors J Urol. 2. The severity of NF-1 varies widely, and little is known about what causes a person to have a more severe or less severe case. Von Recklinghausen’s disease or neurofibromatosis type 1 (NF1) is a genetic disease with a prevalence of 1/2500 births. The association between von Reckinghausen's disease and optic nerve glioma/phaeochr … We report a case of 49 years old women who presented with elevated blood pressure and von Recklinghausen's neurofibromatosis. Results: They are usually benign (3 to 14% of malignant forms). The patient is a 36‐year‐old Japanese male and diagnosed as vRD both clinically and pathologically. The tumors presented satisfactory histological features in favor of pheochromocytoma … Laboratory examination showed a marked level in the urinary excretion of cathecholamine. Mean patient age was 42 years (range 1.5 to 74) in 87 women and 61 men at presentation with pheochromocytoma. National Library of Medicine The combination of Von Recklinghausen disease with a pheochromocytoma has a genetic linkage but is an exceedingly rare occurrence. The computed tomography showed a right adrenal tumor … The mean age at presentation with pheochromocytoma in von Recklinghausen's disease patients age is 42 years. Initial testing (for symptomatic patient or incidental finding) – can use plasma-free metanephrines alone, urine metanephrines alone, or both together 1.1. Please enable it to take advantage of the complete set of features! von Recklinghausen's disease and pheochromocytomas. Conclusions: A 19-year-old woman with von Recklinghausen's disease was referred to our hospital because of right adrenal pheochromocytoma. The association of Von Recklinghausen’s disease and a pheochromocytoma is found in 10% of cases. This site needs JavaScript to work properly. Laboratory examination showed a marked level in the urinary excretion of cathecholamine. Neurooncol Adv. Autopsy revealed malignant pheochromocytoma with liver, lung, bone and lymph nodes metastases. Mean patient age was 42 years (range 1.5 to 74) in 87 women and 61 men at presentation with pheochromocytoma. Purpose: LEHMAN DJ, Jr, ROSOF J. We report a case of 49 years old women who presented with elevated blood pressure and von Recklinghausen's neurofibromatosis. Malignant Pheochromocytoma With Ganglioneuroblastomatous Elements in a Patient With von Recklinghausen ’s Disease AKlRA NAKAGAWARA, MD,’ KEllCHl IKEDA, MD,’ MASAZUMI TSUNEYOSHI, MD,t YUTAKA DAIMARU, MD,t AND MUNETOMO ENJOJI, MDt A 14-year-old girl with numerous cafG-au-lait spots in her skin was hospitalized because of fever, weight loss, and a mass of the right upper … Of the 148 patients 6% died during pregnancy or a medical procedure, or due to hypertensive crisis without apparent provocation, 8.8% had gastrointestinal carcinoid tumors and 11.5% had metastases or local invasion from pheochromocytoma. J Community Hosp Intern Med Perspect. PMID: 11411100 2020 Jun 14;10(3):250-254. doi: 10.1080/20009666.2020.1767272. From their own experience and from the literature, Griffiths et al. Pheochromocytomas are associated with several genetic syndromes: Von Hippel-Lindau syndrome (vHL) Multiple endocrine neoplasia type 2 (MEN2) Neurofibromatosis type 1 (NF1), also called Von Recklinghausen's disease; Familial paraganglioma syndrome (SDHx) Thereafter, the patient presented severe hypertensive and hypotensive crises, … The largest series of VHL patients with pheochromocytomas was described by Walther et al [ 23 ]. Clipboard, Search History, and several other advanced features are temporarily unavailable. Hig… The rare association between Von Recklin-ghausen's disease (VRD) and tumours other than in central nervous system is well recognized. Arif AA, Kim PTW, Melck A, Churg A, Schwartz Z, Stuart HC. Unable to load your collection due to an error, Unable to load your delegates due to an error. During routine preop-erative examination he was incidentally found to have hypertension and gave a history of 2 years of headache. While a pheochromocytoma is a neuroendocrine tumor characterized by intermittent hypertension. 