Solitary superficial microcystic lymphatic malformation (lymphangioma circumscriptum) of the gingiva.

Solitary superficial microcystic lymphatic malformation (lymphangioma circumscriptum) of the gingiva.

October 2006

Kalpidis CD,
Lysitsa SN,
Kolokotronis AE,
Samson J,
Lombardi T.

Department of Periodontology and Implant Biology, School of Dentistry, Aristotle University, Thessaloniki, Greece. ckperio@otenet.gr

BACKGROUND:

Lymphatic malformations are uncommon, hamartomatous, developmental aberrations of the lymphatic system. The case presented in this report is a rare solitary gingival involvement of a microcystic-type lymphatic malformation.

METHODS:

The lesion presented clinically as a small vesicular swelling of a buccal interdental papilla in a 16-year-old girl. Involved tissues were excised and submitted for routine histologic examination. The expression of the endothelial marker CD34 was investigated using immunohistochemical staining.

RESULTS:

A physical examination failed to reveal similar or other abnormalities elsewhere in the body of the patient, including the oral cavity. Histopathologic analysis of the specimen demonstrated the presence of subepithelial, thin-walled, distended vascular cavities forming confluent vesicles containing lymph. The dilated lymphatic formations were lined by flattened CD34-negative endothelial cells. These features were consistent with a microcystic gingival lymphatic malformation. To the best of our knowledge, only two additional reports of this malformation have been published to date, but both presented with bilateral gingival involvement.

CONCLUSION:

Even though lymphatic malformations are encountered very infrequently on gingiva, they should be considered in the differential diagnosis of related conditions with a vesicular clinical appearance.

PMID: 17032125 [PubMed - in process]

Lymphangioleiomyomatosis.

Lymphangioleiomyomatosis.

Cancer Control. 2006 Oct;13(4):276-85

Taveira-DaSilva AM,
Steagall WK,
Moss J.

Pulmonary-Critical Care Medicine Branch, NHLBI, NIH, Bethesda, MD 20892, USA. dasilvaa@nhlbi.nih.gov

BACKGROUND:

Lymphangioleiomyomatosis (LAM) is a rare disease that is characterized by proliferation of abnormal smooth muscle-like cells (LAM cells), which leads to the formation of lung cysts, fluid-filled cystic structures in the axial lymphatics, and abdominal tumors. It primarily affects women.

METHODS:

The authors present a review of large series, registries, and protocols to highlight the prevalence, pathology, clinical features, diagnosis, and treatment options for patients with LAM.

RESULTS:

LAM commonly presents with progressive breathlessness or with recurrent pneumothorax, chylothorax, or sudden abdominal hemorrhage. Computed tomography (CT) scans show numerous thin-walled cysts throughout the lungs, abdominal angiomyolipomas, and lymphangioleiomyomas. Pulmonary function tests show decreased forced expiratory volume in 1 second (FEV1) and diffusion capacity for carbon monoxide (DLCO). Exercise testing shows gas-exchange abnormalities, ventilatory limitation, and hypoxemia that may occur with near-normal lung function.

CONCLUSIONS:

No effective treatment currently exists for this progressive disorder. However, recent progress in cancer and smooth muscle cell biology and a better understanding of the factors regulating angiogenesis and lymphangiogenesis may provide a foundation for the development of new therapeutic strategies.

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