Carney Complex

Over a year ago I predicted that I had a pituitary tumor. No amount of heckling, belittling or guffaws could get me to back down from my theory. I work in an industry where I engineer automatic building systems that work on negative feedback. On a superficial level, endocrine systems work the same way. If I had an entire building system fail, lights - air handlers - boiler - evaporative cooler - et al, I certainly wouldn't go around looking at each individual system endlessly; scratching my head like an idiot. The main controller is the first place to start, not the last place to check. That is precisely how it only made sense that either my pituitary or hypothalamus were at fault.

As sure as I was about that, is as sure as I am that this goes further than my pituitary tumors. My main suspect is Carney Complex.

57% female and 57% of the deaths are due to heart related causes. Nice little 'rhyme' there.

Quick read:

Spotty skin pigmentation lesions, such as lentigines (small, brown to black, non or slightly elevated, round or irregular) and blue nevi (large, blue to black, domed lesion) observed primarily in the face, eyelids, ears, and borders of the lips are the most common clinical manifestation of CNC (77%). Lentigines tend to fade with the age, usually after the fourth decade of life.

Myxomas are frequent lesions in CNC patients; heart myxomas (53%) occur multicentrically, and in any, or all, cardiac chambers; skin myxomas (33%) are detected in the eyelid, the external ear canal, the nipple, the oropharynx, the female genital tract and the female pelvis. Breast myxomas are often bilateral and present in more than 70% of adult women with CNC.

Psammomatous melanotic shwannomas , very rare tumors (10%), may occur anywhere in the peripheral nervous system, but most frequently in the gastrointestinal tract and paraspinal symphatetic chain.

Breast ductal adenomas, unusual mammary tumors akin to intraductal papillomas have been detected in 3% of CNC cases.

Endocrine lesions in CNC include testicular neoplasms (33%), primary pigmented nodule adrenocortical disease (PPNAD) (26%), growth hormone (GH) and prolactin-producing pituitary tumors (14%) and thyroid cancer (5%).

Long Read:

Pituitary involvement with Carney's syndrome was found in 11% of patients.

Adrenal glands:
PPNAD pigmented adrenocortical nodules ranging in size from submicroscopic to 10 mm in diameter, The adrenal glands feature multiple black and brown cortical nodules that contain large cells with pigment-laden, eosinophilic cytoplasm in the presence of internodular cortical atrophy. Furthermore, PPNAD is characterized by undetectable or low levels of ACTH.

The most common presenting signs or symptoms of Cushing's syndrome with PPNAD were approximately the same as those associated with Cushing's syndrome and included central obesity, weight gain, hirsutism, and hypertension in addition to other manifestations.

Computed tomography (CT) scanning of the adrenals was performed in 33 cases in the above study. The adrenals appeared normal in 45% and bilaterally enlarged in 27%. These data show that adrenal gland imaging of Carney's complex can be indistinguishable from that of other adrenal condition characterized by adrenal nodularity, which is frequently present in other primary forms of the Cushing syndrome and in normal elderly persons.

Heart:
Familial atrial myxomas, in contrast to non-familial myxomas, occur earlier in life, typically presenting in the second and third decades (average age 24 years).

Breasts:
In one study, breast myxoid fibroadenomas or other myxoid lesions were reported in 20% (19 patients) of females with Carney's complex, usually presenting as one or more discrete, asymptomatic mammary masses. Tumors were bilateral in slightly less than 10% of subjects (8 female patients). The breast lesions associated with Carney's Complex ranged in size from 2 mm to 2 cm in diameter. Mammary ductal adenoma was also found to occur in four patients.

Schwannomas:
Carney's complex involves tumors that affect peripheral nerves as well. In 1934 Bjorneboe first described these tumors which later became know as psammomatous melanotic schwannomas (Bjorneboe, 1934). The lesion was found to occur equally between sexes (Killeen et al., 1988). This particular tumor has malignant potential and four patients died as a result of metastasis.

I have tons lentigines in all the wrong places, cutaneous myxomas, undiagnosed adrenal-something on the MRI but normal CT reading, 90% of the time my ATCH is undetectable, pituitary tumor(s), large ovarian cyst, a breast lump-something that was diagnosed when I was 15 that hasn't changed in size [I believe they said fibrous? I cannot remember], and I really do need to see a cardiologist. I don't buy the two abnormal stress ECHOs and a Cardiac X diagnosis with all of these other problems staring at me. I think I need a 2nd opinion, ummn ok I have to have one before surgery anyhow or my specialist will kick my a**. I'll call tomorrow, I promise.