Costal exostosis with fungation: A rare presentation

Hashmukh Shantilal Varma; Sachin Upadhyay

doi:10.4103/0975-7341.134034

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CASE REPORT

Year : 2014 | Volume : 7 | Issue : 1 | Page : 97-100

Costal exostosis with fungation: A rare presentation

Hashmukh Shantilal Varma, Sachin Upadhyay
Department of Orthopedics, Traumatology and Rehabilitation, Netaji Subhash Chandra Bose Medical College, Jabalpur, Madhya Pradesh, India

Date of Web Publication

6-Jun-2014

Correspondence Address:
Sachin Upadhyay
Department of Orthopedics, Traumatology and Rehabilitation, Netaji Subhash Chandra Bose Medical College, Jabalpur, Madhya Pradesh
India

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/0975-7341.134034

Abstract

Costal exostosis occurs either sporadically or as a manifestation of hereditary multiple osteochondromas (HMOs), a disorder that is inherited in an autosomal dominant manner. Costal exostosis may cause chest pain and, on rare occasions, result in intra-thoracic complications. We report a case of a 15-year-old boy with exostosis in the right anterior fifth rib, who presented with protruded painful skin lesions that had ulcerated over the last one month. He previously received a diagnosis of multiple exostoses. There was no evidence of a malignant change on histopathological analysis. Enbloc excision was done and there has been no recurrence to date. To the best of our knowledge, the present case is the only report of costal exostosis presenting as an extrathoracic chest wall ulcerating mass. The present report illustrates this rare clinical scenario and reviews the relevant literature.

Keywords: Chest pain, costal exostosis, exostosis, hereditary multiple osteochondromas

How to cite this article:
Varma HS, Upadhyay S. Costal exostosis with fungation: A rare presentation. J Orthop Traumatol Rehabil 2014;7:97-100

How to cite this URL:
Varma HS, Upadhyay S. Costal exostosis with fungation: A rare presentation. J Orthop Traumatol Rehabil [serial online] 2014 [cited 2020 Oct 27];7:97-100. Available from: https://www.jotr.in/text.asp?2014/7/1/97/134034

Introduction

Osteochondroma is the most common benign bone tumor and usually occurs in the metaphyseal region of the long bones. ^{[1],[2],[3],[4],[5]} Exostoses that affect 'almost every bone in the body' were first described by John Hunter in his lecture on the principles of surgery in 1786. ^[6] Exostosis is broadly classified into 'solitary' or 'multiple'. The majority of these tumors present as solitary, nonhereditary lesions. Approximately 10-15% of osteochondromas occur in the context of hereditary multiple exostoses (HME), an autosomal dominant disorder characterized by multiple exostoses ^[7] that frequently involve multiple flat bones. Costal exostosis may originate in the region of the costochondral junction or near the vertebral end. Rib exostosis may cause considerable chest wall deformity with spreading of ribs. In addition, it also causes intrathoracic and extrathoracic soft tissue masses. Costal exostosis is an unusual condition; usually asymptomatic, but intrathoracic complications have been reported. ^[8],[9],[10] The complications related to solitary osteochondroma are often the result of mechanical interference with the adjoining anatomic structures. ^[10] We report a rare case of extrathoracic costal exostosis presenting as a stony, hard, painful, ulcerated mass and discuss the indications for surgical intervention.

Case report

A 15-year-old boy was admitted to our hospital because of right-side chest pain owing to an ulcerated mass [Figure 1]. He was also complaining of pain attributed to multiple bony swellings over the gluteal region, right shoulder, around the knee on the right side, right ankle, left ankle, around the knee on the left side, and left humerus [Figure 2]. He had no history of recent trauma. He had previously received a diagnosis of multiple exostoses. Chest computed tomography (CT) revealed an exostosis from the outer aspect of anterior right fifth ribs with spurs pointing directly outward to the thoracic cavity [Figure 3]. An ulcerated exophytic mass was present in the chest region [Figure 4]. Although not compromising the respiratory function, it was painful and fungating on the skin and also cosmetically it was not accepted by the patient and the patient's parent. An ulcerative/fungating mass is usually more worrisome for surgeons, as an ulcerated mass is a potential site for squamous cell carcinoma. Surgical treatment was recommended. A bony exostosis was found arising from the anterior aspect of the anterior portion of the fifth rib. A 3-cm segment of the right fifth rib including the exostosis was resected along with the skin margin [Figure 5]a and b. The specimen [Figure 6] was sent for histopathological analysis. A pathological evaluation of the specimen revealed cartilage, bone, and fibroconnective tissue, suggestive of exostosis [Figure 7]. There was no evidence of malignancy. The skin margins were negative for squamous cell carcinoma. The patient had an uneventful recovery; he was discharged on the fifth postoperative day.

