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Gastric Stromal Sarcoma, Pulmonary Chondroma, and Extra-adrenal Paraganglioma (Carney Triad): Natural History, Adrenocortical Component, and Possible Familial Occurrence

  • J. Aidan Carney
    Correspondence
    Address reprint requests and correspondence to J. Aidan Carney, MD, Emeritus Staff Center, Mayo Clinic Rochester, 200 First St SW, Rochester, MN 55905
    Affiliations
    Department of Laboratory Medicine and Pathology (emeritus member), Mayo Clinic Rochester, Rochester, Minn
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      Objective

      To investigate the natural history of the triad of gastric stromal sarcoma, pulmonary chondroma, and extra-adrenal paraganglioma, a rare syndrome of unknown cause primarily affecting young women.

      Methods

      Mayo Clinic records, the world literature, and the author's files were searched for patients with all or 2 of the 3 tumors.

      Results

      Seventy-nine patients, 67 women and 12 men, were identified, 17 (22%) with the 3 tumors and 62 (78%) with 2 tumors. Forty-two (53%) had gastric and pulmonary tumors, the most common combination. The longest interval between detection of the first and second components was 26 years (mean, 8.4 years; median, 6 years). Follow-up ranged from 1 year to 49 years (mean, 20.6 years; median, 20 years). Sixty-four patients (81%) were alive, 19 (24%) apparently free of disease and 45 (57%) with residual or metastatic tumors. Thirty-two patients (41%) had 1 or more local recurrences of the gastric sarcoma; the longest interval to first recurrence was 36 years. Twenty-one survivors (27%) had hepatic metastatic gastric sarcoma with follow-up of 1 year to 25 years (mean, 9.3 years; median, 7 years). Fifteen patients (19%) were dead, 10 (13%) of whom died of the disorder. Ten patients (13%) had nonfunctioning adrenocortical tumors. Two patients each had a sibling with 1 component of the triad.

      Conclusions

      The triad is a chronic, persistent, and indolent disease. Benign adrenocortical tumors are a component of the condition. The disorder may be familial.
      131I MIBG (radioactive iodine-metaiodobenzylgnanidine)
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      References

        • Carney JA
        • Sheps SG
        • Go VLW
        • Gordon H
        The triad of gastric leiomyosarcorna, functioning extra-adrenal paraganglioma and pulmonary chondroma.
        N Engl J Med. 1977; 296: 1517-1518
        • Margulies KB
        • Shcps SG
        Carney's triad: guidelines for management.
        Mayo Clin Proc. 1988; 63: 496-502
        • Carney JA
        The triad of gastric epithelioid leiomyosarcorna, pulmonary chondroma, and functioning extra-adrenal paraganglioma: a five-year review.
        Medicine (Baltimore). 1983; 62: 159-169
        • Appelman HD
        • Hclwig EB
        Gastric epithelioid leiomyoma and leiomyosarcorna (leiomyoblastoma).
        Cancer. 1976; 38: 708-728
        • Jordan SM
        A case of multiple leiomyosarcomata of the stomach.
        Surg Clin North Am. 1938; 18: 683-686
        • Lahey FH
        • Colcock BP
        Diagnosis and surgical management of leiomyomala and leiomyosarcomata of stomach.
        Ann Surg. 1940; 112: 671-686
        • Fife R
        • Jarvie J
        Leiomyosarcorna of stomach: brief review including report of case with multiple metastases.
        Glasgow Med J. 1952; 33: 490-495
        • Bateson EM
        Cartilage-containing tumours of the lung: relationship between the purely cartilaginous type (chondroma) and the mixed type (so-called hamartoma); an unusual case of multiple tumours.
        Thorax. 1967; 22: 256-259
        • Dupuy R
        • Daumet P
        • Gavelle P
        Association de tumeurs conjunctives pulmonaircs à des tumeurs gastriques.
        Sem Hop Paris. 1967; 43: 889-893
        • Vio A
        • Abate L
        • Leogrande G
        Considerazioni su un singolare Caso di tumore gastrico a nodi multipli (leiomioma bizzarro di Stout) associato ad amartocondroma polmonare in una giovinetta di 12 anni.
        Ann Ital Chir. 1969; 45: 750-768
        • Bateson EM
        Histogcncsis of intrapu I monary and endobronchial hamartomas and chondrumas (cartilage-containing tumours): a hypothesis.
        J Pathol. 1970; 101: 77-83
        • De Castro FJ
        • Olsen WR
        • Littler ER
        Gastric Iciomyoblastoma in an adolescent.
