Treatment Outcome of Langerhans Cell Histocytosis

So Hak Chung; Jae Do Kim; Hyun Ik Jo

doi:10.5292/jkbjts.2014.20.1.14

Journal List > J Korean Bone Joint Tumor Soc > v.20(1) > 1052042

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Chung, Kim, and Jo: Treatment Outcome of Langerhans Cell Histocytosis

Original Article

J Korean Bone Joint Tumor Soc 2014;20(1):14-21.

Published online: 10 June 2014

DOI: https://doi.org/10.5292/jkbjts.2014.20.1.14

Treatment Outcome of Langerhans Cell Histocytosis

So Hak Chung, Jae Do Kim, Hyun Ik Jo

Department of Orthopedic Surgery, Kosin University Gospel Hospital, Busan, Korea

Correspondence to: So Hak Chung Department of Orthopedic Surgery, Kosin University Gospel Hospital, 34 Amnam‐dong, Seo‐gu, Busan 602‐702, Korea TEL: +82-51-990-6467 FAX: +82-51-243-0181 E-mail: shchung@kosin.ac.kr

Received 1 April 2014 Revised 21 May 2014 Accepted 29 May 2014

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Purpose

To analyze the clinical features and treatment outcome of Langerhans’ cell histocytosis.

Materials and Methods

From August 1996 to June 2013, 28 patients who histologically proven with LCH were analyzed of medical records, radiography, pathologic character retrospectively.

Results

A total of 28 cases of LCH including 22 child has been reported. Onset age was 0.6 to 51 years old, occurred in the average age was 14.8 years. Follow‐up period was 6 months to 134 months average was 44.6 months. The M:F ratio was 2.5:1. The initial symptoms was pain in 18 cases, 5 cases of pathologic fracture, 3 case of palpable mass, 1 case of discovered by accident in radiography, 1 case of torticollis. In radiological examination osteolysis was seen all cases, 7 cases showed a periosteal reaction, 1 case showed soft tissue extension. Clinical type of all cases were eosinophilic granuloma. 25 cases were classified as unifocal disease and 3 cases were multifocal single systemic diseases. In all cases, incisional biopsy was performed. After histologic confirmed, 14 cases was treated with curettage or surgical excision of the lesion and the other 14 cases were followed up without treatment. There is no death during follow up period. 11 cases has no radiological improvement after 3‐6 months observation, intralesional steroid injection was performed.

Conclusion

Patients with LCH who has rapid systemic onset is very rare, so if you meet the young children who suspected LCH, you shoulder avoid the examination which cause excessive radiation exposure to the young patient. In order to confirm the diagnosis of disease, biopsy is needed. Close observation after confirmed by histological method will bring the satisfactory results. But the patients who had pathologic fracture or wide bone destruction already may need curettage and bone grafting to lesion or internal fixation. The lesion which has no radiological improvement after 3‐6 months observation or appear with pain interferes daily life may need local steroid injection as a good treatment.

Keywords: Langerhans cell histiocytosis, intralesional steroid injection

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Figure 1.

Lateral view of LCH in the cervical spine. Kyphotic deformity was seen at initial view (Left). After ILSI patient recover the normal curvature (right).

Figure 2.

CT view showing osteolytic lesion in the 2, 3 cervical vertebra.

Figure 3.

AP view of LCH in the right humeral shaft area. Osteolytic lesion and periosteal reaction was seen. (Left) After surgical biopsy simple observation was applied. 8 months after the observation bone healing with sclerosis was seen.

Figure 4.

LCH lesion with pathologic fracture at right proximal humerus. 11 month after operation with TENs bone union was acquired.

Table 1.

Langerhans Cell Histiocytosis: Clinical Types and Categorization Schemes⁹⁾

Clinical types of Langerhans cell histiocytosis

Categorization by Histiocyte Society for treatment protocols (current)

Eosinophilic granuloma
  Most common form of Langerhans cell histiocytosis
  Localized form, most benign
  Older children and adults
  >75% of affected individuals younger than 20 years
  Unifocal lesions 3 times more common (skull > femur > pelvis > vertebra > jaws)
  Multifocal lesions less common (50% skull, 16% Jaws) Rare skin lesions
  Letterer-Siwe disease
  Usually 1st year of life
  Mucocutaneous lesions including gingival and oral mucosa
  Seborrheic dermatitis-like skin lesions
  Purpuric red-brown nodules
  Ulcerated painful nodules involving perineal, inguinal, retroauricular, and external auditory canal regions Lung, liver, and spleen involvement
Hand-Schuller-Christian disease
  Usually 2–6 year old children Classic triad: osteolytic lesions, exophthalmos, and diabetes insipidus Skin and oral lesions
Congenital self-healing Langerhans cell histiocytosis (reticulohistiocytosis, Hashimoto – Pritzker disease)
Pulmonary Langerhans cell histiocytosis

