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The use of sirolimus in the treatment of giant cystic lymphangioma: Four case reports and update of medical therapy.

Abstract Lymphatic malformations (LMs) are rare and benign anomalies resulting from the defective embryological development of the primordial lymphatic structures. Due to their permeative growth throughout all tissue layers, treatment is often challenging. Small asymptomatic lesions can be conservatively managed, while symptomatic lesions require active management. Surgery has been historically considered the treatment of choice, but today less invasive therapeutic options are preferred (sclerotherapy, laser therapy, oral medications). However, there are not uniform therapeutic protocols. Sirolimus is an oral medication that has been reported to be effective in the recent literature. Here we present the case of 4 newborns with giant multicystic lymphangioma treated with oral sirolimus after surgical resection had failed.
PMID
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Authors

Mayor MeshTerms
Keywords
Journal Title medicine
Publication Year Start




PMID- 29390423
OWN - NLM
STAT- MEDLINE
DCOM- 20180213
LR  - 20180213
IS  - 1536-5964 (Electronic)
IS  - 0025-7974 (Linking)
VI  - 96
IP  - 51
DP  - 2017 Dec
TI  - The use of sirolimus in the treatment of giant cystic lymphangioma: Four case
      reports and update of medical therapy.
PG  - e8871
LID - 10.1097/MD.0000000000008871 [doi]
AB  - RATIONALE: Lymphatic malformations (LMs) are rare and benign anomalies resulting 
      from the defective embryological development of the primordial lymphatic
      structures. Due to their permeative growth throughout all tissue layers,
      treatment is often challenging. Small asymptomatic lesions can be conservatively 
      managed, while symptomatic lesions require active management. Surgery has been
      historically considered the treatment of choice, but today less invasive
      therapeutic options are preferred (sclerotherapy, laser therapy, oral
      medications). However, there are not uniform therapeutic protocols. Sirolimus is 
      an oral medication that has been reported to be effective in the recent
      literature. Here we present the case of 4 newborns with giant multicystic
      lymphangioma treated with oral sirolimus after surgical resection had failed.
      PATIENT CONCERNS: At birth the LMs were clinically appreciated as giant masses
      involving different organs and structures. DIAGNOSES: All patients had a prenatal
      diagnosis of giant multicystic lymphangioma confirmed at histological and
      cytological analysis. INTERVENTIONS: Patients were treated with oral sirolimus
      after unsuccessful surgical resection. OUTCOMES: In all patients, sirolimus
      determined an overall reduction of the mass and a global involution from the
      macro- to the microcystic composition. Sirolimus was safe and poor disadvantages 
      had been observed. The main and isolated adverse effect at laboratory analysis
      was progressive dyslipidemia, with increasing levels of total cholesterol and
      triglycerides. LESSONS: To date, our experience with sirolimus in the management 
      of LMs is favorable. We recommend the use of sirolimus after unsuccessful
      surgical excision have been tried or when the surgical approach is not feasible. 
      A multidisciplinary follow-up is needed to monitor disease evolution.
CI  - Copyright (c) 2017 The Authors. Published by Wolters Kluwer Health, Inc. All
      rights reserved.
FAU - Amodeo, Ilaria
AU  - Amodeo I
AD  - Neonatal Intensive Care Unit.
FAU - Colnaghi, Mariarosa
AU  - Colnaghi M
AD  - Neonatal Intensive Care Unit.
FAU - Raffaeli, Genny
AU  - Raffaeli G
AD  - Neonatal Intensive Care Unit.
FAU - Cavallaro, Giacomo
AU  - Cavallaro G
AD  - Neonatal Intensive Care Unit.
FAU - Ciralli, Fabrizio
AU  - Ciralli F
AD  - Neonatal Intensive Care Unit.
FAU - Gangi, Silvana
AU  - Gangi S
AD  - Neonatal Intensive Care Unit.
FAU - Leva, Ernesto
AU  - Leva E
AD  - Department of Pediatric Surgery.
FAU - Pignataro, Lorenzo
AU  - Pignataro L
AD  - Department of Otolaryngology.
FAU - Borzani, Irene
AU  - Borzani I
AD  - Pediatric Radiology Unit, Fondazione IRCCS Ca Granda Ospedale Maggiore
      Policlinico, Universita degli Studi di Milano, Milan, Italy.
FAU - Pugni, Lorenza
AU  - Pugni L
AD  - Neonatal Intensive Care Unit.
FAU - Mosca, Fabio
AU  - Mosca F
AD  - Neonatal Intensive Care Unit.
LA  - eng
PT  - Case Reports
PT  - Journal Article
PL  - United States
TA  - Medicine (Baltimore)
JT  - Medicine
JID - 2985248R
RN  - 0 (Antibiotics, Antineoplastic)
RN  - W36ZG6FT64 (Sirolimus)
SB  - AIM
SB  - IM
MH  - Administration, Oral
MH  - Antibiotics, Antineoplastic/administration & dosage/*therapeutic use
MH  - Diagnosis, Differential
MH  - Female
MH  - Humans
MH  - Infant, Newborn
MH  - Lymphangioma, Cystic/*diagnosis/diagnostic imaging/drug therapy
MH  - Magnetic Resonance Imaging
MH  - Male
MH  - Sirolimus/administration & dosage/*therapeutic use
PMC - PMC5758125
EDAT- 2018/02/03 06:00
MHDA- 2018/02/14 06:00
CRDT- 2018/02/03 06:00
PHST- 2018/02/03 06:00 [entrez]
PHST- 2018/02/03 06:00 [pubmed]
PHST- 2018/02/14 06:00 [medline]
AID - 10.1097/MD.0000000000008871 [doi]
AID - 00005792-201712220-00010 [pii]
PST - ppublish
SO  - Medicine (Baltimore). 2017 Dec;96(51):e8871. doi: 10.1097/MD.0000000000008871.