Vertebral Brown Tumor in Childhood (Case Report)

doi:10.4236/ss.2011.26074

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Surgical Science, 2011, 2, 344-347

doi:10.4236/ss.2011.26074 Published Online August 2011 (http://www.SciRP.org/journal/ss)

Vertebral Brown Tumor in Childhood (Case Report)

Csenge Szeverényi1, Balázs Dezső2, Tamás Deményi3, Zoltán Csernátony1

1Department of Orthopedic Surgery, Debrecen, Hungary

2Departmen t o f Pathology, Debrecen, Hungary

3University of Debrecen Medical and Health Science Center, General Practitioner, Debrecen, Hungary

E-mail: [email protected]

Received February 23 , 20 11; revised May 27, 2011; accepted June 9, 2011

Abstract

One form of renal osteodystrophy secondary to chronic renal failure is the osteitis fibrosa cystica, which is

the complication of secondary hyperparathyroidism. Osteitis fibrosa cystica is a histologically benign, tu-

mor-like lesion of bones. Since early diagnosis and treatment of hyperparathyroidism is available nowadays,

the development of osteitis fibrosa cystica has become rare. It can only be found in neglected cases and in

those where the treatment of chronic renal failure is unsuccessful. We present the case of a boy, who deve-

loped osteitis fibrosa cystica as a complication of secondary hyperparathyroidism. Our article is the first to

report a case about an osteitis fibrosa cystica found in a child’s vertebra and this tumor was the only one,

which reached a size of approximately 10 × 15 × 15 cm. We present the CT films and X-ray pictures, the

surgical solution and the results of the histological examination.

Keywords: Vertebral Localization, Osteitis Fibrosa Cystica, Secondary Hyperparathyroidism, Chronic Renal

Failure

1. Introduction

Brown tumor (i.e. osteitis fibrosa cystica, osteoclastoma,

giant cell reparative granuloma) is a lytic bony lesion in

non-treated or uncontrollable hyperparathyroidism. Ostei-

tis fibrosa cystica may develop in primary and in secon-

dary hyperparathyroidism as well. The most frequent

cause of secondary hyperparathyroidism is chronic renal

failure. Since the early diagnosis and treatment of hy-

perparathyroidism are available nowadays, the occur-

rence of brown tumor became very rare [1-7].

Our main objective with this case report is to draw at-

tention to the possible occurrence of brown tumors in

rare locations throughout the body and to provide an in-

sight on the diverse manifestation and localizations of

these lesions and the comprehensive treatment of this

disease and its complications.

2. Materials and Methods

Case Report

The 9-year-old male was taken to the doctor because of

enuresis. His renal failure was diagnosed at that time.

The results of the kidney biopsy performed were unable

to clarify the cause of the disease since it was in an ad-

vanced stage. Histology revealed severe glomerular,

nodular sclerosis. Conservative therapy (calcium carbon-

ate, sodium bicarbonate) was started in order to treat the

renal failure and it was recommended to keep a strict diet.

However, regular haemodialysis became necessary two

years later. Elevated parathyroid hormone levels were

detected 6 months after diagnosing the child’s renal fail-

ure {PTH: 27.0 pmol/L (Reference range: 0.8 - 5.2 pmol/L;

Primary hyperparathyroidism: 45.0 - 58.6 pmol/L; chro-

nic renal failure: 0.9 - 418.0 pmol/L)}, which was the

result of his chronic renal failure. Parathyroid scintigra-

phy was performed four years later because of his ex-

tremely high PTH levels. This study excluded the possi-

bility of a parathyroid adenoma in the cervical region and

in the mediastinum as well. Cervical CT scan revealed a

persistent thymus and calcifications in the soft tissue in

the retrosternal region and near the spinal processes of

the vertebrae. Meanwhile, the patient was excluded from

the waiting list of renal transplantation candidates, be-

cause of his severe obesity. Subtotal parathyroidectomy

was performed four months later due to high PTH levels

(PTH: 188.5 pmol/L). Histopathology of the parathyroid

revealed hyperplasia. A few months later, the child was

admitted to our Department, in order to remove the large

(15 × 15 × 10 cm) tumor on the rear side of his neck

C. SZEVERÉNYI ET AL.345

(Figure 1). The abnormality was detected a half year

before the operation for the first time and it was only 2 ×

1 × 3 cm large at that time.

Chest CT scan verified calcifications in the soft tissue

at the levels of the C7-Th4 vertebrae in the deep back

muscles of the left paravertebral region, the destruction

and calcification of the spinous process of the 7th cervi-

cal vertebra and a 10x15x15 cm large cystic tumor at the

level of C1-Th2 vertebrae, whose caudal part was calci-

fied (Figure 1).

During the surgery the cervical cyst was prepared and

removed with multiple cysts and solid tumors spreading

into the muscles of the back. There was no communica-

tion between the cyst and the liquor space, but the cyst

contained thick, white-yellowish fluid (Figure 2).

3. Results

According to the histological findings, the removed tu-

mor was diagnosed as osteitis fibrosa cystica, which was

the complication of the chronic hyperparathyroidism

(Figure 3).

