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CAS CLINIQUE / CASE REPORT

LARGE THYMOLIPOMA CAUSING PRIMARILY RESPIRATORY DISTRESS


http://www.lebanesemedicaljournal.org/articles/60-2/case2.pdf

Moussa ABI GHANEM , Georges HABAMBO , Joudy BAHOUS , Camil CHOUAIRY , Bassam ABU KHALIL
1 1 2 3 1

Abi Ghanem M, Habambo G, Bahous J, Chouairy C, Abu Abi Ghanem M, Habambo G, Bahous J, Chouairy C, Abu
Khalil B. Large thymolipoma causing primarily respiratory Khalil B. Large thymolipome entraînant une détresse respira-
distress. J Med Liban 2012 ; 60 (2) : 106-109. toire sévère. J Med Liban 2012 ; 60 (2) : 106-109.

ABSTRACT : Thymolipoma, is an anterior mediastinal RÉSUMÉ : Le thymolipome est une tumeur bénigne
tumor that is benign in origin. It is often asymptomatic, du médiastin antérieur. Il est souvent asymptomatique,
but can enlarge significantly prior to diagnosis. It con- mais peut devenir assez énorme avant d’être diagnos-
sists of thymic and fatty tissue and is surrounded by a tiqué. Il est constitué de tissus thymique et adipeux en-
capsule. tourés par une capsule.
Less than a hundred cases have been reported world- Moins de cent cas ont été décrits dans la littérature.
wide. Although rare, it should be considered in the dif- Bien que rare, il doit être considéré dans le diagnostic
ferential diagnosis of mediastinal tumors. différentiel des tumeurs médiastinales.
In this paper, we report the case of a 44-year-old Dans cet article, on présente le cas d’une patiente âgée
woman presenting with a giant thymolipoma causing de 44 ans présentant un thymolipome géant entraînant
symptoms of severe respiratory distress. The patient des symptômes de détresse respiratoire sévère. La pa-
underwent surgical resection, which is the main treat- tiente a subi une résection chirurgicale, qui est la moda-
ment, and remains free of disease. lité de traitement de choix. Elle est toujours asymptoma-
tique, sans aucune récurrence.

INTRODUCTION prior to admission. Her pulmonary function tests showed


severe obstruction with a forced vital capacity (FVC)
Thymolipoma is a slow growing mediastinal tumor of of 1.72 L and a forced expiratory volume (FEV1) of
thymic origin that is benign. It occurs between 2 and 9% 50%. Her arterial blood gas (ABG’s) was: pH: 7.41;
of all thymic neoplasms. It accounts for 1.1% of all solid pCO2: 42 mmHg; pO2: 65 mmHg; HCO3: 22 mEq/L. She
mediastinal tumors [1]. had a normal echocardiogram.
Patients with thymolipoma, usually remain asympto- Her chest X-ray (Fig. 1) showed a large mass in the
matic until the tumor achieves enormous proportions, anterior mediastinum, extending to the right hemithorax
causing airway and parenchymal compression [2]. That’s with collapse of the right inferior and middle lobes. Com-
why most thymolipomas are diagnosed incidentally after puter tomography scan (Fig. 1) demonstrated a large right
routine radiographic imaging [3]. subpulmonary fat containing lesion, extending to the
Thymolipoma may be rarely associated with myasthe- upper anterior mediastinum, and across the midline, to the
nia gravis, as well as with aplastic anemia and Grave’s left. Its transverse diameter was 23 cm, and its height
disease. [4] 17 cm. The mass was suspicious for a fatty tumor. A trans-
Our patient had severe shortness of breath, occurring at thoracic biopsy revealed the presence of fatty tissue.
rest, due to the enormous size of her thymolipoma. This It was decided to proceed with a right thoracotomy to
incapacitating dyspnea pushed her to seek medical care. either resect the tumor or to obtain an open biopsy. The
An urgent and succesfull surgery was sufficient to re- surgical procedure was performed under general double
lieve all her symptoms. lumen endotracheal anesthesia through a right thoraco-
tomy. The mass was occupying the majority of the chest
CASE DESCRIPTION without any local invasion.
The tumor appeared as a solid well circumscribed and
This is a 44-year-old woman complaining of shortness of encapsulated mass, composed of yellow soft adipose tis-
breath, nasal congestion and sneezing for five months. sue with small randomly scattered solid gray white areas
She was treated with bronchodilators initially. Later she (Fig. 2). The mass was removed in two parts due to its
developed a productive cough and early weakness. Her size. A 23.5 x 14.5 x 5 cm mediastinal mass was first
dyspnea was incapacitating at rest and she also had tachy- excised and sent for frozen section biopsy, followed by the
pnea and palpitation. Her physical examination revealed remaining mass measuring 21.5 x 20 x 15 cm. The two
decrease breath sounds on the right side. masses weighed 520 g and 1610 g respectively, which
These symptoms worsened over the last two weeks adds up to a total of 2130 g. Microscopically (Fig. 3), the

Saint George Hospital University Medical Center, Beirut, Lebanon. Departments of Surgery, Cardiothoracic Surgery Division;
1

Medicine, Pulmonary Medicine Division; Anatomic Pathology.