Unifocal disease
  Single system disease with single site of involvement Most commonly bone Older children and adults Good prognosis
Multifocal single system disease
  Multiple sites of involvement in single organ system Most commonly bone Young children Intermediate prognosis
Multifocal multisystem disease
  Multiple involved sites in more than one organ system Most commonly bone, skin, liver, spleen, and lymph nodes Children younger than 2 years of age and infants Poor prognosis
Congenital self-healing Langerhans cell histiocytosis
  Multiple skin lesions at birth or shortly after mimicking congenital neuroblastoma or leukemia (“blueberry muffin baby”) Neonates and infants Self-healing involution
Pulmonary Langerhans cell histiocytosis
  Young adult smokers (“smokers malady”) Indolent progression to pulmonary fibrosis Strong association with malignancies Extremely rare in children
Secondary Langerhans cell histiocytosis associated with neoplasms
  Acute lymphoblastic and myelogenous leukemias Chronic myelogenous leukemia Myelodysplastic disorder association
  Non-Hodgkin and Hodgkin lymphoma Retinoblastoma Osteosarcoma Thyroid carcinoma
  Lung cancer (adenocarcinoma, small cell carcinoma) Prostate cancer
  Breast cancer Parathyroid adenoma Pancreatic cystadenoma

Table 2.

Patient's Characteristics

Case No	Age	Sex	Osseous lesion	Clinical type	HS type	Treatment	F/U (month)	ILSI
1	11	F	Pelvis, spine, skull	EG	MS	Bx, Cu, Total Corpectomy	134	0
2	2	F	Femur, spine	EG	MS	Bx, ILSI	108	3
3	6	M	Tibia	EG	US	Bx, B/G, ILSI	135	4
4	9	M	Femur	EG	US	Bx, Cu, B/G, OR/IF c Plate	39	0
5	10	F	Fibular	EG	US	Bx	108	0
6	2	M	Humerus	EG	US	Bx, Cu, B/G	18	0
7	12	M	Radius	EG	US	Bx, ILSI	9	2
8	51	F	Scapular	EG	US	Bx, ILSI	102	1
9	3	F	Radius	EG	US	Bx, ILSI	11	1
10	0.6	M	Ulna, femur	EG	MS	Bx	47	0
11	12	M	Femur	EG	US	Bx, Cu, B/G, CR/IF c Ender Nail	119	0
12	40	M	Scapular	EG	US	Bx, Cu, B/G	28	0
13	22	M	Humerus	EG	US	Bx, Cu, B/G, OR/IF c Plate	29	0
14	11	F	Humerus	EG	US	Bx	27	0
15	17	M	Pelvis	EG	US	Bx, Cu, B/G	29	0
16	4	F	Spine	EG	US	Bx, ILSI	58	1
17	16	M	Scapular	EG	US	Bx, ILSI	44	1
18	31	M	Clavicle	EG	US	Bx, Cu, B/G, CR/IF c K-wire	9	0
19	16	M	Clavicle	EG	US	Bx, Cu, B/G	34	0
20	7	M	Humerus	EG	US	Bx, Cu, B/G, CR/IF c TEN	8	0
21	4	M	Humerus	EG	US	Bx, ILSI	49	1
22	49	M	Humerus	EG	US	Bx	29	0
23	3	M	Tibia	EG	US	Bx, ILSI	10	1
24	3	M	Clavicle	EG	US	Bx, ILSI	36	2
25	21	M	Scapular	EG	US	Bx, Cu, B/G	9	0
26	1	F	Humerus	EG	US	Bx, ILSI	8	1
27	13	M	Humerus	EG	US	Bx	7	0
28	15	M	Pelvis	EG	US	Bx	6	0

US, Unifocal Single systemic disease; MS, Multifocal Single systemic disease; EG, Eosinophilic Granuloma; Bx, Biopsy; Cu, Curettage; B/G, Bone Graft; ILSI, Intra lesional steroid injection.

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