        Am JSurg. 1972; 123: 614-616
        • Muiler M
        • Ryneki PV
        • Saegesscr F
        • Gardiol D
        Hamartomes pulmonaires multiples bilatéreaux: a propos d'une jeunc femme préalablement opérée d'une tumeur musculaire de 1'estomac.
        Schweii Med Wochenschr. 1974; 104: 511-514
        • Lacquct LK
        • Moulijn AC
        • Jongerius CM
        • Limburg M
        • Rensing JB
        Intrathoracic chemodectoma with multiple localisations.
        Thorax. 1977; 32: 203-209
        • Roenspies U
        • Morin D
        • Gloor E
        • von Hochstetter AR
        • Saegesser F
        • Scnning A
        Bronchopulmonale Humartome, Chondrome, Fibromc und Myxome.
        Schweiz Med Wochenschr. 1978; 108: 332-339
        • Tisell L-E
        • Angervall L
        • Dahl I
        • Merck C
        • Zachrisson BF
        Recurrent and metastasizing gastric leiomyoblastoma (epithelioid leiomyosarcoma) associated with multiple pulmonary chondro-hamartomas: long survival of a patient treated with repeated operations.
        Cancer. 1978; 41: 259-265
        • Knake JB
        • Gross MD
        Extraadrenal paraganglioma, pulmonary chondroma, and gastric leiomyoblastoma: triad in young females.
        AIR Am J Roentgenol. 1979; 132: 448-451
        • Grace MP
        • Batist G
        • Grace WR
        • Gillooley JF
        Aorticopulmonary paraganglioma and gastric Iciomyoblastoma in a young woman.
        Am J Med. 1981; 70: 1288-1292
        • Chander S
        • Oliverio Jr, R
        • Fermon C
        • Chander P
        Triad of neoplasms: leiomyosarcoma, functioning extra-adrenal tumors, and pulmonary chondroma.
        NY State J Med. 1981; 81: 392-393
        • Dajee A
        • Dajee H
        • Hinrichs S
        • Lillington G
        Pulmonary chondroma, extra-adrenal paraganglioma, and gastric leiomyosarcoma: Carney's triad.
        J Thorac Cardiovasc Surg. 1982; 84: 377-381
        • Baril A
        • Boucheron S
        • Cuilleret J
        • Pichon A
        • Serpenlié P
        Association d'une tumeur myoi'de de I'estomac et d'un paragangliome extrasurrénalien fonctionnel: une nouvelle observation en faveur de l'individualisation d'un syndrome.
        Arch Anal Cytol Palhot. 1982; 30: 185-189
        • McGahan JP
        Carney syndrome: usefulness of computed tomography in demonstrating pulmonary chondromas.
        J Compul Assist Tomogr. 1983; 7: 137-139
        • Chahinian AP
        • Kirschner PA
        • Dikman SH
        • Rammos KS
        • Holland JF
        Pulmonary mctastatic leiomyosarcoma coexisting with pulmonary chondroma in Carney's triad.
        Arch intern Med. 1983; 143: 1462-1464
        • Xu CP
        • Li GY
        • Liao FQ
        • Hong DL
        • Liu WW
        Gastric leiomyoblastoma with multiple pulmonary chondrohamartomas: a case report.
        Chin MedJ(Engl). 1983; 96: 367-369
        • Sawai T
        • Ikeda T
        • Ito S
        • et al.
        Case of three peculiar tumors of gastric epithelioid leiomyosarcoma, retroperjtoneal paraganglioma and pulmonary chondroma [in Japanese].
        Gan No Rinsho. 1983; 29: 177-182
        • Lacquet LK
        • Jongerius CM
        • Limburg M
        • Rensing JB
        La triade: chémodectome du médiastin, chondrome du poumon et léio-myome dc l'oesophagc.
        Rev Pneumol Clin. 1984; 40: 63-67
        • Cuilleret J
        • Baril A
        • Boucheron S
        • Pichon A
        • Serpantie P
        La triade de Carney.
        Presse Med. 1984; 13: 79-82
        • Bigolli G
        • Lopez M
        Concomitanza di leiomiosarcoma epitelioide gastrico, paraganglioma extrasurrenalico funzionalc e condroma del polmone.
        Clin Ter. 1984; 110: 271-275
        • Cucto-Garcia L
        • Shub C
        • Sheps SG
        • Puga FJ
        Two-dimensional echocardiographic detection of mediastinal pheochromocytoma.