2 3

Correspondence to: Moussa Abi Ghanem, MD. St George Hospital University Medical Center. PO Box 166378. Beirut. Lebanon.
e-mail: moussaabighanem@hotmail.com
FIGURE 1. Chest X-ray shows a large mass in the anterior mediastinum, extending to the right hemithorax with collapse of the right inferior
and middle lobes. CT scan shows a large right subpulmonary fat containing lesion, extending to the upper anterior mediastinum, and
across the midline, to the left. Its transverse diameter is 23 cm, and its height 17 cm.

FIGURE 2. Macroscopic finding: 21.5 x 20 x 15 cm mass of adipose tissue covered by a thin translucent capsule.

FIGURE 3. Histologic findings: Mature adipose tissue (black filled star) with intimately admixed benign non neoplastic thymic tissue (open star)
where we can find several Hassall’s corpuscles (arrow).

M. ABI GHANEM et al. – Large thymolipoma Lebanese Medical Journal 2012 • Volume 60 (2) 107
yellow fatty tissue consisted of mature adipose tissue changes. Also Hassall’s corpuscules and lymphocytes are
devoid of atypia. The scattered gray white areas were seen.
composed of thymic tissue showing typical cortical and The two reports with the biggest number of thymolipo-
medullary zones with Hassall’s corpuscles. The final diag- ma cases were: “Thymolipoma: Analysis of 27 cases”
nosis was a benign thymolipoma without evidence of any published in 1994 [11], and “Thymolipoma: Clinico-
malignancy. pathologic review of 33 cases” published in 1995 [8].
Resection of the tumor resulted in immediate improve- The first report is a retrospective review of 27 cases
ment in the patient’s pulmonary status. The postoperative collected over 43 years. There were 15 male patients and
course was uneventful and the patient was discharged 12 female patients with age ranging from 2 to 66 years
home in seven days. At one month her pulmonary function (mean age 26.7 years). Eight patients (30%) were asymp-
tests (PFTs) returned to normal (FVC: 1.98 L and FEV1: tomatic, 15 patients (56%) had symptoms and four pa-
84%) and her oxygenation increased (pO2: 85 mmHg). tients with unknown clinical history. All tumors were
Today, after four years of follow-up, the patient is still found in the anterior mediastinum. Their sizes ranged from
doing fine without any complaint or any evidence of recur- 4 to 36 cm in longest dimension (mean size 18.4 cm). Only
rence on chest X-rays. one case had large components in both hemithoraces and
was resected in two pieces (one 33 cm and the other 17 cm
DISCUSSION in longest dimension).
The second report described 33 cases of thymolipoma
The first case of thymolipoma was described by Lange in identified from the files of the Department of Pulmonary
1916, where the tumor was discovered by chance during and Mediastinal Pathology at the Armed Forces Institute
the autopsy of a patient who died from metastasis of the of Pathology, Washington, D.C. and the Department
corpus uteri [5]. Andrus first successfully removed this of Pathology and Laboratory Medicine, Mount Sinai
tumor via a right thoracotomy [6]. Hall in 1948 suggested Medical Center of Greater Miami, Florida. There were
the term “thymolipoma” for tumors consisting of fat and 18 male patients and 15 female patients with age ranging
thymus tissue [7]. from 2 to 64 years (mean age 33 years). Eighteen patients
Thymolipoma accounts for 2 to 9% of all thymus neo- (54.5%) were asymptomatic. All tumors were located in
plasms and 1.1% of all solid mediastinal tumors [1]. They the anterior mediastinum. The tumors varied in size from
are found in children and adults with an average age of 4,5 to 36 cm in greatest dimension. No complications
33 years, with no gender predilection [8]. were observed in any of those patients after complete
Most reports state that this is an asymptomatic tumor. surgical resection.
However, others note that more than 50% of patients pre- Despite many case reports and large clinicopathologic
sent with symptoms, secondary to the local growth and reviews, the pathogenesis of thymolipoma remains un-
mechanical compression of the neighboring organs [3]. clear and very controversial [12]. Normally the thymus
Symptomatic patients mostly have dyspnea, chest pain, undergoes regression in late childhood and adulthood
upper respiratory infections and chest heaviness. Less where the parenchyma is replaced with fatty tissue. With
frequently patients report easy fatiguability, dysphagia, thymolipoma there is a definite increase in the thymus and
hemoptysis and weight fluctuation. fatty tissue. The vast majority of authors believe that thy-
Ten percent of these tumors can be associated with molipoma is a benign, mixed tumor of mesenchymal-
myasthenia gravis, aplastic anemia, Grave’s disease, lym- endodermal origin, and that what induces the concomitant
phangioma, chronic lymphatic leukemia, Hodgkin’s dis- proliferation of fatty and thymus tissue is not known.
ease, erythematous systemic lupus, hypogammaglobulin- Based on different histopathological and clinical find-
emia, and erythroblastopenia [9]. ings, R. Hudacko et al. discussed three possible theories
Thymolipomas are slow growing, non-infiltrating be- of thymolipomas pathogenesis [12].
nign tumors. Thus they usually are discovered accidently The 1st theory advocates a neoplastic origin from the
at autopsy. They usually do not recur after surgical resec- mesenchymal tissue (lipoma of thymic fat) or a mixed
tion [9]. tumor of both endodermal and mesenchymal origins
The differential diagnosis includes: mediastinal cyst, (mixed neoplasm of adipose and thymic tissue).
lipoma, mediastinal lipomatosis, thymoma, mature ter- The 2nd theory advocates a hamartomatous origin from
atoma, malignant neoplasm (liposarcoma, lymphoma, the thymic epithelium and adipose tissue.
and thymic carcinoma), and aneurysm of the ascending The 3rd theory advocates a regression category that
aorta. includes two theories of thymic involution (involuted thy-
Thymolipomas usually attain huge size (68% weighing moma v/s involuted thymic hyperplasia).
> 500 g, 23% > 2 kg, and one weighed over 12 kg) [10]. The association of thymolipoma with myasthenia gravis
On gross examination they have a flat surface, and are and other autoimmune disorders suggests that these tumors
surrounded by a capsule of connective tissue with a light are at least related [13]. This is in favor of the theory
yellowish-white surface. Histologically they consist most- suggesting that thymolipoma originates from a thymoma,
ly of mature fatty tissue, and small islands of thymus tis- with subsequent involution and replacement with adipose
sue that can be normal, hyperplasic or showing regressive tissue.

