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Langerhans Cell Histiocytosis of the Atlas: a Report of Three Cases

Posted on: Wednesday, 26 October 2005, 09:00 CDT

By Tanaka, Nobuhiro; Fujimoto, Yoshinori; Okuda, Teruaki; Nakanishi, Kazuyoshi; Et al

Langerhans cell histiocytosis sometimes involves the cervical spine and can be associated with collapse of the vertebral body1, but involvement of the atlas is rare. To the best of our knowledge, the cases of only eight patients with Langerhans cell histiocytosis of the atlas have been reported2-8.

We report the cases of three patients with Langerhans cell histiocytosis of the atlas, two of whom appeared to have been successfully treated with low-dose chemotherapy. The patients and/ or their parents were informed that information concerning the case would be submitted for publication.

Case Reports

CASE 1. An eleven-year-old boy had neck pain for two weeks followed by the development of persistent torticollis. The neck pain gradually increased, and the symptoms did not improve after the administration of antipyretic drugs. Two weeks after he began treatment, magnetic resonance imaging showed a destructive process within the lateral mass of C1, and the patient was referred to our hospital. The medical history was otherwise unremarkable.

Four weeks after the onset of symptoms, the boy required walking aids because of severe neck pain and persistent torticollis; the neck was bent to the left side and slightly rotated to the right side. Physical examination revealed stiffness, pain upon motion of the cervical spine, and tenderness of the left upper cervical spine. There were no neurological deficits. Blood tests showed a slightly increased C-reactive protein level of 0.5 mg/dL (5 mg/L) (normal, 0.0 to 0.4 mg/dL [0 to 4 mg/ L]) and alkaline phosphatase of 414 U/ L (normal range for an adult, 90 to 340 U/L), while the white blood- cell counts were within the normal range.

Cervical radiographs showed torticollis of the cervical spine, and computed tomography demonstrated an osteolytic lesion in the left lateral mass of the atlas (Fig. 1). Magnetic resonance imaging confirmed a mass that was isointensive on the T1-weighted image and high-intensity on the T2-weighted image (Fig. 2). The mass was shown to be expanding to the outside of the atlas on the left side.

With the patient under local anesthesia and in a prone position, a percutaneous needle biopsy was performed with computed tomography guidance (Fig. 3, A and B). The biopsy needle was inserted posterolaterally to the lesion. There were no complications, and the histologic findings were diagnostic of Langerhans cell histiocytosis (Fig. 3, C).

After immobilization of the cervical spine with a collar, the pain and torticollis gradually improved. Low-dose chemotherapy with oral alternate-day prednisone (40 mg per square meter of skin per day) and weekly methotrexate (20 mg per square meter of skin once weekly) was administered for six months, and a cervical collar was worn for four months. No complications accompanied the chemotherapy. The symptoms resolved at twelve weeks, and computed tomography demonstrated partial bone-remodeling. No new symptoms occurred during the follow-up period of twenty-four months, and computed tomography scans made at the latest follow-up examination demonstrated excellent remodeling of the atlas (Fig. 4).

Fig. 1

Case 1. Axial computed tomography scan showing an osteolytic lesion in the left lateral mass of the atlas.

Fig. 2

Case 1. Axial magnetic resonance imaging scans showing an isointensive mass on the T1-weighted image (A) and a high-intensity mass on the T2-weighted image (B).

CASE 2. A three-year-old boy had neck pain and persistent torticollis for three days. The initial diagnosis was inflammatory torticollis, and he was treated with a cervical collar and antipyretic drugs. Two weeks after he was first seen, a computed tomography scan showed a lytic lesion in C1 (Fig. 5, A). Persistent torticollis, pain with motion, and tenderness of the cervical spine were found. No neurological deficits were noted on physical examination, and the medical history was unremarkable. Blood tests showed increased alkaline phosphatase of 655 U/L (normal range for an adult, 90 to 340 U/L), while the white blood-cell count and C- reactive protein values were within the normal range.

