1/6. A new observation of the Carney's triad with long follow-Up period and additional tumors.The etiology of the Carney's triad (gastrointestinal stromal tumors, pulmonary chondromas, and paragangliomas) is unknown, and only 57 cases have been reported since its identification in 1977. We report the clinical course of a female with the complete triad and some additional tumors. Bilateral vagal paragangliomas were treated surgically and with radiotherapy between the ages of 24 and 26 years. Subsequently she underwent surgery for a gastric leiomyosarcoma (27 years), a pleomorphic adenoma of the parotid gland (49 years) and a multifocal breast cancer with axillary spread (50 years). A calcified lesion was also noticed in the left lung, the radiologic diagnosis of which was consistent with chondroma. A mediastinal paraganglioma, detected at 56 years on a control X-ray of the chest, was partially excised at 63 years. At the last control, performed at 66 years, the patient was alive with residual cervical and mediastinal paraganglioma. Her younger brother was affected by Hirschsprung's disease and died at 54 years of rectal cancer. Her daughter is 33 and has been suffering since birth with severe constipation. In conclusion, this is one of the longest followed-up patients with Carney's triad. Her case illustrates the need for early recognition of the setting in order to detect the component tumors at a stage when surgery may be curative, and careful and life-long follow-up, both because the multicentricity of the classic components tends to manifest metachronously and because of the tendency to develop other tumors, some of which may be malignant. Furthermore, the presence of Hirschsprung's disease in the patient's family, coupled with the alleged common origin of two component lesions from derivatives of the neural crest, open new avenues for the understanding of this disorder.- - - - - - - - - - ranking = 1keywords = gastrointestinal stromal, stromal (Clic here for more details about this article) |
2/6. Typical and atypical Carney's triad presenting with malignant hypertension and papilledema.This report encourages clinicians to consider a diagnosis of Carney's triad in patients with multifocal gastric stromal sarcoma, extraadrenal paraganglioma (predominantly mediastinal), or pulmonary chondroma. The authors conducted a retrospective 20-year survey at the Hospital for Sick Children and identified two children with Carney's triad. One child, presenting atypically with papilledema and fundal hemorrhages from malignant hypertension and benign intracranial hypertension from chronic iron-deficiency anemia, is the second patient ever to date be described with the complete Carney's triad of neoplasms at diagnosis. Another child presented more typically with gastric stromal sarcoma and pulmonary chondroma without paraganglioma. Carney's triad is a rare differential diagnosis for "idiopathic" hypertension or iron-deficiency anemia from chronic gastrointestinal bleeding. If missed, patients with Carney's triad may have the debilitating physical and mental consequences of chronic iron deficiency and may die of untreated prolonged hypertension and metastatic leiomyosarcoma.- - - - - - - - - - ranking = 0.020120520475675keywords = stromal (Clic here for more details about this article) |
3/6. An unusual case of complete Carney's triad in a 14-year-old boy.Carney's triad represents the association of gastric gastrointestinal stromal tumor, pulmonary chondroma, and extraadrenal paraganglioma. Only 79 cases of this rare condition have been described. Here, the authors describe the unusual case of a 14-year-old boy who presented with a complete Carney's triad. This is only the second reported case in the world literature of a patient manifesting a complete Carney's triad at presentation. The management of each tumor is discussed.- - - - - - - - - - ranking = 1keywords = gastrointestinal stromal, stromal (Clic here for more details about this article) |
4/6. Atypical Carney's triad with coincidental clear cell renal carcinoma in an 84-year old patient-a finding at autopsy.Carney's triad is a syndrome of unknown etiology, representing a combination of gastrointestinal stromal tumors, bronchial chondromas and vagal, adrenal or paraadrenal paragangliomas. Two of the Carney's triad components-the paragangliomas and the gastrointestinal stromal tumors-are potentially lethal. Since its first description in 1977, 79 cases have been reported so far. We report an 84-year-old male patient, who died of a hypertensive cerebral hemorrhage. Well-differentiated clear cell carcinoma of the right kidney, chondroma of the right bronchus and multiple jejunal stromal tumors were detected at autopsy. To our knowledge, this is the first report of a coincidental clear cell renal carcinoma in a patient with an atypical Carney's triad.- - - - - - - - - - ranking = 2.0100602602378keywords = gastrointestinal stromal, stromal (Clic here for more details about this article) |
5/6. Endosonographic diagnosis of recurrent gastrointestinal stromal tumors associated with Carney's syndrome.This is a report of a patient who manifests all of the features of Carney's syndrome, including gastrointestinal stromal tumors (GISTs), extra-adrenal paragangliomas, and pulmonary chondromas. The patient underwent surgical resection of a gastric GIST; a retroperitoneal, nonfunctional paraganglioma; and a mediastinal, catecholamine-secreting paraganglioma (pheochromocytoma). Recently, new gastric GISTs were diagnosed by endoscopic, ultrasound-guided, fine-needle aspiration (EUS-FNA) biopsy and were resected. recurrence of stromal tumors following complete resection is common in Carney's syndrome and presents considerable management challenges. This case illustrates several important points: (1) Carney's syndrome, although rare, should be considered in patients with foregut GISTs; (2) GISTs associated with Carney's syndrome, like sporadic gastric GISTs, may have a more indolent clinical course; and (3) EUS-FNA may be useful for the diagnosis and management of GISTs in this syndrome.- - - - - - - - - - ranking = 5.0100602602378keywords = gastrointestinal stromal, stromal (Clic here for more details about this article) |
6/6. Dual CD117 expression in gastrointestinal stromal tumor (GIST) and paraganglioma of Carney triad: a case report.Carney triad is a rare syndrome, with only 20 complete cases reported. We report a 36-year-old white woman with complete Carney triad, including metastatic gastric stromal tumor (GIST), pulmonary chondroma, and nonfunctioning extra-adrenal paraganglioma. immunohistochemistry was positive for CD34 and CD117 (c-kit) in the GIST, and positive for chromogranin and CD117 in the paraganglioma. Ultrastructural studies demonstrated skeinoid fibers in the GIST. To our knowledge, this is the 21st complete Carney triad case reported and the first report of dual expression CD117 in both GIST and paraganglioma, a finding with intriguing pathogenetic implications related to the organization of the autonomic nervous system.- - - - - - - - - - ranking = 4.0100602602378keywords = gastrointestinal stromal, stromal (Clic here for more details about this article) |