Lipoblastoma with unique localization requiring trachealand esophageal resectionMichele Torre a,⁎, Cecilia Borel b, Francisco Saitua c, Francisco Ossandon c,Juan Jose Latorre d, Patricio Varela c
aPediatric Surgery, Istituto Gaslini, Genova 16148, ItalybENT Unit, Calvo Mackenna Hospital, Santiago 7500539, ChilecPediatric Surgery, Calvo Mackenna Hospital, Santiago 7500539, ChiledPathology Unit, Calvo Mackenna Hospital, Santiago 7500539, Chile
Received 24 March 2010; revised 17 June 2010; accepted 19 June 2010
Lipoblastoma is a rare tumor of embryonic fat, mainlyoccurring in infants. Lipoblastomatosis has been defined asthe diffuse multifocal type of lipoblastoma [1]. Even ifbiologically benign, these neoplasms, in particular lipoblas-tomatosis, can be difficult to resect completely because oftheir undefined margins; and relapses are therefore frequent.In the literature, there are few published case series oflipoblastoma/lipoblastomatosis [2-4]. Neck localization isnot uncommon; and in around a third of cases, it was foundto cause respiratory symptoms owing to external compres-sion and dislocation of the upper airway [5]. We report on aninfant in whom the tumor infiltrated both tracheal andesophageal walls, which required a challenging surgicalprocedure. To our knowledge, this is the first case oflipoblastoma localized in upper airway and esophagus.
A 7-month-old baby girl with no significant clinicalhistory was admitted to our emergency department forsymptoms mimicking acute laryngitis: respiratory distress,polypnea, prolonged expiration time, and dysphonic voice.Despite treatment with adrenalin, bronchodilators, andantibiotics, the symptoms persisted; so the baby underwentnoninvasive ventilation and, after few days, was intubated.Airway endoscopy was performed and showed only a smalltracheal granuloma. Cervical ultrasound detected a cervicallobulated mass; and computed tomography confirmed thepresence of a mass of 3.9 × 2.7 × 2.3 cm, determiningextrinsic compression of the airway and dislocation of theneurovascular bundle of the neck. Results of blood and urineanalysis for neuroblastoma and germinal cell tumors werenegative. Surgical resection was decided on, and a lateralright cervical approach was undertaken. The mass appearedlobulated, blankish, and firm, in close contact with trachea,
Fig. 1 Tracheal resection including 5 tracheal rings with expositionof the anterior wall of the esophagus, infiltrated by lipoblastoma.
Fig. 2 Infiltration of the tracheal wall by lipoblastoma.
Fig. 3 Intraluminal lipoblastoma localization at endoscopicevaluation.
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esophagus, and carotid artery. The impression of the surgeonwas that the tumor originated from the tracheal wall, wherehe could not find a clear cleavage. The tumor was thendissected from the esophagus and from the carotid artery.The resection appeared macroscopically complete. Histologyconcluded a benign lipoblastoma. The baby remainedintubated for 3 days and then was extubated afterlaryngotracheoscopy, which showed disappearance of thetracheal granuloma. After 1 month without symptoms, thepatient presented stridor; and the upper airway wasreevaluated endoscopically. A rounded intraluminal massoriginating from the posterior wall of the cervical tracheawas occluding 85% to 90% of the tracheal lumen. Few daysafter, the patient was intubated for respiratory distress.Angiomagnetic resonance showed a tumor with lipomatousfeatures located at the posterolateral wall of the trachea, withan estimated longitudinal and transverse extension of 2 cmand 7 mm, respectively. Contrast enhancement of the masswas poor. Surgical approach included a cervical incision anda wide opening of the airway to completely expose the tumor
(Fig. 1). Tracheal resection extended from the inferiormargin of the cricoid to the fifth tracheal ring, removingmacroscopically all the tumor. The esophagus was exposedanteriorly, and the tumor appeared to be infiltrating theanterior wall. Resection of the anterior esophageal wall withsuture of the lateral esophageal walls on the midline wasperformed. Airway continuity was reestablished by crico-tracheal anastomosis. A flap of sternocleidomastoid musclewas interposed between esophageal and tracheal planes.Laparoscopic gastrostomy was performed at the same time.Histopathologic examination revealed a lipoblastoma infil-trating both esophageal and tracheal walls, with reactiveadenopathies. Microscopic examination showed airwayinfiltration extending from the cricoid to the third trachealring (Fig. 2). Laryngotracheoscopies performed at 1 and 3months after surgery showed grade 1 subglottic stenosis inthe absence of respiratory symptoms. However, 1 monthafter surgery, esophageal endoscopy showed a small roundedmass that was excised endoscopically and resulted to be arelapse of the lipoblastoma (Fig. 3). The esophagoscopy (3months from surgery) showed no evidence of localizedtumor, but multiple biopsies detected lipoblastomatosisinfiltration of the esophageal wall in the proximal andmiddle esophagus. Nine months after surgery, the patientremains asymptomatic; she can eat orally, and the gastro-stomy is closed. She is now under strict clinical follow-upwhile waiting for future endoscopic evaluations.
