Approach Considerations
Subcutaneous lipomas associated with multiple endocrine neoplasia type 1 (MEN1) may be surgically removed for cosmetic reasons; they typically do not recur. Facial angiofibromas and collagenomas usually do not require treatment. [31]
Surgical Care
Patients with MEN1 may desire to have their cutaneous tumors removed because of cosmetic concerns, particularly with the larger facial angiofibromas. A variety of surgical methods have been used to treat angiofibromas in patients with tuberous sclerosis, and these treatments are likely applicable to angiofibromas in patients with MEN1. These methods include the following [32, 33] :
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Shave excision
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Dermabrasion
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Carbon dioxide laser
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Combined pulsed dye laser and fractional resurfacing
Cosmetic improvement of facial angiofibromas in patients with MEN1 has been obtained with shave excision. In some cases, however, lesions treated in this way have slowly reappeared.
Collagenomas and lipomas can be excised, and lipomas can also be treated by liposuction.
Consultations
In view of the wide spectrum of clinical manifestations of MEN1 and the complexities in its management, Endocrine Society guidelines recommend that patients with MEN1 and their families be cared for by a multidisciplinary team comprising relevant specialists with experience in the diagnosis and treatment of endocrine tumors. The guidelines also recommend that the overall care of these patients and their families be coordinated by an endocrinologist with expertise in MEN syndromes. [1]
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A 27-year-old man has telangiectatic, red papules on the nose, the nasolabial fold, and the upper lip. Histologic examination of one of these lesions confirmed the clinical diagnosis of angiofibroma. In addition to multiple facial angiofibromas, this patient has multiple collagenomas and gingival papules, as well as hyperparathyroidism and a positive family history for multiple endocrine neoplasia type 1.
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The shoulder of a 65-year-old man shows multiple firm, skin-colored to slightly hypopigmented papules. Biopsy results of the largest lesion revealed collagenoma. Endocrinologic features of multiple endocrine neoplasia type 1 in this patient are hyperparathyroidism and Zollinger-Ellison syndrome. Note that the photograph was taken with side lighting to accentuate the lesions.
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A close-up view of a large collagenoma on the shoulder of a 65-year-old man shows multiple firm, skin-colored to slightly hypopigmented papules. Endocrinologic features of multiple endocrine neoplasia type 1 in this patient are hyperparathyroidism and Zollinger-Ellison syndrome.
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A 39-year-old woman with multiple endocrine neoplasia type 1 has a soft nodule on the forehead that is consistent with lipoma. Lipomas in patients with multiple endocrine neoplasia type 1 can be single or multiple, and they are sometimes large.
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On the attached gingiva of a 27-year-old man with multiple endocrine neoplasia type 1, a few small, whitish papules are present. Gingival papules are a rare and subtle finding in multiple endocrine neoplasia type 1. In addition to multiple facial angiofibromas, this patient has multiple collagenomas, hyperparathyroidism, and a positive family history for multiple endocrine neoplasia type 1.
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Light microscopic evaluation of a section of an angiofibroma shows prominent vessels and concentric rings of collagen around vessels and adnexal structures (hematoxylin and eosin, original magnification X100). These findings are indistinguishable from those observed in angiofibromas in patients with tuberous sclerosis.
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Histologic examination of a collagenoma reveals dense, thick collagen in the reticular dermis (hematoxylin and eosin, original magnification X40). An elastic stain showed reduced elastic fibers (not shown). Biopsy samples of collagenomas can be mistaken for healthy skin unless an elliptical excision containing surrounding healthy skin is obtained for comparison. The contrast with healthy skin accentuates the thickened dermis and collagen alterations seen in collagenomas.
