Table of Contents  
CASE REPORT
Year : 2015  |  Volume : 8  |  Issue : 5  |  Page : 659-662  

Solitary eosinophilic granuloma of humerus in a 2-month-old infant: A case report with 3 years follow-up


Department of Paediatric Orthopaedic Surgery, Shalby Hospitals, Ahmedabad, Gujarat, India

Date of Web Publication10-Sep-2015

Correspondence Address:
Prateek S Joshi
H/102 Shaligram Flora, Near Sangeeni Bunglows, Opposite Shaligram 3, Thaltej, Ahmedabad - 380 054, Gujarat
India
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Source of Support: Nil., Conflict of Interest: None declared.


DOI: 10.4103/0975-2870.164962

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  Abstract 

Eosinophilic granulomais (EG) a benign self-limiting disease which belongs to the spectrum of Langerhans cell histiocytosis. It is characterized by single or multiple skeletal lesions involving skull, mandible, ribs, spine and long bones predominately in children <12 years. We report a relatively rare case of left proximal humerus solitary EG in a month old infant who was brought to us with reduced movements of left upper limb and swelling of left shoulder. X-ray revealed osteolytic lesion in left upper humerus. No associated lesions were revealed by other imaging modalities. Open biopsy and curettage of lesion revealed proliferation of histiocytes with an infiltration of eosinophils. Immunohistochemistry was positive for S-100 and CD1a. Hence, diagnosis of solitary EG was made. Baby was followed up every 6 monthly for 3 years. There was no evidence of recurrence or detection of new lesion elsewhere at last follow-up.

Keywords: Eosinophilic granuloma, humerus, langerhans cell histiocytosis


How to cite this article:
Joshi PS, Devmurari KN. Solitary eosinophilic granuloma of humerus in a 2-month-old infant: A case report with 3 years follow-up. Med J DY Patil Univ 2015;8:659-62

How to cite this URL:
Joshi PS, Devmurari KN. Solitary eosinophilic granuloma of humerus in a 2-month-old infant: A case report with 3 years follow-up. Med J DY Patil Univ [serial online] 2015 [cited 2020 Oct 21];8:659-62. Available from: https://www.mjdrdypu.org/text.asp?2015/8/5/659/164962


  Introduction Top


Langerhans cell histiocytosis (LCH) is a relatively uncommon disease suffered by infants and children. Many factors have been considered as the cause of this disease. Willman et al. described that LCH is probably a clonal neoplastic disorder with highly variable biologic behaviour.[1] Shannon and Newton described that LCH is Langerhans cell accumulation and proliferation at a focal site by immunoregulatory dysfunctions because many patients of LCH show a decrease of suppressor T-lymphocytes in the peripheral blood, thymic dysfunction, or hypergammaglobulinemia.[2] However, no conclusion has yet been reached. In 1987 the Writing Group of the Histiocytosis Society proposed that LCH has become the preferred term,[3] replacing histiocytosis × which had been proposed by Lichtenstein in 1953.[4] This disease encompasses three disorders: Eosinophilic granuloma (EG), Hand-Schuller-Christian syndrome, and Letterer-Siwe syndrome according to their clinical and pathologic features.[4] EG is the most common subtype, accounting for about 70% of LCH cases.[5] EG has got a good prognosis and may spontaneously regress; it is extremely radiosensitive.[6] We present one such relatively rare case of an infant presented to us at 1-month of age with left proximal humerus Solitary EG.


  Case Report Top


A 1-month-old infant was brought to us with complains of reduced spontaneous movements of left upper extremity, excessive crying when held at left shoulder and mild fullness of left shoulder [Figure 1]. Baby was the first child born at full gestation by normal vaginal delivery to nonconsanguineous marriage. On general examination, infant's nutritional status, developmental milestones were within normal range. There were scaly erythmatous rashes over trunk [Figure 2]. On local examination, there was tender bony swelling of left proximal humerus. Left shoulder movements provoked excessive crying. Distal neuro-vascular status was intact. There was osteolytic expansile lesion of left proximal humerus involving metaphysis extending till metaphysio-diaphyseal junction on X-ray [Figure 3]. Magnetic resonance imaging revealed low signal intensity in the proximal humerus on T1-weighted images and high signal intensity on T2-weighted images [Figure 4]a. By using computed tomography (CT) scan, the lesion was demarcated and breach of cortex and joint penetration were excluded. As per CT scan the dimensions of the lesion were 23.3 mm in medio-lateral, 22.2 mm in antero-posterior and 20.9 mm in supero-inferior plane [Figure 4]b. Technetium bone scintigraphy revealed increased uptakes in proximal humerus. There were no other bones involved. Blood tests results were within normal range.
Figure 1: Pre-operative clinical photograph

