CASE REPORT Year : 2022  |  Volume : 30  |  Issue : 2  |  Page : 47-49

A rare tumor at an unusual age: Pediatric bizarre parosteal osteochondromatous proliferation nora's lesion of the phalanx

Murat Celik1, Murat Kara1, Hatice Unverdi2, Ugur Kocer1
1 Department of Plastic Reconstructive and Aesthetic Surgery, Ankara Training and Research Hospital, Ankara, Turkey
2 Department of Pathology, Ankara Training and Research Hospital, Ankara, Turkey

Date of Submission06-Nov-2021Date of Acceptance20-Dec-2021Date of Web Publication23-Mar-2022

Correspondence Address:
Dr. Murat Celik
Hacettepe, Ulucanlar Cd. No: 89, Altındağ 06230, Ankara
Turkey

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/tjps.tjps_60_21

Bizarre parosteal osteochondromatous proliferation (BPOP) is a rare lesion that usually arises in short bones of the hand and feet. It is typically seen in the third and fourth decades, seldom seen in the pediatric age group. A 6-year-old girl was presented with swelling over the ring finger of the left hand. The radiograph showed ectopic calcification and periosteal reaction in the ring finger. Computed tomography was performed, and a BPOP diagnosis was made on the preoperative finding. Excision of the lesion with overlying pseudocapsule and decortication was performed with a piezoelectric device. The patient is currently in a routine follow-up without recurrence. There is not enough data for treatment and follow-up BPOP in pediatric patients. The present case would contribute to the literature in the management of this rare presentation.

Keywords: Bizarre parosteal osteochondromatous proliferation, bone tumors, hand tumors, Nora's lesion, piezosurgery

How to cite this article:
Celik M, Kara M, Unverdi H, Kocer U. A rare tumor at an unusual age: Pediatric bizarre parosteal osteochondromatous proliferation nora's lesion of the phalanx. Turk J Plast Surg 2022;30:47-9
How to cite this URL:
Celik M, Kara M, Unverdi H, Kocer U. A rare tumor at an unusual age: Pediatric bizarre parosteal osteochondromatous proliferation nora's lesion of the phalanx. Turk J Plast Surg [serial online] 2022 [cited 2022 Mar 23];30:47-9. Available from: http://www.turkjplastsurg.org/text.asp?2022/30/2/47/340455   Introduction 

Bizarre parosteal osteochondromatous proliferation (BPOP) is a rare osteocartilaginous lesion first described by Nora et al. in 1983.[1] It is an exophytic growth consisting of the bone, cartilage, and fibrous tissue.[2] It is a benign lesion, and there is no evidence for malignant transformation in the literature.[3] It grows rapidly, and recurrence rates are reported up to 55% of the cases following the surgical excision.[4]

In the literature, around 200 BPOP cases have been reported up to date.[5] Besides this infrequency, most phalangeal lesions in the literature have been described in adults within the second and third decades of life. The incidence of phalangeal BPOP is quite rare in the pediatric age group, especially in the first decade of life.[5] At this point, the present study aims to report a pediatric patient with BPOP in the phalanx, which would contribute to the literature in the treatment and follow-up of this rare clinical condition. Furthermore, the excision of the lesion is performed by a piezoelectric device, which has rarely been used in hand surgery.

  Case Report 

A 6-year-old girl with a 4-month history of swelling on the ring finger of her left hand was admitted to the plastic surgery clinic. In the examination, a painless, rigid, nonmobile swelling of 1 cm × 1 cm in size was palpable on the ulnar aspect of the ring finger, at the level of the middle phalanx [Figure 1]a and [Figure 1]b. There was no pain or restriction with the motions in any joints of the affected finger or hand. The sensory examination of the affected finger was intact. There was no history of trauma and any other swelling in the body.

Figure 1: Preoperative view of the finger and radiological imaging. (a). Lateral view, (b) Anteroposterior view, (c) direct radiographic view, (d) computed tomography view. (orange arrows indicate the lesions)

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Radiographs revealed ectopic calcifications in the ulnar aspect of the middle phalanx [Figure 1]c. The underlying cortex of the bone appeared slightly irregular without a clear margin. In the computed tomography (CT) imaging, a 6 mm × 3 mm exophytic lesion with an intact underlying bony medullary cavity was observed that was compatible with BPOP [Figure 1]d. An excisional biopsy of the lesion was planned for histopathologic diagnosis.

Under general anesthesia and finger tourniquet, the lesion was accessed through a dorsolateral skin incision and splitting of the terminal tendon. En bloc excision of the lesion, including overlying pseudocapsule, was performed with a piezoelectric device (Arteotomy OP-1, Bonart Co. Ltd., New Taipei City, Taiwan). Following excision of the lesion, underlying periosteal and irregular cortical areas were shaved to remove the possible tumoral remnants. After deflating the tourniquet, hemostasis was performed, and the operation was completed with the closure of the skin. The hand and forearm were immobilized in the functional position with splinting. The excised specimen was prepared for histopathologic examination. In the pathological assessment, the lesion was composed of cartilage and bone tissue, consisting of atypical chondrocytes in patches that were compatible with the diagnosis of BPOP [Figure 2].