2. Discussion
Lipoblastoma and lipoblastomatosis are both benigntumors of infancy arising from embryonic fat. The differencebetween them is that the former is unifocal in origin and thelatter is multifocal [1]. No malignant transformation has everbeen reported; but relapse is quite common, especially inlipoblastomatosis [4]. These tumors are rare; but numerous
case reports have been published, and a comprehensivereview of the literature with a large series from a singleinstitution was published in 2008 [4]. Localization inextremities, neck, trunk, scrotum, retroperitoneum, medias-tinum, and omentum has been observed. Neck lipoblastomaand lipoblastomatosis are frequently asymptomatic, butrespiratory compromise has been reported in around 30% ofcases [5,6] owing to airway dislocation and compression bythe tumor. Horner syndrome [6,7] or hemiparesis [3,7] mayalso occur. Lung lipoblastoma is unique [8,9], whereas to ourknowledge, lipoblastoma localized in the upper airway andesophagus has never been reported. Our patient was initiallyadmitted for respiratory distress owing to the compression ofthe airway by the neck tumor. At this time, the laryngo-tracheal endoscopy did not show any intraluminal lesion.However, at surgery, the tumor appeared firmly attached tothe tracheal wall. Unfortunately, frozen sections were nottaken; and we therefore lacked histologic evaluation of thetracheal wall, which however appeared to be macroscopicallyinfiltrated. Although tracheal resection at initial surgery couldhave probably avoided local relapse, it seemed too aggressiveto the pediatric surgeon who performed this operation, whoenvisaged the possibility of a benign lipomatous nature of themass. Later on, respiratory symptoms became evident owingto airway intraluminal obstruction. Although lipoblastoma-tosis may have amultifocal origin, there are no descriptions oflipoblastoma/lipoblastomatosis with multiorgan involve-ment. Moreover, intraluminal growth of lipoblastoma hasnot been described so far. Therefore, our case is unique for theinvolvement of multiple organs and for the intraluminalinvolvement of both tracheal and esophageal walls.
Chromosomal abnormalities have been reported inlipomatous tumors, and molecular analysis can be used asa complementary diagnostic tool [2]. In our case, we did notperform any genetic study because the histopathologicevaluation alone was definitive.
Histologic features of lipoblastoma are well defined,consisting of a lobulated tumor of fat tissue with connectivetissue septae. Fat cells can show different degrees ofdifferentiation, and a myxoid background is quite typical.In most cases, a capillary network is permanent. Liposar-coma and especially its myxoid variant can share similarfeatures with lipoblastoma, but age at onset and the absenceof hyperchromasia and nuclear atypia in lipoblastoma arehelpful in making a differential diagnosis.
Although lipoblastoma and lipoblastomatosis are benigntumors, wide resection is recommended to avoid recurrence[10]. There are sporadic reports of spontaneous maturationof lipoblastoma to lipoma [11,12], which raise doubts aboutthe necessity of an aggressive surgical treatment. Asprognosis is generally good, nonmutilating surgery isadvocated [4]. In nonresectable tumors, a staged approachproved to be beneficial [4]. In our case, the tumor relapsed
with a surprisingly rapid growth, with relapses occurringtwice, each at about 1-month interval from previous surgery.This finding is not in agreement with reported data because, inthe literature, recurrences were observed between 4 monthsand 10 years, with an average time to recurrence of 3 years[4]. However, at 9 months from the second surgery, ourpatient is still asymptomatic; so the clinical importance ofesophageal wall infiltration by lipoblastomatosis, as well asthe possible need of future treatment (esophageal replace-ment), remains to be defined.
In conclusion, we report on the first case of lipoblasto-matosis localized in both tracheal and esophageal walls.After failure of first resection, a challenging surgicalapproach, including extended tracheal and esophagealresection, was performed. The primary site of the tumorremains indeterminate. Esophageal lipoblastomatosis wasstill present at the last endoscopic evaluation, but its clinicalimplication remains unknown.
Acknowledgment
The authors acknowledge Anna Capurro for helping inreviewing the manuscript.
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