1999, 162: 1582-6. The association between von Recklinghausen's disease and pheochromocytoma is present about 10% of cases. An autopsy case of von Recklinghausen's disease (vRD) associated with malignant pheochromocytoma is reported. 3Department of pathology and INsERM U 916, Institut Bergonié, Bordeaux, France. Read "Malignant pheochromocytoma with ganglioneuroblastomatous elements in a patient with von recklinghausen's disease, Cancer" on DeepDyve, the largest online rental service for scholarly research with thousands of academic publications available at your fingertips. Riccardi C, Perrone L, Napolitano F, Sampaolo S, Melone MAB. Our patient was considerably older. FOIA Massive hemorrhage into an adrenal pheochromocytoma. The mean age at diagnosis was 29.9 year, which was statistically significantly younger than the mean age at diagnosis in a control group of patients with sporadic pheochromocytoma (39.7 years)[ 24 ]. The association between von Recklinghausen's disease and pheochromocytoma is present about 10% of cases. Pheochromocytomas occur in a small but defined number of patients with von Recklinghausen's disease, and can be associated with significant morbidity and mortality if not detected. To the best of our knowledge this is the first report of a patient with von Recklinghausen's disease developing polymyositis. Neurofibromatozis type 1 is a complex disease, with patients having an increased prevalence of benign and malignant neoplasms throughout the body due to mutations of the NF gene leading to abnormal tumor suppression. Screening of patients with von Recklinghausen's disease and hypertension or before provocative procedures or pregnancy seems to be indicated. her two sisters had von Recklinghausen's disease and another sister suffered from follicular carcinoma of the thyroid gland. We report a case of 49 years old women who presented with elevated blood pressure and von Recklinghausen's neurofibromatosis. N Engl J Med. FOIA Multiple gastrointestinal stromal tumors and pheochromocytoma in a patient with von Recklinghausen's disease November 2012 International Journal of Surgery Case Reports 4(2):216-218 However, the concomitance of VRD, a carcinoid of the ampulla of Vater, and a pheochromocytoma has been described very rarely in literature. Walther MM, Herring J, Enquist E, Keiser HR, Linehan WM: Von Recklinghausen's disease and pheochromocytomas. Understanding the Biological Activities of Vitamin D in Type 1 Neurofibromatosis: New Insights into Disease Pathogenesis and Therapeutic Design. Laboratory examination showed a marked level in the urinary excretion of cathecholamine. Preferred in 1.1.1.1. Key words: gastrointestinal stromal tumors (GIST), pheochromocytoma, von Recklinghausen’s disease. Von Recklinghausen’s disease is a hereditary condition, is transmitted in the autosomal dominant mode, which requires genetic screening, because it has a variable expression depending on the individual. 'Adrenal rush' in a patient with Neurofibromatosis-1. HEMORRHAGIC PHEOCHROMOCYTOMA WITH SHOCK AND ABDOMINAL PAIN. Von Recklinghausen's Disease (Neurofibromatosis) Associated with Malignant Pheochromocytoma Okada, Eikichi; Shozawa, Takeshi 1984-03-01 00:00:00 An autopsy case of von Recklinghausen's disease (vRD) associated with malignant pheochromocytoma is reported. Multiple gastrointestinal stromal tumors and pheochromocytoma in a patient with von Recklinghausen's disease November 2012 International Journal of Surgery Case Reports 4(2):216-218 Screening of patients with von Recklinghausen's disease and hypertension or before provocative procedures or pregnancy seems to be indicated. Pheochromocytomas may also develop in people who have von Hippel–Lindau disease and in those who have neurofibromatosis (von Recklinghausen disease) or a number of other genetic diseases. Recently he was admitted to a peripheral hospital for repair of bilateral inguinal hernia. Please enable it to take advantage of the complete set of features! To our knowledge, only 7 cases of malignant pheochromocytoma associated with von Recklinghausen's disease have been reported in Japan. The incidence in the general population is believed to be around 0.05% based on autopsy series9. METHODS/RESULTS: