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CARNEY'S TRIAD
Authored By: Brandon Peters and Henry Royal.
Patient: 34 year old female
History: 34 year old female found to have left femoral lesion during evaluation of gallstones.
Image Size:[small][as-submitted]

Fig. 1
MDP Bone scan

Fig. 2
Frontal radiograph of the left femur

Fig. 3
Frontal chest radiograph

Fig. 4
Lateral chest radiograph

Fig. 5
Contrast enhanced chest CT
Image Size:[small][as-submitted]

Findings: MDP Bone scan: Focal uptake corresponding to the mid left femoral lesion.  The uptake is most intense peripherally with slightly less uptake centrally.  Additionally, there is focally increased uptake in the left upper lobe corresponding to the soft tissue mass

Chest radiograph: Hyperdense lobulated soft tissue mass in the left upper lobe.

Chest CT scan:  Lobulated soft tissue mass with smooth margins containing interspersed fat and calcification.

Left femur radiograph:  Expansile lytic lesion centered in the mid femoral diaphysis.  No matrix or soft tissue component is demonstrated.   
DDx: Bone lesion:  Fibrous dysplasia, solitary bone cyst.
Diagnosis: Carney's triad

Bone lesion:  unknown.  Likely solitary bone cyst or fibrous dysplasia
General Discussion: 34 year old female with gallstones underwent ERCP which showed numerous submucosal gastric masses.  Also the patient was found to have a left adrenal mass. The patient underwent total gastrectomy and left adrenalectomy and the gastric masses were demonstrated to all be gastrointestinal stromal tumors and the left adrenal mass was demonstrated to be hyperplasia.  The mass in the left upper lobe is consistent with a chondroma. 

The lytic lesion in the left femur likely represents a solitary bone cyst or fibrous dysplasia.  Although an MRI was recommended for further evaluation, this has not occured to date.

In 1977 Carney described the concurrent appearance of 3 unusual tumors in 4 young patients: pulmonary chondroma, epitheliod leiomyosarcomas, and functioning paraganglioma. In those reports and those that have followed, the tumors of this triad occur at various times, often with long periods of time between their appearance in a given patient.  Thus, only two of the three tumor types are required for this diagnosis. Long term follow-up is needed if only two of the three components are present to search for the third component of the triad.  In fact, some suggest looking for the other two components if even one is found.  Although both the gastric leiomyosarcoma and the paraganglioma can be metatstatic, the leiomyosarcoma tends to have a more indolent coarse when seen as part of Carney's triad.  In both the original cases described by Carney and those that have followed, the majority of the patients are young females.  Symptoms related to the gastrointestinal stromal tumor, such as GI bleeding or abdominal mass, are common presenting symptoms.  In other cases, symptoms ascribed to the functioning paraganglioma are the presenting symptoms.
Specific Discussion: Despite an extensive search, no connection between Carney's triad and gallstones was found.  Also, no connection between the triad and fibrous dysplasia, simple bone cysts, or other benign bone lesions was found.  One report suggested the connection of Carney's triad and osseous chondrosarcoma (Reference #3).
References: 1. Carney, JA et al.  The triad of gastric leiomyosacroma, functioning, extra-adrenal paraganglioma, and pulmonary chondroma.  New England Journal of Medicine 1977; 296: 1517-18.

2.  DeJong, E. et al.  Carney's triad.  Eur J Surg Oncol. 1998 Apr(2): 147-9.

3.  Wahid ST et al.  A new variant of Carney's triad: Phaeochromocytoma and chondrosarcoma.  Postgrad Med J. 2001 Aug; 77(910): 527-8.
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Additional Details:

Case Number: 90178Owner(s): Brandon Peters and Henry RoyalLast Updated: 02-07-2013
Anatomy: Cardiopulmonary   Pathology: Benign Mass, Cyst
Modality: CT, Conventional Radiograph, Nuc MedAccess Level: Readable by all users, writable by NucMed Certifiers
Keywords: bsnm

Case has been viewed 70 times.
Certified by Henry Royal on 04-19-2007

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