(LAM) associated with both renal and hepatic angiomyolipomas (AMLs) in a TSC patient is very rare.
Perivascular epithelioid cell tumors (PEComas) of the kidney are a group of tumor family covering classical angiomyolipoma (AML), microscopic AML (microhamartoma), intraglomerular lesions, cystic AML, epithelioid AML, oncocytoma-like AML and lymphangiomyomatosis
of the renal sinus.
and angiomyolipoma: closely related entities characterized by hamartomatous proliferation of HMB-45-positive smooth muscle," Histopathology, vol.
Similar effects were noted in TSC patients with other types of benign tumors, including renal angiomyolipoma, fascial angiofibroma, lymphangiomyomatosis
, cardiac rhabdomyoma, and retinal astrocytic hamartoma .
(3) As noted by Davis et al, (2) a dominant feature in all cases of AMLEC is a prominent lymphatic channel network in the smooth muscle component forming slitlike branched and curvilinear spaces strongly resembling those seen in lymphangiomyomatosis
. This feature is also present, albeit focally, in most triphasic and myomatous or muscle-predominant AMLs.
Major features of TSC include cutaneous lesions such as adenoma sebaceum, more than three hypomelanotic macules, shagreen patch, and periungual fibromas; cortical tubers, subependymal nodules, retinal hamartomas, heart rhabdomyomas, renal angiomyolipomas, and lung lymphangiomyomatosis
. Minor features include bone cysts, confetti skin lesions, CNS white matter migration abnormalities, dental enamel pits, gingival fibromas, rectal polyps, multiple renal cysts, nonrenal hamartomas, and a retinal achromic patch.
- Cardiac involvement occurs during the intrauterine or neonatal period.- Rhabdomyomas tend to regress over time.- Epilepsy autism and developmental delays manifest themselves from infancy to adolescence.- Polycystic kidney disease usually is apparent in infancy or early childhood.- AMLs may develop at any time from childhood into adult life.- Lymphangiomyomatosis
typically presents in the third or fourth decade of life.
The four main causes of chylothorax include: malignancy; trauma; idiopathic; and miscellaneous causes such as thrombosis of the superior vena cava or subclavian vein, cirrhosis and rarely, pulmonary lymphangiomyomatosis
He had a history of right ureterolithotomy and diabetes, although no history of tuberous sclerosis, lymphangiomyomatosis
, renal cancer or renal cyst was present.
In comparison, lymphangiomyomatosis
(LAM) shows larger thin-walled cysts that are diffusely distributed in the lungs and eventually involve the entire lung parenchyma.
Mrs Priest, 61, of Colders Drive, suffers from a respiratory disease, pulmonary lymphangiomyomatosis
in the visualized lung bases.