Successful control of extensive thoracic lymphangiomatosis by irradiation

doi:10.1016/S0936-6555(97)80140-9

Clinical Oncology

Volume 9, Issue 6, 1997, Pages 407-411

https://doi.org/10.1016/S0936-6555(97)80140-9 Get rights and content

Abstract

Lymphangiomas are very rare benign tumours, believed to arise from congenital malformations of the lymphatic system. They grow very slowly, but are capable of massive expansion and infiltration of the surrounding tissues. If left untreated they can cause serious and sometimes fatal complications. The thorax is commonly affected, with involvement of the ribs, pleura, pericardium, lung parenchyma, mediastinum and vertebrae. Treatment depends on the extent of the disease. While local excision is the treatment of choice for localized lesions, radiotherapy is used for more extensive disease. In this report we describe three patients with extensive thoracic lymphangiomatosis who were treated successfully with irradiation alone. The value of early diagnosis and treatment is emphasized.

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Case reports have demonstrated variable success with surgical resection, local radiation, and sclerotherapy, but these approaches are limited to isolated disease only (Bermejo Casero et al., 2004; Satria et al., 2011; Kadakia et al., 2013). Interferon therapy has also been used with marginal success, but again, treatment is limited by significant toxicity and long-term side effects (Kandil et al., 1997; Kadakia et al., 2013). More recently, experimental treatment with systemic VEG-F inhibitors (propranolol, sirolimus, bevacizumab), which inhibit lymphangiogenesis, has been employed in a handful of cases with early reports of success (Ozeki et al., 2011; Reinglas et al., 2011; Aman et al., 2012).
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Charts were reviewed on 26 patients (16 male, 10 female) from January 1997 to July 2007 who underwent CO₂ laser resurfacing for symptomatic treatment of intraoral LM. A questionnaire was given in order to elucidate effectiveness in controlling symptoms and speed of postoperative recovery.
Mean age at time of first treatment was 9.2 years (median 6.8). Mean number of treatments was 3.0 (median 2.5). Average time between treatments was 9.7 months (median 5.6). Questionnaires were returned for 17 patients (65%). Common preoperative symptoms included swelling, bleeding, vesicle formation, and pain. All 17 patients reported symptomatic improvement after laser treatment. Five patients (29%) tolerated oral intake immediately, 10 (59%) the following day, and 1 (6%) was gastric tube dependent. Four patients (24%) returned to normal activity immediately after treatments, six (35%) by the following day, six (35%) within a few days, and one (6%) within a week. No postoperative complications were seen.
CO₂ laser resurfacing appears to be both safe and efficacious in treatment of symptoms related to intraoral LM. Intermittent treatments for recurrent symptoms is expected.
Effective initial treatment of diffuse pulmonary lymphangiomatosis with sirolimus and propranolol: A case report
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Original articleSuccessful control of extensive thoracic lymphangiomatosis by irradiation

Abstract

Ann Thorac Surg

J Pediatr Surg

Mayo Clin Proc

Ophthalmology

J Pediatr Surg

Int J Radiat Oncol Biol Phys

J Pediatr Surg

A unified concept of cystic hygroma

Surg Gynecol Obstet

Posterior mediastinal lymphangioma presenting with thoracic inlet compression

Br J Hosp Med

Successful control of lymphangioma circumscriptum by superficial X-rays

Br J Dermatol

Cervical, cervicomediastinal and intrathoracic lymphangioma [review]

Prog Pediatr Surg

Lymphangioma circumscriptum of the lid and conjunctiva

Br J Ophthalmol

Fractionated beta irradiation of a conjunctival lymphangioma

Ophthalmologica

Lymphangioma presenting as a primary parotid neoplasm in an adult. Report of a case with the diagnosis suggested by fine needle aspiration biopsy

Acta Cytol

Original article
Successful control of extensive thoracic lymphangiomatosis by irradiation