        Chest. 1985; 87: 834-836
        • Rüfenacht H
        • Mihatsch MJ
        • Jundt K
        • Gächter A
        • Tanner K
        • Heitz PU
        Gastric epithelioid leiomyomas, pulmonary chondroma, non-functioning metastasizing extra-adrenal paraganglioma and myxoma: a variant of Carney's triad; report of a patient.
        Klin Wochenschr. 1985; 63: 282-284
        • Mishkin FS
        • Vasinrapee P
        • Vora L
        • Bier R
        • Mackabee JR
        Carney's triad: radiographic diagnosis, natural history and importance of pulmonary chondromas.
        J Can Assoc Radiol. 1985; 36: 264-266
        • Walker P
        • Dvorak AM
        Gastrointestinal autonomic nerve (GAN) tumor: ultrastructural evidence for a newly recognized entity.
        Arch Pathol Lab Med. 1986; 110: 309-316
        • Segal A
        • Carcllo S
        • Caterina P
        • Papadimitriou JM
        • Spagnolo DV
        Gastrointestinal autonomic nerve tumors: a clinicopathological, immunohistochemical and ultrastructural study of 10 cases.
        Pathology. 1994; 26: 439-447
        • Raafat F
        • Salman WD
        • Roberts K
        • Ingram L
        • Rees R
        • Mann JR
        Carney's triad: gastric leiomyosarcoma, pulmonary chondroma and extra-adrenal paraganglioma in young females.
        Histopathology. 1986; 10: 1325-1333
        • Tortella BJ
        • Matthews JB
        • Antonioli DA
        • Dvorak AM
        • Silen W
        Gastric autonomic nerve (GAN) tumor and extra-adrenal paraganglioma in Carney's triad: a common origin.
        Ann Surg. 1987; 205: 221-225
        • McLaughlin SJ
        • Dodge EA
        • Ashworth J
        • Connors J
        Carney's triad.
        Aust N Z J Surg. 1988; 58: 679-681
        • Mazas-Artasona L
        • Romeo M
        • Felices R
        • et al.
        Gastro-cesophageal leiomyoblastomas and multiple pulmonary chondromas: an incomplete variant of Carney's triad.
        Br J Radiol. 1988; 61: 1181-1184
        • Evans RA
        • Salisbury JR
        • Gimson A
        • Michell MJ
        Indolent gastric epithelioid leiomyosarcoma in Carney's triad.
        Clin Radiol. 1990; 42: 437-439
        • Tossou H
        • Delamarre J
        • Sevestre H
        • Deschepper B
        • Jouet-Gondry C
        • Capron JP
        Syndrome de Carney révélé par un hémoperitoine.
        Gastroenlerol Clin Biol. 1990; 14: 394-398
        • Beigelman C
        • Perez T
        • Rémy-Jardjn M
        • Ribel M
        • Rémy J
        La tnade de Carney: I'imagerie dans le diagnostic et la surveillance.
        Rev Am Med. 1990; 2: 171-177
        • Le Blanc L
        • Laurent M
        • Bokobza B
        • Michot F
        • Teniere P
        Triade de Carney: un nouveau cas associé à un adénome cortico-surrénalien.
        Gastroenlerol Clin Biol. 1990; 14: 399-401
        • Cardwell RJ
        • Solaiman AR
        • Kim K
        • Thomford NR
        Carney's triad with recurrent gastric Iciomyoblastoma.
        Am Surg. 1990; 56: 355-359
        • Khafagi FA
        • Shapiro B
        • Fischer M
        • Sisson JC
        • Hutchinson R
        • Beierwaltes WH
        Phaeochromocytoma and functioning paraganglioma in childhood and adolesence: role of iodine 131 meta-iodobenzylguanidine.
        Eur J Nucl Med. 1991; 18: 191-198
        • Persson S
        • Kindblom LG
        • Angervall L
        • Tisell LE
        Metastasizing gastric epithelioid Iciomyosarcomas (leiomyoblastomas) in young individuals with long-term survival.
        Cancer. 1992; 70: 721-732
        • Blei E
        • Gonzalez-Crussi F
        The intriguing nature of gastric tumors in Carney's triad: ultrastructural and immunohistochemical observations.
        Cancer. 1992; 69: 292-300
        • Barcena R
        • Erdozain JC
        • San Roman AL
        • Garcia M
        • Boixeda D
        • Perez FA
        Triada de Carney: breve revision a propdsito de un caso.