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Figure 2: Skin rashes

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Figure 3: X-ray showing expansile lytic lesion in proximal humerus

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Figure 4: Magnetic resonance imaging (a) computed tomography (b)

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After thorough evaluation, an incisional biopsy and curettage of lesion was performed through delto-pectoral approach. Primary bone grafting wasn't attempted because of relatively small dimension of lesion. Arm was protected in shoulder immobilizer for 4 weeks. Histo-pathological examination revealed proliferation of histiocytes with an infiltration of eosinophils [Figure 5]. Immunohistochemically, these histiocytes were positive for S-100 and CD1a. A diagnosis of Solitary EG was made. Gradual increased use of left extremity was noted over period of 6 months. Child was followed up every 6 monthly for total duration of 3 years. At final follow-up child has near normal function at left shoulder without evidence of local recurrence, involvement of other bones or systemic involvement [Figure 6]a and [Figure 6]b.
Figure 5: Histopathology of bone showing proliferation of histiocytes with eosinophils

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Figure 6: Functional outcome at 3 years follow-up (a) X-ray at 3 years follow-up (b)

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  Discussion Top


Solitary EG of bone can present dilemmas of both diagnosis and treatment. The etiology is unclear and the therapeutic approach controversial. Since the clinical course of the disease is usually benign a simple, minimally invasive form of treatment with a low rate of complications is desirable. In view of the possibility of spontaneous resolution, biopsy and curettage alone to confirm the diagnosis[7] could be a strategy for treatment. Our results support this approach for the treatment of solitary EG of bone in the skeletally immature.

Intra-lesional infiltration with corticosteroids, either as an adjunct to treatment or as primary treatment, was recommended by Capanna et al.[8] as being effective, convenient and safe. Egeler et al.[9] found that the results of treatment with intralesional steroids were comparable to those of other forms of treatment reported in the literature. Although this treatment seems minimally invasive they reported 2 complications in fifteen patients, 1 case of femoral osteomyelitis and one of obstructive hydrocephalus. Some lesions fail to respond or are unsuitable for treatment by injection because of their site, impending fracture or soft tissue invasion. The resolution of symptoms in the skeletally immature after confirmation of the diagnosis is analogous to the previous findings of vertebral remodeling in patients with EG of the spine. Raab et al.[10] showed that, in fourteen patients without neurological signs who were aged between 1.2 and 11.3 years, conservative treatment with immobilization in a brace was sufficient to allow remodeling and reconstitution of the vertebral height. Similar spontaneous resolution in skeletally immature patients can be predicted at other anatomical sites too, as is seen in our case. Previous studies[11],[12] show a wide variation in the outcome after different treatments for EG. One explanation may be the age variation in the mix of the patients. Since EG in skeletally immature patients is more likely to resolve, regardless of the form of treatment, a series with a higher percentage of younger patients should show a better rate of success. The discovery of clonality in unifocal EG does not allow either the biological outcome or the clinical behavior to be predicted.[13]

Apart from curettage and observation, bracing and local steroid injections, role of bone grafting, systemic steroid, indomethacin, chemotherapy and local radiotherapy has been described in literature. All of these treatments are reported to give satisfactory results with a recurrence rate of <20%.[11],[12],[14],[15] When the lesion is extensive and curettage leads to significant dead space, one should consider bone grafting to prevent pathological fracture and promote early healing. Systemic steroid and chemotherapy may be useful as an adjunct to surgical treatment in cases with systemic involvement. Local radiotherapy has role in patients with tumor involving locations which are difficult to access surgically.

In our case there was no breach of cortex or adjacent joint and cavity dimensions were not extensive hence primary bone grafting was not performed and limb was protected with shoulder immobilizer for 4 weeks. At 3 years follow-up child has neither local recurrence nor other bone involvement nor systemic involvement with near normal functional status of left upper limb.