We report on a case of malignant bilateral pheochromocytoma with hepatic and pulmonary metastasis, associated with von Recklinghausen's neurofibromatosis. Materials and methods: 1956 Mar 8; 254 (10):474–476. In her family history, her mother and one of her two sisters had von Recklinghausen's disease and another sister suffered from follicular carcinoma of the thyroid gland. RESULTS Pheochromocytomas have been clinically identified in 0.1 to 5.7% of patients with von Recklinghausen's disease. Laboratory examination showed a marked level in the urinary excretion of cathecholamine. The diagnosis of NF1 is based on criteria established by US National Institutes of … We describe a 35-year-old Iranian female with previously diagnosed von Recklinghausen ' s disease who suffered spontaneous rupture of an adrenal pheochromocytoma, misdiagnosed as renal colic followed by an extensive retroperitoneal hematoma, … The computed tomography showed a right adrenal tumor suggesting a pheochromocytoma. The latter is most often asymptomatic. The authors discuss the pathogenetic hypothesis of this rare pathological association, the diagnostic methods and the therapeutic procedure. Careers. We review the literature and characterize the clinical findings of von Recklinghausen's associated pheochromocytoma. https://columbiasurgery.org/conditions-and-treatments/pheochromocytoma We report a case of 49 years old women who presented with elevated blood pressure and von Recklinghausen's neurofibromatosis. Of the 148 patients 84% had solitary adrenal tumors, 9.6% bilateral adrenal disease and 6.1% ectopic pheochromocytomas. Intestinal Obstruction and pheochromocytoma in a patient suffering from von Recklinghausen’s Disease and B. Chamontin1 1Department of Internal Medicine and Hypertension, CHU Rangueil, Toulouse, France. Privacy, Help Most gastrointestinal stromal tumors (GISTs) are solitary and sporadic. 10.1016/S0022-5347(05)68171-2. Pheochromocytoma is observed with higher frequency in patients with von Recklinghausen neurofibromatosis. Laboratory examination showed a marked level in the urinary excretion of cathecholamine. Bethesda, MD 20894, Copyright ’ This article reports a case of malig- nant catecholamine-secreting pheochromocytoma con- taining ganglioneuroblastomatous elements occurring in a patient with von Recklinghausen’s disease; such con- comitance has never before been reported to our knowl- edge. Pheochromocytomas occur in a small but defined number of patients with von Recklinghausen's disease, and can be associated with significant morbidity and mortality if not detected. An adrenal mass can … Case. 2020 Jun 25;2(Suppl 1):i124-i133. Surgery achieved good results and the patient is well seven years postoperatively. Am J Med. J Community Hosp Intern Med Perspect. Of the 148 patients 84% had solitary adrenal tumors, 9.6% bilateral adrenal disease and 6.1% ectopic pheochromocytomas. Intestinal Obstruction and pheochromocytoma in a patient suffering from von Recklinghausen’s Disease and B. Chamontin1 1Department of Internal Medicine and Hypertension, CHU Rangueil, Toulouse, France. The leiomyosarcoma recurred and metastasized to the liver. From their own experience and from the literature, Griffiths et al. The estimated prevalence of pheochromocytomas in hypertensive adults is thought to range from 0.1 to 0.6%. Multiple GISTs however, are associated with clinical syndromes particularly von Recklinghausen's disease. The duodenal carcinoids had an unusual morphology. Forty-eight year old male patient was admitted to our Emergency Department with the complaint of blackish stool discharge. Type 2 VHL disease, where pheochromocytoma develops, accounts for 10% of VHL disease cases [21, 22]. Mean patient age was 42 years (range 1.5 to 74) in 87 women and 61 men at presentation with pheochromocytoma. Symptoms related to pheochromocytoma … Pheochromocytoma occurs in a small but defined number of patients with von Recklinghausen's disease (in 0.1 to 5.7% of patients with von Recklinghausen's disease), and can be associated with significant morbidity and mortality if not detected. Our patient was considerably older. The majority