        Rev Clin Esp. 1992; 190: 410-412
        • Percz-Atayde AR
        • Shamberger RC
        • Kozakewich HWP
        Neuroectodermal differentiation of the gastrointestinal tumors in the Carney triad: an ultrastructural and immunohistochemical study.
        Am J Surg Pathol. 1993; 17: 706-714
        • Argos MD
        • Ruiz A
        • Sanchez F
        • Garcia C
        • Gaztambide J
        Gastric Iciomyoblastoma associated with extraadrenal paraganglioma and pulmonary chondroma: a new case of Carney's triad.
        J PediatrSurg. 1993; 28: 1545-1549
        • Fernandez Aguilar JR
        • Lopez Rueda B
        • Santoyo Santoyo J
        • et al.
        Triada de Camcy: lciomiosarcoma gastrico recidvado.
        Cirug Esp. 1993; 54: 352-354
        • Acha T
        • Picazo B
        • Garcia-Martín FJ
        • Urda A
        • Campos J
        Carney's triad: a propos of a new case.
        Med Pediatr Oncol. 1994; 22: 216-220
        • Prayag AK
        • Vora IM
        • Antani RD
        • Shenoy S
        Carney's triad with twin lesions.
        Indian J Gastroenlerol. 1993; 12: 61
        • Erb RE
        • Delbeke D
        • Falkc THM
        • Sandier MP
        Magnetic reso nance imaging of Carney's triad.
        South MedJ. 1994; 87: 951-955
        • Schmutz GR
        • Fisch-Ponsot C
        • Sylvestre J
        Carney syndrome: radiologic features.
        Can Assoc RadiolJ. 1994; 45: 148-150
        • Luchi K
        Carney's triad.
        Ryoikihetsu Shokogun Shiriiu. 1994; 4: 687-690
        • Kawamura M
        • Takahashi Y
        • Sakata Y
        Carney's triad with gastric leiomyosarcoma and multiple hamartomas: a case report (in Japanese].
        JJap Assoc Chest Surg. 1994; 8: 190-195
        • Aulicino MR
        • Hytiroglou P
        • Dikman SH
        • Kaneko M
        Gastric stump carcinoma occurring in a patient with Carney's triad 38 years after partial gaslrectomy for epithelioid leiomyosarcoma [leller].
        Arch Intern Med. 1995; 155 (547.): 544
        • Amoroso A
        • Pasquarelli C
        • Di Rosa R
        • Paggi A
        • Addessi MA
        Associazione feocromocitoma-leiomioblastoma gastrico: pos-sibile expressione di triade di Carney; un caso di nostra osservazione.
        Recenti Prog Med. 1995; 86: 348-352
        • Majcrus B
        • Dekoninck X
        • Debongnte JC
        • Machiels J
        • Bleeckx AF
        • Wibin E
        Syndrome de Carney: deux nouveaux cas.
        Ann Chir. 1996; 50: 470-473
        • Salgado M
        • Galan MC
        • Pctegri A
        • Ribé A
        • Grau JJ
        Carney's triad: incomplete presentation of a patient with gastric leiomyosarcoma and extra-adrenal paragangliotna.
        Neopiasia. 1996; 13: 182-185
        • de Jong E
        • Mulder W
        • Nooitgedacht E
        • Taat CW
        • Bras J
        Carney's triad.
        EurJSurg Oncol. 1998; 24: 147-149
        • Lamovec J
        • Frkovic-Grazio S
        • Bracko M
        Nonsporadic cases and unusual morphological features in pheochromocytoma and paraganglioma.
        Arch Pathol Lab Med. 1998; 122: 63-68
        • Hirota S
        • Isozaki K
        • Moriyama Y
        • et al.
        Gain-of-function mutations of c-kir in human gastrointestinal stromal tumors.
        Science. 1998; 279: 577-580
        • Carney JA
        Adrenal gland.
        in: Sternberg SS Histology for Pathohgists. 2nd ed. Lippincoll-Raven Publishers, Philadelphia, Pa1997: 1107-1131
        • McKusick VA
        11th ed. Mendelian Inheritance in Man: A Catalog of Human Genes and Genetic Disorders. Vol I. The Johns Hopkins University Press, Baltimore, Md1994: 1093 (Entry 168000.)
        • Chase WH
        Familial and bilateral tumours of carotid body.
        J Pathol Bacteriol. 1933; 36: 1-12
        • Sprong Jr, DH
        • Kirby FG
        Familial carotid body tumors: report of 9 cases in 11 siblings.
        Ann West Med Surg. 1949; 3: 241-242
        • Lewison EF
        • Weinberg T
        Carotid body tumors: case report of bilateral carotid body tumors with unusual family incidence.
