Solitary juvenile xanthogranuloma in cervical spine: case report and review of literature

Abstract

Solitary juvenile xanthogranuloma (JXG) in the spinal column is extremely rare. Here, we report and characterize the case of xanthogranuloma of the upper cervical spine. A 18-year-old male presented with neck pain for 3 months, along with progressive quadriparesis and sensory loss of 2 months duration with urinary retention. Motor examination revealed spastic quadriparesis with power of 2/5 in all the 4 limbs. Magnetic Resonance Imaging (MRI) spine with contrast showed a dorsally placed intradural extramedullary lesion at the level of C2-C4 vertebral body. The lesion, measuring 2.9x1.7x1.4 cm, was isointense on T1WI, hypointense on T2WI, and enhanced homogenously on contrast. He underwent an emergency C2-C4 laminectomy and complete excision of the lesion. At 3-month follow-up, he was asymptomatic except for mild neck pain. MRI scan of the cervical spine done at follow-up, revealed complete excision of tumor without any residual lesion. Histopathological examination of the mass revealed a polymorphous population of sheets of bloated pale foamy histiocytes (xanthoma cells), numerous admixed mature lymphocytes and several Touton giant cells. The cells were positive for CD68, a histiocytic marker, and negative for CD1a (excludes LCH) and S-100 (excludes RDD). KeywOrds: Juvenile xanthogranuloma, Cervical spine, MRI, Complete resection ÖZOmurgada soliter jüvenil ksantogranülom çok nadirdir. Burada üst servikal omurgada bir ksantogranülom olgusunu bildiriyoruz. 18 yaşında bir erkek 3 aydır boyun ağrısı, 2 aydır progresif kuadriparezi ve duyu kaybı ile üriner retansiyonla geldi. Motor inceleme 4 uzuvda 2/5 güçle spastik kuadriparezi gösterdi. Omurganın kontrastlı Manyetik Rezonans Görüntülemesi (MRG) C2-C4 vertebral cisim seviyesinde dorsal yerleşimli bir intradural ekstramedüller lezyon ortaya koydu. 2,9x1,7x1,4 cm boyutlarında olan lezyon T1WI ile izointens, T2WI ile hipointens ve kontrast ile homojen tutulum göstermekteydi. Acil C2-C4 laminektomi ve lezyonun tam eksizyonu yapıldı. 3 aylık takipte hafif boyun ağrısı dışında semptom yoktu. Takipte yapılan servikal omurga MRG taraması herhangi bir rezidüel lezyon olmadan tümörün tam eksizyonunu gösterdi. Kitlenin histopatolojik incelemesi şiş ve soluk köpüklü histiosit tabakalarının (ksantoma hücreleri) polimorfik bir popülasyonunu, araya karışmış çok sayıda matür lenfositi ve birkaç Touton dev hücresini gösterdi. Hücreler bir histiositik belirteç olan CD68 için pozitifti ve CD1a (LCH ekarte eder) ve S-100 (RDD ekarte eder) için negatifti.

Spontaneous regression of infantile dural-based non-Langerhans cell histiocytosis after surgery: case report

“…16,26,37 Among the 42 cases of isolated intracraniospinal nonLCHs (reported as JXG, fibrous xanthoma, xanthoma, fibroxanthoma, and XG) including our case (Table 1), 18 cases (43%) were dural-based lesions. 1,2,5,9,11,16,21,22,[25][26][27][28]30,32,35,37,38 Eleven of these 18 cases were intracranial 1,2,5,9,16,21,27,28,30,38 and the other 7 were spinal. 11,22,25,26,32,35,37 No cases showed recurrence during the follow-up period and essentially followed benign courses.…”

Imaging Features of Juvenile Xanthogranuloma of the Pediatric Head and Neck

“…2 Extracutaneous HN JXG has been reported in locations such as the tympanic membrane, nasal cavity, tongue, orbit and paranasal sinus, subglottis, cervical spine, skull base including the temporal bone, intracranial compartment, and HN muscles. [2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21] Cutaneous JXG typically presents as a solitary macule or papule with a flesh-colored, erythematous, or yellowish hue. 2 In such cases, radiographic imaging is not indicated.…”

Systemic juvenile xanthogranuloma: a case of spontaneous regression of intramedullary spinal cord, cerebral, and cutaneous lesions

“…1,8,19 Involvement of the spinal cord by JXG was reported in 8 patients, and all lesions had an extramedullary location. 8,16 Thus, our report is the first to describe an intramedullary spinal cord lesion of JXG associated with both brain and cutaneous lesions that all regressed spontaneously.…”

“…Thirty-three cases involved the central nervous system, 12 cases involved the peripheral nervous system, and 3 cases involved intraosseous JXGs, whose primary origin was a vertebral body. 1,8,16,19 Of the 12 patients with JXG lesions of the peripheral nervous system, 4 patients (33%) had lesions in the trigeminal nerve, 3 patients (25%) had lesions in a peripheral nerve, 3 patients (25%) had lesions in a spinal nerve, and 2 patients (17%) had lesions in the cauda equina. 1,8 Of the 33 patients with a central nervous system lesion of JXG, 22 patients (67%) had lesions in the brain, the cerebellum, or the posterior fossa and 1 patient (3%) each had a lesion in the optic nerve, the sellar region, and the cavernous sinus.…”

“…1,8 Of the 33 patients reported with a JXG lesion of the central nervous system, 17 were treated only surgically, 6 had medical treatment (chemotherapy and/or corticotherapy), 2 had both surgical and medical treatments, 5 underwent chemotherapy and radiotherapy, 1 patient was observed, and treatment was unknown in 2 patients. 1,8,16,19 Fifty-eight percent and 17% of the patients were disease free after surgery and after medical treatment (chemotherapy and/or corticotherapy), respectively. 1,8,16,19 All the patients treated with chemotherapy and radiotherapy had an intracerebral lesion, and complete remission was never attained.…”