Unusual presentation of giant cell tumor originating from a facet joint of the thoracic spine in a child: a case report and review of the literature

Journal of Medical Case Reports 07/2013; 7(1):178. DOI: 10.1186/1752-1947-7-178
Source: PubMed

ABSTRACT Giant cell tumor of the synovium is a common benign lesion that frequently occurs at the tendon sheaths in the hand; it is usually found in adults over 30 years old. It is related to pigmented villonodular synovitis. Giant cell tumor of the synovium or pigmented villonodular synovitis has been described rarely in the axial skeleton especially in the thoracic vertebrae of a child.
A previously healthy 7-year-old Thai girl presented with back pain and progressive paraparesis and was unable to walk for 1 month. She had weakness and hyperreflexia of both lower extremities. Magnetic resonance imaging showed a well-defined homogeneously and intensely enhanced extradural mass with cord compression at T4 to T7 levels. The patient underwent laminectomy at T4 through to T7 and total tumor removal. Permanent histopathologic sections and immunostains revealed a giant cell tumor of the synovium. Postoperative neurological status recovered to grade V. Magnetic resonance imaging at the 1-year follow-up showed no recurrence and there was no clinical recurrence at the 2-year follow-up.
We report an extremely rare case of giant cell tumor in the epidural space that extended from a thoracic facet joint. The tumor was removed successfully through laminectomies. Although giant cell tumor of a facet joint of the thoracic spine is very rare, it must be considered in the differential diagnosis for masses occurring in the epidural space in a child. Total tumor removal is the best treatment. Careful monitoring of recurrence can achieve a good clinical outcome.

Download full-text


Available from: Koopong Siribumrungwong, Dec 25, 2013
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Pigmented villonodular synovitis (PVNS) is a proliferative benign lesion originating from the synovium and commonly affects large joints of the extremities. PVNS can arise from any synovium in the whole body and rarely affects the zygapophyseal joints of the spine. Spinal PVNS is diagnosed mostly after resection of the mass. In our case we present a 22-year-old male patient showing progressive spastic paraparesis with insidious onset of back pain and difficulty of walking in a relatively short period of 1 month. After gross excision of the mass, diagnosis was established through histopathology. Two years of follow-up period reveals complete resolution of the patient's complaints and no recurrence on radiologic images.
    09/2013; 2013:870324. DOI:10.1155/2013/870324
  • [Show abstract] [Hide abstract]
    ABSTRACT: Three patients with spinal primary diffuse-type tenosynovial giant cell tumor (DTGCT) received surgical treatment in our department between 2002 and 2012. All 3 patients were female and aged 23, 33, and 44 years. The mean time from symptom onset to diagnosis was 17 months (range, 5-24 months). One case involved the C1 right lateral mass and C2 vertebral body, the second involved the C1-2 left lateral masses and C2 vertebral body, and the third involved the C5-7 left lateral mass and C6 vertebral body. All patients underwent computed tomographyguided biopsy to confirm the diagnosis of tenosynovial giant cell tumor. Gross total resection was achieved in all patients, including 2 piecemeal resections and 1 en-bloc resection. The mean follow-up time was 6 years (range, 1-11 years), and there was no sign of recurrence in the patients. Seventy cases have been identified so far in the English literature. The male to female ratio is 1:1.38. The mean patient age is 38.5 ± 17.9 years. The tumor distribution includes 32 cases in the cervical spine, 14 in the thoracic spine, 22 in the lumbar spine, and 1 in the sacrococcygeal region. The recurrence rate for patients who underwent gross total resection was 7.7%, and tumor progression was observed in 66.7% of patients who underwent subtotal resection. Above all, DTGCT is a rare primary spinal neoplasm. Preoperative image-guided biopsies play an important role in the diagnosis and treatment strategy. Gross total resection is the best treatment strategy and can reduce the recurrence rate.
    Turkish neurosurgery 09/2014; 24(5):804-813. DOI:10.5137/1019-5149.JTN.9594-13.1
  • [Show abstract] [Hide abstract]
    ABSTRACT: Introduction Pigmented Villonodular synovitis (PVNS) is a rare vertebral pathology--around 50 reports, only 3 concerning C1-C2 location. Case Report A 64-year-old man, submitted to a right nephrectomy for a clear cell carcinoma, presented with an asymptomatic osteolytic C1-C2 lesion. Even though the diagnosis of metastatic disease was the most probable, the presence of a solitary lesion without other osseous or systemic localization and the predicted low risk of recurrence imposed a surgical biopsy. A pigmented villonodular synovitis diagnosis was made, a rare vertebral pathology--around 50 reports, only 3 concerning C1-C2 location. No further treatment was assigned precluding the iatrogenic consequences of empirical treatments based on clinical diagnosis with no histopathological support. The patient remains stable at 18 months of follow-up. Conclusion A large differential diagnosis should be made when the typical findings for metastatic disease are absent precluding the iatrogenic consequences of empirical treatments based on clinical diagnosis with no histopathological support.
    European Spine Journal 06/2014; 24(S4). DOI:10.1007/s00586-014-3396-6