Fig. 3

Case 1. A and B: Axial computed tomography scans showing a biopsy needle inserted into the lesion. The white arrow indicates the direction of the needle, and the black arrow shows the tip of the needle. C: Photomicrograph showing numerous Langerhans cells and eosinophils (hematoxylin and eosin, original magnification 400).

Fig. 4

Case 1. Axial computed tomography scan (A) and reconstructed computed tomography image (B) made at the two-year follow-up examination, showing excellent remodeling of the atlas.

A computed tomography-guided percutaneous needle biopsy through a posterolateral approach was performed without any complications. Histological analysis revealed Langerhans cell histiocytosis. Conservative treatment with immobilization in a cervical collar for three months and low-dose chemotherapy, consisting of oral alternate- day prednisone (40 mg per square meter of skin per day) and weekly methotrexate (20 mg per square meter of skin once weekly) for a period of six months, was administered. The pain and torticollis completely resolved without any complications, and a computed tomography scan at the two-year follow-up showed excellent bone- remodeling (Fig. 5, B).

CASE 3. A six-year-old girl had been treated with chemotherapy and radiation therapy for multifocal Langerhans cell histiocytosis of the thoracic spine, right femur, and skull for four years. She had neck pain and torticollis for three months. A computed tomography study revealed severe torticollis, a lesion involving C1, and severe atlantoaxial subluxation (Fig. 6, A). Physical examination revealed pain with motion and tenderness of the cervical spine but no neurological deficits.

Fig. 5

Case 2. A: Axial computed tomography scan showing an osteolytic lesion in the left lateral mass of the atlas. B: Axial computed tomography scan made at the two-year follow-up examination, showing excellent remodeling of the atlas.

A halo vest was worn for two weeks, and then an operation that included curettage of the lesion and posterior fixation with instrumentation and bone-grafting was performed (Fig. 6, B). Histological analysis revealed Langerhans cell histiocytosis. A cervical collar was worn for three months, and the pain and torticollis completely resolved without any complications. A follow- up radiograph made eight years later demonstrated excellent bone- remodeling and fusion with no recurrence of the Langerhans cell histiocytosis.

Discussion

Langerhans cell histiocytosis is a benign condition of localized or multifocal proliferation of histiocytes. Its prevalence is approximately 1:1,500,000 people9. Schajowicz reported that 81% of all patients with Langerhans cell histiocytosis have a solitary eosinophilic granuloma, 7% have multiple eosinophilic granulomas, and 11% have widely disseminated lesions of Hand-Schller-Christian disease syndrome or Letterer-Siwe syndrome10. This group of diseases was introduced as histiocytosis X and was later renamed Langerhans cell histiocytosis on the basis of the pathologic findings11,12. Langerhans cell histiocytosis in the spine is relatively rare, with a frequency that has been reported to range between 6.5% and 25%10,13,14. Clinical symptoms are often severe, and the diagnosis is sometimes difficult. Bertram et al.15 reported that the cervical spine at C3-C5 is involved in 60% of children with Langerhans cell histiocytosis, but it is quite rare for the disease to affect the atlas.

Fig. 6

Case 3. A: Axial computed tomography scan showing severe torticollis and atlantoaxial subluxation. B: Postoperative radiograph showing the occipitocervical posterior fusion.

The most common symptoms of Langerhans cell histiocytosis involving the cervical spine are neck pain and restricted range of motion, or torticollis. The three patients described in the present report demonstrated all of these symptoms without neurological deficits. Although lytic lesions of the atlas were found on radiographs, vertebral plana, which is a typical radiographic finding in pediatric patients, was not found. Because the magnetic resonance imaging and computed tomography studies suggested diseases such as osteosarcoma, Ewing sarcoma, and spinal infection, biopsy specimens for histologic diagnosis were required. Computed tomography-guided percutaneous needle biopsies were performed without complications. In our institution, the radiologist does the procedure and frozen sections are made to confirm that the biopsy had obtained lesional tissue. Computed tomography-guided needle biopsy has been reported to be a safe and effective technique for histologic diagnosis, with an accuracy in achieving a diagnosis of 70% to 100%16-18.