On the basis of the imagery the tumor’s origin was in

the spinous process of the 7th cervical vertebra and

starting from that location, it invaded the nearby soft

tissues. Despite of the parathyroidectomy performed 4

months earlier, the child’s PTH serum level remained

high.

Figure 1. The tumor on the neck of the child grew to 15 × 5

× 10 cm large in half a year. On the chest CT scan image,

the deformity and calcification of the spinous process of the

7th cervical vertebra and the cystic tumor are visible.

Figure 2. During the surgical intervention, the cervical cyst

was removed with the other cystic and solid tumors

spreading into the muscles of the back.

Figure 3. Upper left section: the histopathology of the he-

matoxylin-eosin (HE) stain tissue section clearly shows that

the wall of the cyst is not covered by epithelium, therefore

the cyst is actually a pseudocyst. The lumen is covered by

necrotic debris with inflammatory cells, mainly macro-

phages (original magnification: 10x). Upper right section:

immunohistochemistry (IHC) for CD68 identifies macro-

phages (cells with brown membrane staining) including

foreign body giant macrophages (arrows). Note that osteo-

clast type of giant cells are CD68 negative (arrowheads).

(IHC with hematoxylin nuclear counter staining; original

magnification: 20x). Lower left section: Berlin blue staining

reveals the accumulation of hemosiderin laden macro-

phages (original magnification: 10x). Lower right section:

despite the huge size of the tumor, some remnant bone tra-

beculae (arrows) can be discovered in the histological sec-

tions. The big dark-blue (basophilic) precipitates corre-

spond to dystrophic calcifications (original magnification:

40x).

4. Discussion

In view of the fact that in chronic renal failure the matrix

of the kidneys shrinks, the production of the active vita-

min D will decrease, which has a primary etiological role

in the development of the brown tumor. Vitamin D fa-

cilitates the absorption of calcium from the intestines,

and it inhibits phosphate reabsorption in the kidneys.

Low levels of vitamin D lead to hypocalcaemia and

phosphate retention, which stimulate parathyroid hor-

mone secretion [4-6].

Parathyroid hormone facilitates the differentiation of

osteoclasts and these cells increase the serum calcium

level by bone resorption. In osteitis fibrosa cystica, reac-

tive fibrosis of the bone marrow can also be detected in

addition to the bone resorption caused by the activity of

the osteoclasts. The focal bone resorption and the pro-

gression of the fibrosis result in macroscopically visible

cysts, which build up the brown tumor [3,4,7-10 ]. During

the tumor growth the bone may become weak resulting

in pathologic fractures or spread into the soft tissues

C. SZEVERÉNYI ET AL.

346

causing compressive symptoms [4,6]. Furthermore, as in

our case, the tumor has a potential to become so large

that it makes everyday life difficult.

PTH also has an effect on the kidneys as it increases

phosphate excretion. Therefore, hypersecretion of PTH

can be inhibited by eliminating phosphate excess. It can

be achieved by oral antacids, dietetic restrictions and

peritoneal dialysis or haemodialysis [4,10,11,12]. How-

ever, after a certain period of time, the enlarged parathy-

roid gland might not react to the fluctuant serum calcium

level, therefore some degree of autonomous glandular

function develops [5,13]. When this occurs, conservative

treatment cannot prevent progressive bone resorption,

hypercalcaemia and metastatic calcifications anymore. In

these cases subtotal parathyroidectomy may be effective,

although secondary hyperparathyroidism might recur,

due to the presence of any residual parathyroid gland

tissue [4]. In our case, even though parathyroidectomy

was performed, serum PTH levels remained high (PTH:

206.1 pmol/L). This arose the suspicion the presence of

an autonomous functioning, ectopic parathyroid tissue in

the persistent thymus or somewhere else in the body,

causing hypersecretion of PTH.

Based on the literature reviewed, the incidence of

brown tumor involving bones is between 1.5% - 13%

among patients suffering from chronic renal failure [4,6,

7]. Furthermore, the lesion develops most frequently in

the pelvic bones, costae, sternum, maxillary sinus, man-

dible and in the femur [3,4,7,8,12,13]. The presence of

brown tumor in the vertebral column is rare. After re-

viewing the literature since 1974, only 19 similar cases

were published, with 8 patients having primary and 11

having secondary hyperparathyroidism as the underlying

cause of the disease [1,4-7,12,14]. We did not find a sin-

gle case of brown tumor developing from a vertebra of a

child and reaching such an enormous size. The signifi-

cance of osteitis fibrosa cystica involving th e verteb rae is

that it may cause the pathologic fractures of the vertebrae

or it may invade the spinal canal causing the compres-

sion of the spinal cord [1,4-7,12,14].

5. Conclusions

Our publication focused on the diagnosis, adequate

treatment and the prevention of possible complications of

brown tumors, which manifested as a divers multi-local

disease in our case. Brown tumor is a complication of

chronic renal failure and even though brown tumor has

become very rare nowadays, one has to think of it in pa-

tients suffering from chronic renal failure, and especially

in those cases in which the metabolic balance cannot be

achieved by conservative methods. In certain cases sur-

gical excision or spine-stabilizing intervention may be-

come necessary due to the localization or the size of the

tumor. In our case, finding and removing the suspected

ectopic parathyroid tissue and completing the kidney

transplantation may bring significan t improvement to the

patient’s state of health.

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