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Ann Thorac Surg 2009;88:1695-1697. doi:10.1016/j.athoracsur.2009.03.033
© 2009 The Society of Thoracic Surgeons
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Case Reports

Lipoblastoma: A Rare Mediastinal Tumor

Alsmady Moaath, MDa,*, Ennab Raed, MDa, Rashadan Mohammad, MDa, Saleem Mohammad, MDb

a Department of Cardiothoracic Surgery, University of Jordan, Amman, Jordan
b Department of Pediatric Surgery, University of Jordan, Amman, Jordan

Accepted for publication March 11, 2009.

* Address correspondence to Dr Alsmady, Department of Cardiothoracic Surgery, University of Jordan, PO Box 2086, Aljbiha, Amman, 11941, Jordan (Email: moaath5{at}yahoo.com).


   Abstract
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Lipoblastoma is a rare benign mesenchymal tumor of embryonal fat that occurs almost exclusively in infants and children less than age 3. It is a benign tumor with a high recurrence rate. We present a case of mediastinal lipoblastoma in a 21-month-old girl who presented with respiratory infection and progressive dyspnea. Complete surgical excision of the mass was achieved through a lateral thoracotomy. Her postoperative course was uneventful and the histopathologic examination proved that the mass was a lipoblastoma. We emphasize that this rare mediastinal tumor should be included in the differential diagnosis of infants having a mediastinal mass.


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Lipoblastoma and lipoblastomatosis are very rare benign mesenchymal tumors most often found in children less than 3 years old. They may present as a localized, well-circumscribed lesion, the so-called lipoblastoma, or as a multicentric, deep, and infiltrative type, which is lipoblastomatosis.

They may arise almost anywhere within the soft tissues, with the trunk and the extremities being the most common predilection sites; however, the neck and mediastinum are uncommon locations for these tumors [1].

Mediastinal lipoblastoma can be presented with progressive dyspnea, as in our case, or with neurologic symptoms resulting from spinal cord compression [2]. It is important to completely excise the tumor to avoid leaving the residual tumor and to prevent recurrences that mostly occur within 2 years.

A 20-month-old girl presented with respiratory infection and progressive dyspnea. A chest roentgenogram showed that she had a right-sided mass involving the upper zone of the thorax, causing displacement of the trachea to the left side (Fig 1A).


Figure 1
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  		Fig 1. (A) Chest roentgenogram showing the mass within the right hemi-thorax. (B) Computed tomographic scan showing the mediastinal tumor.

 
The patient was treated for pneumonia that resolved a few days later with intravenous antibiotics. A computed tomographic chest scan showed a large fat-containing lesion with some septation in the mediastinum and the right side of the chest, and engulfing some of the main vessels, especially the branches of the aortic arch (Fig 1B).

A lateral thoracotomy was done. Intraoperatively, the mass was found to be engulfing the branches of the aortic arch, but with a clear plane of dissection. A complete surgical excision was achieved without sacrificing any vessels. The postoperative course of the patient was uneventful.

The histopathologic examination revealed adipose tissue composed of mature and immature fat cells in myxoid stroma (Fig 2).


Figure 2
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  		Fig 2. Histologic view showing mature and immature adipose tissue. (Hematoxylin & eosin stain, x600).

 

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Lipoblastoma is a rare tumor of infancy and early childhood. It was first described by Jaffe [3] in 1926; since then many cases were reported. In 1973, Chung and Enzinger [4] suggested the term benign lipoblastoma for the circumscribed type, and the term benign lipoblastomatosis for the diffuse multicentric type of this neoplasm.

Although lipoblastomatous tumors are benign, they may grow very fast, attain large sizes, and become a painless mass, making it the most common symptom. Tumors located at the mediastinum may present with stridulous respiration, and may also present with neurologic symptoms resulting from spinal cord compression like paraparesis and hemiparesis [2, 5].

Although a computed tomographic scan may show a tumor of fatty origin, preoperative differential diagnosis between other fatty tumors, such as lipoma, liposarcoma, and myxoliposarcoma are quite difficult [6]. However, lipoblastomatous tumors differ from lipoma or lipomatosis by their cellular immaturity histologically, and they have a close resemblance to low-grade liposarcomas. On the contrary, liposarcomas are tumors that occur in adults and are extremely rare in infants. Histologic differential diagnosis between lipoblastomatous tumors and liposarcomas is based on the prominent lobular architecture of lipoblastomatous tumors in addition to the lack of nuclear atypia, unlike the myxoid liposarcomas. In addition, myxoid liposarcomas show a characteristic t(12; 16) translocation, which is not present in lipoblastomatous tumors, and recent studies have defined rearrangements of chromosome 8q11-q13 observed as deletions in lipoblastomatous tumors to distinguish from myxoid liposarcomas [7].

Complete surgical resection of lipoblastomas yields an excellent prognosis. Lipoblastomas are benign tumors, and no malignant degeneration has been documented. Recurrence has been reported in 14% to 25% of cases, usually due to incomplete resection or diffuse disease. Lipoblastomatosis exhibits higher recurrence rates partly due to its propensity to localize within deep soft tissues, which makes complete excision more difficult to achieve [8].

In conclusion, mediastinum is an uncommon site of lipoblastoma, but it should be considered in the differential diagnosis of a rapidly growing soft fatty mass in children. Surgical excision is the best therapeutic option and gives excellent results. However, high recurrence rate demands follow-up.


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  1. Lorenzen JC, Godballe C, Kerndrup GB. Lipoblastoma of the neck: a rare cause of respiratory problems in children Ausis Nasus Larynx 2005;32:169-173.
  2. Dogan R, Kara M, Firat P, Gedikoglu G. An unusual tumor of the neck and mediastinum: lipoblastomatosis resulting in paraparesis Eur J Cardio-thorac Surg 2007;31:325-327.[Abstract/Free Full Text]
  3. Jaffe RH. Recurrent lipomatous tumors of the groin Arch Pathol Lab Med 1926;1:381-387.
  4. Chung EB, Enzinger FM. Benign lipoblastomatosis. An analysis of 35 cases. Cancer 1973;32:482-492.[Medline]
  5. Sun JJ, Rasgon BM, Hilsinger RL. Lipoblastomatosis of the neck causing hemi paresis: a case report and review of the literature Head Neck 2003;25:337-340.[Medline]
  6. Stringel G, Shandling B, Mancer K, Ein SH. Lipoblastoma in infants and children J Pediatr Surg 1982;17:277.[Medline]
  7. Harrer J, Hammon G, Wagner T, Bolkenius M. Lipoblastoma and lipoblastomatosis: a report of two cases and review of the literature Eur J Pediatr Surg 2001;11:342-349.[Medline]
  8. Dilley AV, Patel DL, Hicks MJ, Brandt ML. Lipoblastoma: pathophysiology and surgical management J Pediatr Surg 2001;36:229-231.[Medline]



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