        Surgery. 1950; 27: 437-448
        • Desai MG
        • Patel CC
        Heredo-familial carotid body tumors.
        Clin Radiol (Land). 1961; 12: 214-218
        • van der Borden J
        Bilateral non-chromaffin tympano-jugular paraganglioma.
        J Laryngol Otol. 1967; 81: 445-448
        • Ladenhetm JC
        • Sachs Jr, E
        Familial tumors of the “glomus jugulare”: report of 2 cases, with a note on the vascular origin of the glomera.
        Neurology. 1961; 11: 303-309
        • Rush Jr, BF
        Familial bilateral carotid body tumors.
        Ann Surg. 1963; 157: 633-636
        • Katz AD
        Carotid body tumors in a large family group.
        Am J Surg. 1964; 108: 570-573
        • Kroll AJ
        • Alexander B
        • Cochios F
        • Pechet L
        Hereditary deficiencies of clotting factors VII and X associated with carotid-body tumors.
        NF.ngUMed. 1964; 270: 6-13
        • Farr W, H
        Carotid body tumors: a thirty year experience at Memorial Hospital.
        Am J Surg. 1967; 114: 614-619
        • Del Fante FM
        • Watkins Jr, E
        Chemodectoma of the heart in a patient with multiple chemodectomas and familial history: case report and survey of literature.
        Lahey Clin Found Butl. 1967; 16: 224-229
        • Beard M
        • McQuarrie DG
        Brotherly lumps: familial occurrence of chemodectoma.
        Minn Med. January 1968; 51: 79-82
        • Piaget F
        • Charachon R
        • Barge M
        • Gros JC
        • Junien-Lavillauroy C
        A propos de deux observations de tumeur du glomus carotidien: dont un cas familial associé à une tumeur du glomus jugulaire.
        Ann Otolaryngol Chir Cervicofac. 1968; 85: 181-188
        • Ribet M
        • Deminatti M
        • Desaulty-Cousin A
        • Meignie P
        Tumcurs du glomus carotidien ct maladie glomique familiale.
        Presse Med. 1969; 77: 1043-1046
        • Sugarbaker EV
        • Chretien PB
        • Jacobs B, J
        Bilateral familial carotid body tumors: report of a patient with an occult contralateral tumor and postoperative hypertension.
        Ann Surg. 1971; 174: 242-247
        • Pratt LW
        Familial carotid body tumors.
        Arch Otolaryngol. 1973; 97: 334-336
        • Boon CM
        Twee families met glomustumoren.
        Ned Tijdschr Geneeskd. 1976; 120: 505-512
        • Kahn LB
        Vagal body tumor (nonchromaffin paraganglioma, chemodectoma, and carotid body-like tumor) with cervical node metastasis and familial association: ultrastructural study and review.
        Cancer. 1976; 38: 2367-2377
        • Lee SP
        • Nicholson GI
        • Hitchcock G
        Familial abdominal chemodectomas with associated cutaneous angiolipomas.
        Pathology. 1977; 9: 173-177
        • Schmidt FE
        • Lewis Jr, ML
        • Kahle HR
        Chemodectomas of the carotid body and glomus tympanicum: sequential occurrence in sisters.
        JAM A. 1978; 240: 1266-1267
        • Bogdasarian RS
        • Lotz PR
        Multiple simultaneous paragan-gliomas of the head and neck in association with multiple retro-peritoneal pheochromocytomas.
        Otolaryngol Head Neck Surg. 1979; 87: 648-652
        • Rose KG
        • Jahnke K
        • Galanski M
        Die tumoren des Glomus caroticum und des Glomus vagale: Eine Darstellung von 24 einschlisslich familiar gehaufter und bilateraler Tumoren.
        HNO. 1979; 27: 192-200
        • Gnifferman S
        • Gillman MW
        • Pasternak LR
        • Peterson CL
        • Young Jr, WG
        Familial carotid body tumors: case report and epidemi-ologic review.
        Cancer. 1980; 46: 2116-2122
        • Veldman JE
        • Mulder PH
        • Ruijs SH
        • de Haas G
        • van Waes PF
        • Hoekstra A
        Early detection of asymptomatic hereditary chemodectoma with radionuclide scintiangiography: a possibility for family screening and surveillance.
        Arch Otolaryngol. 1980; 106: 547-552
        • Pereira DT
        • Hunter RD
        Familial mukicentric non-chromaffin paragangliomas: a case report on a patient with glomus jugulare and bilateral carotid body tumours.