Figure 2: Pathological view of the specimen (H and E, ×100). Red arrows demonstrate the atypical chondrocytes

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At the end of the postoperative 1st month, extremity splinting was terminated; the patient was consulted to physiotherapy for rehabilitation and pediatric oncology for medical follow-up. In the 7th month's physical examination, motor and sensory examinations of the affected finger were normal, and there was no remarkable pain. The control radiography during the 7th month showed proper bone healing without a sign of recurrence [Figure 3].

Figure 3: Postoperative 7th-month view of the finger and radiographs. Arrows demonstrate surgical site. (a) Anteroposterior view (b and c) direct radiographic views (oblique-anteroposterior views)

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  Discussion 

BPOP could be seen in most of the bones in the body, but it most commonly presents as a bony mass in the short bones of the hands and feet.[6] The involvement of the phalanges is common and is mostly seen in the proximal phalanges.[4]

It is a benign lesion; nevertheless, it should be differentiated from malignant lesions such as parosteal osteosarcoma, conventional osteosarcoma, and chondrosarcoma and benign lesions such as florid reactive periostitis, osteochondroma, periosteal chondroma, and myositis ossificans.[7] Radiographs with the typical appearance that correlate with clinical findings enough to diagnose BPOP.[8] In radiographs, lesions are seen arising from the cortex without the involvement of the medullary cavity. CT and magnetic resonance imaging imaginings can be helpful when radiographs are not sufficient.

It has a recurrence rate reported up to 55% of the cases following the surgical resection.[4] Despite the high recurrence rate, no metastasis, death, or systemic disease is reported in patients with BPOP.[4] Due to high recurrence rates, follow-up of the patient every 6 months for the postoperative 1st year and annual examinations for 2 years is recommended in the literature.[8] Due to the high recurrence rates of BPOP, some authors proposed follow-up should undergo in cancer centers.[2]

Michelsen et al. reported that following the excision of the lesion, decorticating small areas of the bone and excision surrounding abnormal periosteal tissue which may include tumoral remnants reduces the recurrence while not compromising the stability of the bone.[8] Although there are no margin distance recommendations in the literature, Gruber et al. reported that wide resection could lead to segmental amputation, which is not recommended as a first step surgical treatment.[2]

In the present case, excision of the lesion and decortication was done by using the piezoelectric device. Some studies reported that piezoelectric surgery provides lower blood losses and improved bone healing than the conventional method. Therefore, they have suggested the utilization of piezoelectric surgery for the osteotomy of the small bones.[9] Piezoelectric surgery provides a smooth osteotomy line, and damage to the soft tissue is minimal.[9] The osteotomy of the phalanx was easy to control, practical, and the osteotomy line was smooth. Furthermore, minimal blood loss and soft-tissue-friendly osteotomy were achieved in a small surgical area. In the literature, although the utility of the piezoelectric device has been reported in craniomaxillofacial and orthognathic surgery, limited studies have reported the utility of piezo-assisted surgery in hand surgery.[10]

In conclusion, BPOP is a rare disease and usually affects adults and is even less common in the pediatric population. Furthermore, the present study confirms the feasibility of the piezoelectric device in hand surgery, considering its advantages. On the other hand, further studies with large patient series with long-term follow-up are necessary to improve treatment protocol and reveal the surgical results and recurrence rates.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 

  References 
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    2.Gruber G, Giessauf C, Leithner A, Zacherl M, Clar H, Bodo K, et al. Bizarre parosteal osteochondromatous proliferation (Nora lesion): A report of 3 cases and a review of the literature. Can J Surg 2008;51:486-9.  
    3.Zambrano E, Nosé V, Perez-Atayde AR, Gebhardt M, Hresko MT, Kleinman P, et al. Distinct chromosomal rearrangements in subungual (Dupuytren) exostosis and bizarre parosteal osteochondromatous proliferation (Nora lesion). Am J Surg Pathol 2004;28:1033-9.  
    4.Meneses MF, Unni KK, Swee RG. Bizarre parosteal osteochondromatous proliferation of bone (Nora's lesion). Am J Surg Pathol 1993;17:691-7.  
    5.Kumar A, Khan SA, Sampath Kumar V, Sharma MC. Bizarre parosteal osteochondromatous proliferation (Nora's lesion) of phalanx in a child. BMJ Case Rep 2014;2014:bcr2013201714.  
    6.Abramovici L, Steiner GC. Bizarre parosteal osteochondromatous proliferation (Nora's lesion): A retrospective study of 12 cases, 2 arising in long bones. Hum Pathol 2002;33:1205-10.  
    7.Smith NC, Ellis AM, McCarthy S, McNaught P. Bizarre parosteal osteochondromatous proliferation: A review of seven cases. Aust N Z J Surg 1996;66:694-7.  
    8.Michelsen H, Abramovici L, Steiner G, Posner MA. Bizarre parosteal osteochondromatous proliferation (Nora's lesion) in the hand. J Hand Surg Am 2004;29:520-5.  
    9.Abella F, de Ribot J, Doria G, Duran-Sindreu F, Roig M. Applications of piezoelectric surgery in endodontic surgery: A literature review. J Endod 2014;40:325-32.  
    10.Itro A, Lupo G, Carotenuto A, Filipi M, Cocozza E, Marra A. Benefits of piezoelectric surgery in oral and maxillofacial surgery. Review of literature. Minerva Stomatol 2012;61:213-24.  
    
  [Figure 1], [Figure 2], [Figure 3]