As for the treatment of Langerhans cell histiocytosis of the cervical spine, many authors have reported that immobilization is adequate for most patients11,19-22. While an operative procedure may not be needed for a solitary osseous lesion, one of our current patients who had severe torticollis and persistent atlantoaxial subluxation required posterior arthrodesis. On the other hand, Womer et al.23 described using alternate-day prednisone and weekly methotrexate for the treatment of low-risk Langerhans cell histiocytosis in thirteen p\atients who were more than one year old and had no bone marrow involvement or organ dysfunction. Twelve of these patients were treated successfully, with limited toxicity manifested by slight and transient elevations in hepatic enzymes in three patients. Those authors concluded that low-dose chemotherapy was safe and effective for patients with low-risk Langerhans cell histiocytosis. The use of chemotherapy to treat Langerhans cell histiocytosis is controversial, but it seems to have been safe and effective in the two patients in the present report who did not have atlantoaxial subluxation.

In conclusion, three patients with Langerhans cell histiocytosis of the atlas were treated and all had a full recovery and complete resolution of the symptoms. Imaging studies and computed tomography- guided percutaneous needle biopsies were useful for diagnosing Langerhans cell histiocytosis at an early stage. It is important for surgeons to recognize that Langerhans cell histiocytosis may involve the atlas.

References

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15. Bertram C, Madert J, Eggers C. Eosinophilic granuloma of the cervical spine. Spine. 2002;27:1408-13.

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17. Dupuy DE, Rosenberg AE, Punyaratabandhu T, Tan MH, Mankin HJ. Accuracy of CT-guided needle biopsy of musculoskeletal neoplasms. AJR Am J Roentgenol. 1998;171:759-62.

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19. Kumar A. Eosinophilic granuloma of the spine with neurological deficit. Orthopedics. 1990;13:1310-2.

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BY NOBUHIRO TANAKA, MD, YOSHINORI FUJIMOTO, MD, TERUAKI OKUDA, MD, KAZUYOSHI NAKANISHI, MD, TADAYOSHI SUMIDA, MD, HIDEKI MANABE, MD, AND MITSUO OCHI, MD

Investigation performed at the Department of Orthopaedic Surgery, Graduate School of Biomedical Sciences, Hiroshima University, Hiroshima, Japan

Nobuhiro Tanaka, MD

Teruaki Okuda, MD

Kazuyoshi Nakanishi, MD

Mitsuo Ochi, MD

Department of Orthopaedic Surgery, Graduate School of Biomedical Sciences, Hiroshima University, Kasumi 1-2-3, Minami-ku, Hiroshima 734-8551, Japan. E-mail address for N. Tanaka: nobut@hiroshima- u.ac.jp

Yoshinori Fujimoto, MD

Department of Orthopaedic Surgery, Hiroshima General Hospital, Jigozen 1-3-3, Hatsukaichi, Hiroshima 738-8503, Japan

Tadayoshi Sumida, MD

Hideki Manabe, MD

Department of Orthopaedic Surgery, Asa City Hospital, Kabeminami 2-1-1, Asakita-ku, Hiroshima 731-0293, Japan

The authors did not receive grants or outside funding in support of their research or preparation of this manuscript. They did not receive payments or other benefits or a commitment or agreement to provide such benefits from a commercial entity. No commercial entity paid or directed, or agreed to pay or direct, any benefits to any research fund, foundation, educational institution, or other charitable or nonprofit organization with which the authors are affiliated or associated.

doi:10.2106/JBJS.D.03008

Copyright Journal of Bone and Joint Surgery, Inc. Oct 2005

Source: Journal of Bone and Joint Surgery; American volume

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