        Clin Oncol. 1980; 6: 273-275
        • Verhulst J
        • Portmann M
        • Boissiéras P
        • Deminière C
        • Piton J
        Chémodectome bilateral, malin, familial, chez une jeune fille dc 16 ans.
        Rev Laryngol Otol Rhinol (Bord). 1980; 101: 251-261
        • Coia LR
        • Fazckas JT
        • Farb SN
        Familial chemodectoma.
        Int J Radiol Oncol Biol Phys. 1981; 7: 949-952
        • van Baars FM
        • Cremers CW
        • van den Broek P
        • Veldman JE
        Familiar non-chromaffinic paragangliomas (glomus tumors): clinical and genetic aspects (abridged).
        Acta Otolaryngol (Stockh). 1981; 91: 589-593
        • Parkin JL
        Familial multiple glomus tumors and pheochromocytomas.
        Ann Otol Rhinol Laryngol. 1981; 90: 60-63
        • Pritchett PN
        Familial concurrence of carotid body tumor and pheochromocytoma.
        Cancer. 1982; 49: 2578-2579
        • Parry DM
        • Li FP
        • Strong LC
        • et al.
        Carotid body tumors in humans: genetics and epidemiology.
        JNatl Cancer Inst. 1982; 68: 573-578
        • Larraza-Hernandez O
        • Albores-Saavedra J
        • Benavides G
        • Krause LG
        • Pcrez-Merizaldi JC
        • Ginzo A
        Multiple endocrine neopiasia: pituitary adenoma, multieentric papillary thyroid carcinoma, bilateral carotid body paraganglioma, parathyroid hyperplasia, gastric leiomyoma, and systemic amyloidosis.
        Am J Clin Pathol. 1982; 78: 527-532
        • Bleker RJ
        • Wereldsma JC
        Carotid body tumor: familial occurrence.
        Neth J Surg. 1986; 38: 76-80
        • Miccoli P
        • Lacconi P
        • Roncella M
        • Arganini M
        Tumeur du glomus carotidien: presentation d'un cas familial.
        Ada Chir Belg. 1986; 86: 159-162
        • Mikhail RA
        • Moore JS
        • Reed Jr, DN
        • Abbott RR
        Malignant retroperitoneal paragangliomas.
        J Surg Oncol. 1986; 32: 32-36
        • Khafagi F
        • Egerton-Vernon J
        • van Doom T
        • Foster W
        • McPhee IB
        • Allison RW
        Localization and treatment of familial malignant nonfunctional paraganglioma with iodine-131 MIBG: report of two cases.
        J Nucl Med. 1987; 28: 528-531
        • Santini J
        • Batt M
        • Dassonville O
        • et al.
        Paragangliomes de la tete et du cou: formes familiales.
        Ann Otolaryngol Chir Cervicofac. 1989; 106: 589-592
        • Sebastian JP
        • Williams SE
        • Wells M
        • Peake MD
        Familial malignant retroperitone al paraganglioma.
        Postgrad Med J. 1989; 65: 781-784
        • van der Mey AG
        • Maaswinkel-Mooy PD
        • Cornelisse O
        • Schmidt PH
        • van de Kamp UP
        Genomic imprinting in hereditary glomus tumours: evidence for new genetic theory.
        Lancet. 1989; 2: 1291-1294
        • Giron J
        • Joffre P
        • Senac JP
        • Castan P
        Paragangliome du vague associe a une malformation rare de la crosse aortique: a propos d'un cas et revue de la litterature.
        J Radiol. 1989; 70: 477-481
        • Barnes L
        • Taylor SR
        Carotid body paragangliomas: a clinicopathologic and DNA analysis of 13 tumors.
        Arch Otolaryngol Head Neck Surg. 1990; 116: 447-453
        • Granger JK
        • Houn HY
        Bilateral familial carotid body paragangliomas: report of a case with DNA flow cytometric and cytogenetic analyses.
        Arch Pathol Lab Med. 1990; 114: 1272-1275
        • Shedd DP
        • Arias JD
        • Glunk RP
        Familial occurrence of carotid body tumors.
        Head Neck. 1990; 12: 496-499
        • Sobol SM
        • Dailey JC
        Familial multiple cervical paragangliomas: report of a kindred and review of the literature.
        Otolaryngol Head Neck Surg. 1990; 102: 382-390
        • Le Bodie MF
        • Fiche M
        • Aillet G
        • et al.
        Immunohistochemical study of 6 multiple familial cervical paragangliomas with lymph node metastasi s in one case [in French].
