Familial endocrine myxolentiginosis.

We present an unusual case of a left atrial myxoma as a feature of a familial mesoectodermal disorder and review the literature. The new term "familial endocrine myxolentiginosis" is proposed, which is descriptive of the major clinical components of the syndrome. Myriad features of this disorder include (1) cardiac myxomas; (2) cutaneous myxomas; (3) multiple lentigines or blue nevi, particularly of the head and neck; (4) bilateral primary pigmented nodular adrenocortical hyperplasia; (5) unusual testicular tumors; (6) pituitary tumors; (7) myxoid fibroadenomas of the breast; (8) myxomatous disorder of the stroma of the breast; (9) ductal adenoma of the breast; and (10) psammomatous melanotic schwannoma. A tentative diagnosis is suggested by identifying two features and a definitive diagnosis is made by three or more features. The clinical and pathologic features of cardiac myxoma in familial endocrine myxolentiginosis are identical to those of familial cardiac myxoma: age < 40 years, atypical locations, multicentric origins, and recurrent presentations. A Venn diagram classification for cardiac myxomas is proposed. We include photographic, echocardiographic, biopsy, and adrenal computerized tomography documentation in our patient. Recognition of this disorder is important because of its clinical, surgical, and genetic implications. The availability of transesophageal echocardiographic technology should allow early diagnosis of this underdiagnosed entity. Clinicians should consider this entity in the differential diagnosis of their patients with any one of these manifestations.

Epidermoid cyst of the spleen presenting as a generalized peritonitis.

In this report we reviewed 159 cases of epidermoid cyst of the spleen reported since 1929 and we added one case of our own with a unique clinical presentation. In these cases, the patients' age at presentation ranged from newborn to 51 years, with a mean age of 17.7 years. Female-to-male ratio was 2.0 to 1.0. Patients with this lesion usually present with asymptomatic abdominal mass and/or abdominal pain. Only in rare reports has there been infection (4 cases) or rupture (4 cases) of the cyst. In our case, the patient presented with an acute surgical abdomen and diffuse peritonitis. As in three of the previously reported cases associated with infection, Salmonella group organisms were cultured from the cyst abscess. Splenectomy is the surgical treatment of choice and the initial antibiotic regime should include Salmonella coverage.