of cases are sporadic. We here document two cases of von Recklinghausen's disease with both pheochromocytomas and gastrointestinal stromal tumors (GIST). RESULTS: Pheochromocytomas have been clinically identified in 0.1 to 5.7% of patients with von Recklinghausen's disease. Its association with a pheochromocytoma varies from 0.5 to 5%. Plasma and urinary catecholamines level were elevated. There are … A 19-year-old woman with von Recklinghausen's disease was referred to our hospital because of right adrenal pheochromocytoma. Phéochromocytome et maladie de von Recklinghausen Pheochromocytoma and von Recklinghausen's disease. 2Department of Histopathology, CHU purpan, Toulouse, France. It is likely that nearly 50% of people who have pheochromocytomas have a genetic or familial disease … Tumors secreted epinephrine and norepinephrine, and 87% demonstrated metaiodobenzylguanidine uptake. The properly Would you like email updates of new search results? NF-1 was formerly known as von Recklinghausen disease, after the researcher (Friedrich Daniel von Recklinghausen) who first documented the disorder. He died from right adrenal tumor with wide spread metastases to lungs and bone marrow. The disease is characterized by skin lesions called café-au-lait spots and cutaneous neurofibromas . Patients experience a lifetime of morbidity and increased risk of mortality, depending on the extent of the disease. 8600 Rockville Pike A cesarean section was performed at 37 weeks of pr … Accessibility The tumor was detected incidentally with the abdominal ultrasonography when she complained epigastralgia to the home doctor who treated her hypertension. Screening of patients with von Recklinghausen's disease and hypertension or before provocative procedures or pregnancy …. Dare AJ, Gupta AA, Thipphavong S, Miettinen M, Gladdy RA. The tumor was removed by laparoscopy … Cancers (Basel). Neurofibromatosis type 1 associated with pheochromocytoma: a case report and a review of the literature. Am J Case Rep. 2021 Jan 16;22:e927761. PMID: 11411100 MARSHALL D. Glioma of the optic nerve as a manifestation of von Recklinghausen's disease. In 5-10% of cases, a pheochromocytoma is a manifestation of an underlying condition including 1-4,6: 1. multiple endocrine neoplasia type II (MEN II): both MEN IIa and MEN IIb 1.1. account for … rare cause of pheochromocytoma. Pheochromocytomas are rare neuroendocrine tumors. A cesarean section was performed at 37 weeks of pregnancy. Neurofibromatosis type 1 (NF1), which is also known as von Recklinghausen's disease, is a genetic disorder that involves autosomal-dominant mutations. CAS Article PubMed Google Scholar 8. Spontaneous rupture of an adrenal pheochromocytoma is extremely rare and can be lethal because of dramatic changes in the circulation. A 32-year-old woman who died suddenly during pregnancy as a result of an adrenal phaeochromocytoma also suffered from von Recklinghausen's disease. CONCLUSIONS: Pheochromocytomas occur in a small but defined number of patients with von Recklinghausen's disease, and can be associated with significant morbidity and … Careers. Would you like email updates of new search results? Mean patient age was 42 years (range 1.5 to 74) in 87 women and 61 men at presentation with pheochromocytoma. Clipboard, Search History, and several other advanced features are temporarily unavailable. 8600 Rockville Pike Pheochromocytomas are rare neuroendocrine tumors. To our knowledge, only 7 cases of malignant pheochromocytoma associated with von Recklinghausen's disease have been reported in Japan. As far as we know, this paper is the first report of a patient with von Recklinghausen's disease associated with both pheochromocytoma and non … 21 years earlier she had an optic nerve glioma successfully removed. 1. 1965 Sep; 39:502–504. We here document two cases of von Recklinghausen's disease with both pheochromocytomas and gastrointestinal stromal tumors (GIST). Only 1% … A Grateful Med search for the years 1966 to 1999 was performed on the subjects, "von Recklinghausen" and "neurofibromatosis." 