108 Lebanese Medical Journal 2012 • Volume 60 (2) M. ABI GHANEM et al. – Large thymolipoma
On the other hand, Kitano et al. suggest that thymo- 4. Sirpal YM, Chadha SK, Banarjee AK. Thymolipoma -
lipoma may be closely related to massive thymic hyper- Rare mediastinal tumour - Report of two cases and
plasia, in which the thymic tissue is gradually replaced by review of literature. Indian Journal of Cancer 1995; 32:
adipose tissue over time. The normalization of lymphocy- 23-6.
tosis that occurs with resection of both massive thymic 5. Lange I. Uber ein lipom des thymus. Zentralbl Allg
hyperplasia and thymolipoma lends some credibility to Pathol 1916; 27: 97-101.
6. Andurs WD, Foot NC. Report of a large thymic tumor
this theory [14].
successfully removed by operation. J Thorac Cardiovasc
Regardless of their origin, thymolipomas are benign Surg 1937; 6: 648.
tumors. 7. Hall GFM. A case of thymolipoma with observations on
a possible relationship to intrathoracic lipomata. Br J
CONCLUSION Surg 1948; 36: 321-4.
8. Moran CA, Rosado-de-Christenson M, Suster S. Thymo-
In conclusion, thymolipoma is a rare benign, well encap- lipoma: clinicopathologic review of 33 cases. Mod
sulated slowly growing tumor of unknown genesis. Thy- Pathol 1995 Sep; 8 (7): 741-4.
molipomas are usually asymptomatic, but can present with 9. Ferrari G, Paci M, Sgarbi G. Thymolipoma of the an-
respiratory failure. terior mediastinum: Videothoracoscopic removal using
a bilateral approach. Thorac Cardiov Surg 2006; 54:
Treatment consists of surgical removal.
435-9.
Long-term prognosis is excellent with no malignant 10. Thingnam SKS, Puri D, Jha NK, Vasishta RK, Suri RK.
transformation or recurrences reported. Thymolipoma of anterior mediastinum. Asian Cardiovasc
Thorac Ann 1999; 7: 62-4.
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M. ABI GHANEM et al. – Large thymolipoma Lebanese Medical Journal 2012 • Volume 60 (2) 109