Eosinophilic Granuloma should be considered as one of the differential diagnosis in infants with skeletal and systemic involvement. Other differentials to be kept in mind are nonossifying fibroma, chondromyxoid fibroma, monoostotic fibrous dysplasia, aneurysmal bone cyst, bone infarct, metastasis, brodie abscess, chondroblastoma and variants of osteosarcoma. Timely diagnosis by biopsy and curettage with or without bone grafting should be adequate in skeletally immature patients. Patient should be followed up regularly in order to look for local recurrence or systemic symptoms. Chemotherapy and systemic steroid is helpful in cases with systemic involvement. Local radiotherapy should be offered in cases with difficult surgical access.


  Acknowledgment Top


We sincerely thank Dr. Prem Madan for his valuable contributions to our research work.

 
  References Top

1.
Willman CL, Busque L, Griffith BB, Favara BE, McClain KL, Duncan MH, et al. Langerhans'-cell histiocytosis (histiocytosis X): A clonal proliferative disease. N Engl J Med 1994;331:154-60.  Back to cited text no. 1
    
2.
Shannon BT, Newton WA. Suppressor-cell dysfunction in children with histiocytosis-X. J Clin Immunol 1986;6:510-8.  Back to cited text no. 2
    
3.
Histiocytosis syndromes in children. Writing Group of the Histiocyte Society. Lancet 1987;1:208-9.  Back to cited text no. 3
    
4.
Lichtenstein L. Histiocytosis X; integration of eosinophilic granuloma of bone, Letterer-Siwe disease, and Schüller-Christian disease as related manifestations of a single nosologic entity. AMA Arch Pathol 1953;56:84-102.  Back to cited text no. 4
    
5.
Konrad H, Clark BJ, Rose GE. Lymphocytic, plasmacytic, histiocytic, and hematopoietic tumors of the orbit. In: Tasman W, Jaeger EA, editors. Duane's Clinical Ophthalmology. Philadelphia, PA: Lippincott Williams & Wilkins; 2000. p. 30-2.  Back to cited text no. 5
    
6.
Yang JT, Chang CN, Lui TN, Ho YS. Eosinophilic granuloma of the skull: Report of four cases. Changgeng Yi Xue Za Zhi 1993;16:257-62.  Back to cited text no. 6
    
7.
Chu T, D'Angio GJ, Favara B, Ladisch S, Nesbit M, Pritchard J. Histiocytosis syndromes in children. Writing Group of the Histiocyte Society. Lancet 1987;2:41-2.  Back to cited text no. 7
    
8.
Capanna R, Springfield DS, Ruggieri P, Biagini R, Picci P, Bacci G, et al. Direct cortisone injection in eosinophilic granuloma of bone: A preliminary report on 11 patients. J Pediatr Orthop 1985;5:339-42.  Back to cited text no. 8
    
9.
Egeler RM, Thompson RC Jr, Voûte PA, Nesbit ME Jr. Intralesional infiltration of corticosteroids in localized Langerhans' cell histiocytosis. J Pediatr Orthop 1992;12:811-4.  Back to cited text no. 9
    
10.
Raab P, Hohmann F, Kühl J, Krauspe R. Vertebral remodeling in eosinophilic granuloma of the spine. A long-term follow-up. Spine (Phila Pa 1976) 1998;23:1351-4.  Back to cited text no. 10
    
11.
Sessa S, Sommelet D, Lascombes P, Prévot J. Treatment of Langerhans-cell histiocytosis in children. Experience at the Children's Hospital of Nancy. J Bone Joint Surg Am 1994;76:1513-25.  Back to cited text no. 11
    
12.
Womer RB, Raney RB Jr, D'Angio GJ. Healing rates of treated and untreated bone lesions in histiocytosis X. Pediatrics 1985;76:286-8.  Back to cited text no. 12
    
13.
Willman CL. Detection of clonal histiocytes in Langerhans cell histiocytosis: Biology and clinical significance. Br J Cancer Suppl 1994;23:S29-33.  Back to cited text no. 13
    
14.
Ladisch S, Gadner H. Treatment of Langerhans cell histiocytosis — evolution and current approaches. Br J Cancer Suppl 1994;23:S41-6.  Back to cited text no. 14
    
15.
McLelland J, Broadbent V, Yeomans E, Malone M, Pritchard J. Langerhans cell histiocytosis: The case for conservative treatment. Arch Dis Child 1990;65:301-3.  Back to cited text no. 15
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]



 

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  In this article
Abstract
Introduction
Case Report
Discussion
Acknowledgment
Introduction
Case Report
Discussion
Acknowledgment
References
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