10 The association between von Recklinghausen's disease and tumors of neurogenic and neuroendocrine origin, such as meningiomas and pheochromocytomas is … Genetic mutations and pheochromocytoma. 2Department of Histopathology, CHU purpan, Toulouse, France. The duodenal carcinoids had an unusual morphology. It is very rare that these three disorders are found to occur simultaneously, although the fact that preoperative detection of GIST is difficult except with large tumors may be important in this respect. Cardiovascular complications induced by pheochromocytoma associated with neurofibromatosis type 1 (von Recklinghausen’s disease) – case report and review of literature Neurofibromatosis type 1 (also known as von Recklinghausen’s disease) is a genetic disorder characterized by neurofibromas, skin pigmentation, eye and bone abnormalities. The patient is a 36‐year‐old Japanese male and diagnosed as vRD both clinically and pathologically. The association between von Recklinghausen's disease and pheochromocytoma is present about 10% of cases. Privacy, Help von Recklinghausen’s disease is listed as one of the neurocristopathies. This site needs JavaScript to work properly. His physical examination revealed that, arterial blood pressure was 175/95 mmHg, heart rate was 102 1/min, multiple neurofibromas all … We report a 36 years old female with von Recklinghausen neurofibromatosis who developed mild hypertension during the fourth month of pregnancy. Articles from the Grateful Med search were then reviewed to identify older publications. Unable to load your collection due to an error, Unable to load your delegates due to an error. Neurofibromatosis type 1 (NF1), or von Recklinghausen’s disease, is diagnosed upon clinical criteria, and the study of PHEO is advised if hypertension is present. Pheochromocytoma Multisystem Crisis and Masquerading Disseminated Histoplasmosis in a Neurofibromatosis Type 1 Patient With Bilateral Adrenal Tumors. The tumor was histologically confirmed to be pheochromocytoma at the operation. 2020. doi: 10.12659/AJCR.927761. Conclusion Von Recklinghausen’s disease is a hereditary condition, is transmitted in the autosomal dominant mode, which requires genetic screening, because it has a variable expression depending on the individual. Accessibility Of the 148 patients 84% had solitary adrenal tumors, 9.6% bilateral adrenal disease and 6.1% ectopic pheochromocytomas. The prevalence of NF1 in … Introduction It is known that von Recklinghausen’s disease (neurofibromatosis I; NF1) is occasionally associated with pheochromocytoma development and also to a larger extent with GIST. Pheochromocytomas have been clinically identified in 0.1 to 5.7% of patients with von Recklinghausen’s disease. (1983) collected 3 patients with duodenal carcinoid tumor in association with neurofibromatosis and pheochromocytoma and 4 patients with duodenal carcinoid with either von Recklinghausen disease or pheochromocytoma. Case Report Introduction It is known that von Recklinghausen’s disease (neurofibromatosis I; NF1) is occasionally associated with pheochromocytoma development and also to a larger extent with GIST. Heniford B, Arca M, Walsh M, Gill IS: Laparoscopic adrenalectomy for metastasis or cancer. Keywords: Pheochromocytoma; Von recklinghausen; NEM type1 Introduction Von Recklinghausen’s disease or neurofibromatosis type 1 (NF1) is a genetic disease with a prevalence of 1/2500 births. It is very rare that these three disorders are found to occur simultaneously, although the fact that preoperative detection of GIST is difficult except with large tumors may be important in this respect. However, it is very rare for all three to occur simultaneously. Neurofibromatosis type I (NF-1) is a complex multi-system human disorder caused by the mutation of a gene on chromosome 17 that is responsible for production of a protein, called neurofibromin, which is needed for normal function in many human cell types.NF-1 causes tumors along the nervous system which can grow anywhere on the body. Neurofibromatosis, or von Recklinghausen disease, is characterized by congenital anomalies (often benign tumors) of the skin, nervous system, bones, and endocrine glands. 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