What Is Causing This Adolescent's Shoulder Pain?
A 14-year-old girl was brought in to her pediatrician for evaluation of a 3-month history of right shoulder pain, which had become particularly uncomfortable over the previous 2 weeks. Despite being a competitive wrestler at her high school, she denied history of acute trauma to the joint and reiterated the progressive, dull, burning nature of the pain. She also denied any fever, chills, and fatigue. There was no family history of arthritis. Physical examination revealed a bony swelling over the right distal clavicle, which was tender to palpation with no overlying erythema. A radiograph of her right shoulder was performed (Figure 1).
Follow-up magnetic resonance imaging (MRI) scans showed a multiloculated lesion with fluid levels expanding the distal diaphysis of the clavicle and demonstrated enhancement peripherally in the lesion’s thin walls and within its thin septations. No solid intralesional enhancement was seen. However, a small, nodular, enhancing soft-tissue component extending beyond the cortex was seen in some images. Mild edema, likely reactive, was seen in the adjacent subclavius and anterior distal trapezius.
What is your diagnosis?
- Aneurysmal bone cyst
- Cystic fibrous dysplasia
- Eosinophilic granuloma
- Telangiectatic osteosarcoma
Answer on next page.
Answer: A. Aneurysmal bone cyst
The radiograph of the shoulder revealed an expansile, lytic lesion of the right distal clavicle with no acute displaced fractures and no joint space disruption, which was concerning for either aneurysmal bone cyst (ABC) or telangiectatic osteosarcoma.
Results of a follow-up MRI reinforced the impression from the radiograph that the lesion was likely an ABC, but osteosarcoma could not be ruled out from the imaging alone.
A biopsy of the right distal clavicular mass was taken, the results of which confirmed the diagnosis of ABC. Because the lesion was deemed completely resectable and nonaggressive, the patient was scheduled for en bloc excision with grafting. The surgery was successful with no complications.
At her 1-month follow-up appointment, the patient denied any pain or tingling in the affected region but did have weakness and limitation of abduction and flexion of the right arm. Although she was disappointed to miss a wrestling season, she is currently involved in regular physical therapy with the hope of returning to the sport in the future.
Discussion
Aneurysmal bone cysts were first identified as distinct pathologic and clinical entities by Jaffe and Lichtenstein in 1942.1-3 Current evidence suggests that ABCs account for 1% to 3% of all primary bone tumors but 8% to 10% of bone tumors in patients younger than 20 years.4-6 Overall, they occur in approximately 1 in 300,000 individuals, appear significantly more commonly within the first 2 decades of life (average age, 11-13 years), and have no gender predominance.4-9
The long bones and spinal column are affected in more than 80% of cases, with the femur, tibia, humerus, spine, and pelvis accounting for the vast majority of lesions.3-9 In a study of 411 children with primary ABC, the femur (22%), tibia (17%), spine (15%), humerus (10%), pelvis (9%), and fibula (9%) were the most common locations.18 The clavicle is a relatively rare site for this bone tumor.3-5
Diagnosis is made through the use of CT, MRI, and biopsy. Pathologically, they appear as expanding osteolytic lesions consisting of blood-filled spaces of variable size separated by connective-tissue septa containing trabeculae or osteoid tissue and osteoclast giant cells.1,2,7 Inactive cysts have a complete periosteal shell with defined sclerotic bone limits. 18 Active cysts have an incomplete periosteal shell and defined bone limits.10 Their active or aggressive nature requires either curettage with a considerable recurrence rate or a radical segmental excision necessitating complex reconstructive surgery.10
Special care must be taken to distinguish ABCs from necrotic, extremely vascular telangiectatic osteosarcomas, which frequently metastasize.5,6 The standard treatment is curettage (70%-90% local control rate), but en bloc excision, amputation, radiation therapy, embolization, and cryosurgery may also be considered based on location, radiographic appearance, and biopsy results.11-16 The data suggest that 5% to 20% of ABCs are locally controlled with excision with grafting, and most patients have high functional ratings 2 years postsurgery. 2,5,15-17
References:
- Clough JR, Price CHG. Aneurysmal bone cysts: review of twelve cases. J Bone Joint Surg Br. 1968;50(1):116-127.
- Lichtenstein L. Aneurysmal bone cyst: a pathological entity commonly mistaken for giant‐cell tumor and occasionally for hemangioma and osteogenic sarcoma. Cancer. 1950;3(2):279-289.
- Kransdorf MJ, Sweet DE. Aneurysmal bone cyst: concept, controversy, clinical presentation, and imaging. AJR Am J Roentgenol. 1995;164(3):573-580.
- Yashavntha KC, Nalini KB, Menon J, Patro DK. Aneurysmal bone cyst of medial end of clavicle in a child, a rare case report. Indian J Surg Oncol. 2014;5(2):158-160.
- Mankin HJ, Hornicek FJ, Ortiz-Cruz E, Villafuerte J, Gebhardt MC. Aneurysmal bone cyst: a review of 150 patients. J Clin Oncol. 2005;23(27):6756-6762.
- Mendenhall WM, Zlotecki RA, Gibbs CP, Reith JD, Scarborough MT, Mendenhall NP.. Aneurysmal bone cyst. Am J Clin Oncol. 2006;29(3):311-315.
- Zehetgruber H, Bittner B, Gruber D, et al. Prevalence of aneurysmal and solitary bone cysts in young patients. Clin Orthop Relat Res. 2005;439:136-143.
- Leithner A, Windhager R, Lang S, Haas OA, Kainberger F, Kotz R. Aneurysmal bone cyst: a population based epidemiologic study and literature review. Clin Orthop Relat Res. 1999;363:176-179.
- Cottalorda J, Kohler R, Sales de Gauzy J, et al. Epidemiology of aneurysmal bone cyst in children: a multicenter study and literature review. J Pediatr Orthop B. 2004;13(6):389-394.
- Louahem D, Kouyoumdjian P, Ghanem I, et al. Active aneurysmal bone cysts in children: possible evolution after biopsy. J Child Orthop. 2012;6(4):333-338
- Koskinen EV, Visuri TI, Holmström T, Roukkula MA. Aneurysmal bone cyst: evaluation of resection and of curettage in 20 cases. Clin Orthop Relat Res. 1976;118: 136-146.
- Marcove RC, Sheth DS, Takemoto S, Healey JH. The treatment of aneurysmal bone cyst. Clin Orthop Relat Res. 1995;311:157-163.
- Amendola L, Simonetti L, Simoes CE, Bandiera S, De Iure F, Boriani S. Aneurysmal bone cyst of the mobile spine: the therapeutic role of embolization. Eur Spine J. 2013;22(3):533-541.
- De Cristofaro R, Biagini R, Boriani S, et al. Selective arterial embolization in the treatment of aneurysmal bone cyst and angioma of bone. Skeletal Radiol. 1992;21(8):523-527.
- Schreuder HWB, Veth RPH, Pruszczynski M, Lemmens JAM, Koops HS, Molenaar WM. Aneurysmal bone cysts treated by curettage, cryotherapy and bone grafting. J Bone Joint Surg Br. 1997;79(1):20-25.
- Dormans JP, Hanna BG, Johnston DR, Khurana JS. Surgical treatment and recurrence rate of aneurysmal bone cysts in children. Clin Orthop Relat Res. 2004;421:205-211.
- Vergel De Dios AM, Bond JR, Shives TC, McLeod RA, Unni KK. Aneurysmal bone cyst. A clinicopathologic study of 238 cases. Cancer. 1992;69(12):2921-2931.