        Ann Pathol. 1991; 11: 176-180
        • Tali ET
        • Sener RN
        • Ibis E
        • Alper H
        • Ariyurek M
        Familial bilateral glomus jugulare tumors.
        Neuroradiology. 1991; 33: 171-172
        • Jensen JC
        • Choyke PL
        • Rosenfeld M
        • et al.
        A report of familial carotid body tumors and multiple extra-adrenal pheochromocytomas.
        J Urol. 1991; 145: 1040-1042
        • Ophir D
        Familial multicen tric paragangliomas in a child.
        J Laryngol Otol. 1991; 105: 376-380
        • van Gils AP
        • van der Mey AG
        • Hoogma RP
        • et al.
        MRI screening of kindred at risk of developing paragangliomas: support for genomic imprinting in hereditary glomus tumours.
        Br J Cancer. 1992; 65: 903-907
        • Brandwein M
        • Levi G
        • Som P
        • Urken ML
        Paraganglioma of the inferior laryngeal paraganglia: a case report.
        Arch Otolaryngol Head Neck Surg. 1992; 118: 994-996
        • Borowy ZJ
        • Probst L
        • Deitel M
        • Shankar L
        A family exhibiting carotid body tumours.
        Can J Surg. 1992; 35: 546-551
        • Tali ET
        • Yuh WT
        • Mayr NA
        • Nguyen H
        • Gao F
        • Dolan KD
        Magnetic resonance imaging of bil ateral familial paragangliomas.
        Ann Otol Rhinol Laryngol. 1993; 102: 473-477
        • Ridge BA
        • Brewster DC
        • Darling RC
        • Cambria RP
        • LaMuraglia GM
        • Abbott WM
        Familial carotid body tumors: incidence and implications.
        Ann Vasc Surg. 1993; 7: 190-194
        • Mariman EC
        • van Beersum SE
        • Cremers CW
        • van Baars FM
        • Ropers HH
        Analysis of a second family with hereditary nonchromaffin paragangliomas locates the underlying gene at the proximal region of chromosome Ilq.
        Hum Genet. 1993; 91: 357-361
        • Mena J
        • Bowen JC
        • Hollier LH
        Metachronous bilateral nonfunctional intercarotid paraganglioma (carotid body tumor) and functional retroperitoneal paraganglioma: report of a case and review of the literature.
        Surgery. 1993; 114: 107-111
        • McCaffrey TV
        • Meyer FB
        • Michels VV
        • Piepgras DG
        • Marion MS
        Familial paragangliomas of the head and neck.
        Arch Otolaryngol Head Neck Surg. 1994; 120: 1211-1216
        • Hughes-Davies L
        • Mannarino E
        • Alexander III, E
        • Kooy H
        • Loeffler JS
        Technical modifications required to treat cervical chemodactomas with stereotactic radiosurgery.
        Surg Neurol. 1994; 41: 418-420
        • Murphy GF
        • Miller SD
        • Kenady DE
        • et al.
        Familial paraganglioma.
        Urology. 1994; 44: 273-277
        • Zaslav AL
        • Myssiorek D
        • Mucia C
        • Fox JE
        Cytogenetic analysis of tissues from patients with familial paragangliomas of the head and neck.
        Head Neck. 1995; 17: 102-107
        • Wang OO
        • Barros D'Sa AA
        • Johnston CF
        • Buchanan KD
        Oncogene expression in carotid body tumors.
        Cancer. 1996; 77: 2581-2587
        • Gardner P
        • Dalsing M
        • Weisberger E
        • Sawchuk A
        • Miyamoto R
        Carotid body tumors, inheritance, and a high incidence of associated cervical paragangliomas.
        Am J Surg. 1996; 172: 196-199
        • Wharton SM
        • Davis A
        Familial paraganglioma.
        J Laryngol Otol. 1996; 110: 688-690
        • Nakada J
        • Oishi Y
        • Onodera S
        • et al.
        A case of ectopic familial pheochromocytoma [in Japanese].
        Nippon Hinyokika Gakkai Zasshi. 1996; 87: 1060-1063
        • Linn HJ
        • Proctor B
        Tumor of the ganglion nodosum of the vagus nerve.
        Laryngoscope. 1956; 66: 1577-1581
        • Milanesi U
        • Mangili F
        • Milanesi I
        Studio citofluorimetrico diparagangliomi famil iari del corpo carotideo.
        Acta Otorhinolaryngol Ital. 1994; 14: 439-447
        • Struycken PM
        • Cremers CW
        • Mariman EC
        • Joosten FB
        • Bieker RJ
        Glomus tumours and genomic imprinting: influence of inheritance along the paternal or maternal line.
        Clin Otolaryngol. 1997; 22: 71-76
        • Gardner PA
        • Miyamoto RG
        • Shah MV
        • Righi PD
        • Timmerman RD
        Malignant familial glomus jugulare and contralateral carotid body tumor.
        Am J Oto/aryngol. 1997; 18: 269-273
        • Whiteman ML
        • Serafini AN
        • Telischi FF
        • Civantos FJ
        • Falcone S
        In octreotide scintigraphy in the evaluation of head and neck lesions.
        AJNR Am J Neuroradiol. 1997; 18: 1073-1080
        • Milunsky J
        • DeStefano AL
        • Huang XL
        • et al.
        Familial paragangliomas: linkage to chromosome 11q23 and clinical implications.
        Am J Med Genet. 1997; 72: 66-70
        • Baysal BE
        • Farr JE
        • Rubinstein WS
        • et al.
        Fine mapping of an imprinted gene for familial nonchromaffin paragangliomas, on chromosome Ilq23.
        Am J Hum Genet. 1997; 60: 121-132
        • Myssiorek D
        • Palestro CJ
        IlIlndium pentetreotide scan detection of familial paragangliomas.
        Laryngoscope. 1998; 108: 228-231
        • SkOldberg F
        • Grimelius L
        • Woodward ER
        • et al.
        A family with hereditary extra-adrenal paragangliomas without evidence for mutations in the von Hippel-Lindau disease or ret genes.
        Clin Endocrinol (Oxf). 1998; 48: 11-16
        • Blay L
        • Basterra J
        • Marco Algarra J
        • Palop JM
        • Pellin A
        Glomus carotideo bilateral y yugular unilateral familiar: estudio ultrastructural a prop6sito de dos casos clfnicos.
        An Otorrin olaringol Ibero Am. 1985; 12: 209-215
        • Yagi A
        • Takahashi H
        • Yao K
        • Sano H
        • Nitta K
        The two cases of carotid body tumor in the same family [in Japanese].
        Nippon Jibiinkoka Gakkai Kaiho. 1988; 91: 1987-1995
        • Geysens P
        • D'Haenens P
        • Van Steenberge R
        • Wilms G
        • Baert AL
        Familial occurrence of paragangliomas.
        J Beige Radiol. 1989; 72: 95-99
        • Heutink P
        • van Schothorst EM
        • van der Mey AG
        • et al.
        Further localization of the gene for hereditary paragangliomas and evidence for linkage in unrelated families.
        Eur J Hum Genet. 1994; 2: 148-158
        • van Schothorst EM
        • Jansen JC
        • Bardoel AF
        • et al.
        Confinement of PGL, an imprinted gene causing hereditary paragangliomas, to a 2-cM interval on II q22-q23 and exclusion of DRD2 and NCAM as candidate genes.
        Eur J Hum Genet. 1996; 4: 267-273
        • Cimino DA
        Paraganglioma timpano-giuglare bilaterale a carattere familiare.
        Va/salva. 1965; 41: 204-222
        • Marchesi M
        • Biffoni M
        • Tartaglia F
        • et al.
        I paragangliominella casistica dell' istituto di III Clinica Chirurgica (1970- 1995).
        G Chir. 1997; 18: 615-618
        • Marshall JB
        • Diaz-Arias AA
        • Bochna GS
        • Vogele KA
        Achalasia due to diffuse esophagea l leiomyomatosis and inherited as an autosomal dominant disorder: report of a family study.
        Gastroenterology. 1990; 98: 1358-1365
        • Nishida T
        • Hirota S
        • Taniguchi M
        • et al.
        Familial gastrointestinal stromal tumours with germline mutation of the KIT gene [letter].
        Nat Genet. 1998; 19: 323-324

      Linked Article

      • The Carney Triad: A Lesson in Observation, Creativity, and Perseverance
        Mayo Clinic ProceedingsVol. 74Issue 6
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          The triad described by Carney in this issue of Mayo Clinic Proceedings (pages 543 to 552) has special historical interest for me. Twenty-two years ago, the medical community was introduced by Dr Aidan Carney to a syndrome that included gastric sarcomas, pulmonary chondromas, and extra-adrenal paragangliomas that were likely to be functional and cause hypertension. All of these components tended to be multiple.1 This complex has subsequently been designated the Carney triad, which exists thanks to the amazing degree of perseverance by Aidan Carney.
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