Figure 1

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Figure 2:

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Figure 3 (a, b): A chest CT scan showing an exostosis originating from the outer aspect of the anterior right fifth rib, with a bony spicule projecting outward

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Figure 4: Ulcerated mass

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Figure 5:

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Figure 6: Specimen (Enbloc Exostosis with 3cm of rib, with critical skin margins)

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Figure 7: Histopathological image; (10x.H and E stain) the cartilaginous cells are arranged in an orderly fashion with maturation into the trabecular bone. There were abundant chondrocytes without nuclear atypia. The inter-trabecular spaces contain hematopoietic marrow

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Discussion

Although rare in hereditary multiple exostoses, costal exostoses are notorious for causing serious complications. ^{[11],[12],[13]} Movement of the chest wall (lateral movements due to cardiac pulsations and longitudinal movements by the diaphragm during respiration) and long continuous friction between the costal exostosis and viscera are the two etiological mechanisms ^[14] that result in injury to the pleura, ^[15] diaphragm, ^[16] and pericardium, ^[17] causing hemothorax, pneumothorax, and hemopneumothorax. To visualize costal exostosis on a standard chest x-ray film is an arduous task. A chest CT scan is more definitive in establishing the diagnosis and confirming the associated intrathoracic abnormalities. A critical review of the published literature ^{[11],[12],[13],[14],[15],[16],[17]} shows that most studies have reported complications in the context of intrathoracic exostosis. Furthermore, the author speculated that extrathoracic complications due to costal exostosis remain unknown, mainly because of the scarcity of peer-reviewed evidence. To the best of our knowledge, this is probably the first documented description of extrathoracic location - related complications of costal exostosis.

In our patient, the sharp margins of extrathoracic exostosis lacerated the overlying skin during respiratory movements. Furthermore, the growth of the tumor forced the lesion to bulge and protrude from the skin, which was complicated by ulceration/fungation. In the present case complete excision was the aim. Besides cosmetic disfigurement and pain, the primary indication for surgical excision was the fear of developing a malignant growth in view of the ulcerating margins. Fortunately, on histopathological analysis the critical margins of the specimen (ulcerated skin) were negative for squamous cell carcinoma.

Previous reports ^{[11],[12],[13],[14],[15],[16],[17]} advocated surgical treatment of costal exostosis, whether asymptomatic ^[8] or symptomatic, as these lesions may present acutely as a chronic or life-threatening bleeding complication in the form of pneumonitis and empyema. ^[18] The authors also recommended the surgical removal of the exostosis in selected patients, as in the case of the patient described in the present report, wherein, a potential risk of squamous cell carcinoma was suspected in the context of the ulcerated skin. ^[19],[20]

Conclusion

Patients with diaphyseal aclasis should be examined using a CT, to detect the presence of intra- or extrathoracic costal exostosis, and if detected, should promptly be treated. In both instances (intrathoracic or extrathoracic), if the exostosis is symptomatic, we would recommend surgical removal, to avoid the possibility of serious consequences. In the case of asymptomatic exostosis, abstention would be recommended.

References

1.	Saglik Y, Altay M, Unai VS, Basari K, Yildiz Y. Manifestations and management of osteochondromas: Aretrospective analysis of 382 patients. Acta Orthop Belg 2006;72:748-55.
2.	Porter DE, Simpson AH. The neoplastic pathogenesis of solitary and multiple osteochondromas. J Pathol 1999;188:119-25.
3.	Khurana J, Abdul-Karim F, Bovée JV. Osteochondroma. In: World Health Organization Classification of tumours. Pathology and genetics of tumours of soft tissue and bone. In: Fletcher CD, Unni KK, Mertens F, editors. Lyon, France: International Agency for Research on Cancer (IARC); 2002. p. 234-6.
4.	Bovée JV, Hogendoorn PC. Multiple osteochondromas. World Health Organization Classification of tumours. Pathology and genetics of tumours of soft tissue and bone. In: Fletcher CD, Unni KK, Mertens F, editors. Lyon, France: International Agency for Research on Cancer (IARC) 2002. p. 360-2.
5.	Canella P, Gardin F, Borriani S. Exostosis: Development, evolution and relationship to malignant degeneration. Ital J Orthop Traumatol 1981;7:293-8.
6.	Hunter J: The works of John Hunter, F.R.S. Vol 1. Palmer JF, editor. London: Longman, Rees, Orne, Brown, Green, and Longman; 1835.
7.	Virchow R. Ueber th Entstenhung des Enchondroms und seine Besihungen zur Enchondrosis und Exostosis cartilaginea. Manatsberichte der Koniglichen Preussischen Akademie der Wissenshaften, p. 760, 1876.
8.	Nakano T, Endo S, Tsubochi H, Tetsuka K. Thoracoscopic findings of an asymptomatic solitary costal exostosis: Is surgical intervention required? Interact Cardiovasc Thorac Surg 2012;15: 933-4.
9.	Cowles RA, Rowe DH, Arkovitz MS. Hereditary multiple exostoses of the ribs: An unusual cause of hemothorax and pericardial effusion. J Pediatr Surg 2005;40:1197-200.
10.	Uchida K, Kurihara Y, Sekiguchi S, Doi Y, Matsuda K, Miyanaga M, et al. Spontaneous haemothorax caused by costal exostosis. Eur Respir J 1997;10:735-6.
11.	Castells L, Comas P, Gonsalez A, Vargas V, Guardia J, Gifre L. Case report: Haemothorax in hereditary multiple exostosis. Br J Radiol 1993;66:269-70.
12.	Dendale J, Amram S, Dermakar S, Guilaud R, Lesbros D. A rare complication of multiple exostoses: Hemothorax. Arch Pediatr 1995;2:548-50.
13.	Reynolds JR, Morgan E. Haemothorax caused by a solitary costal exostosis. Thorax 1990;45:68-9.
14.	Imai K, Suga Y, Nagatsuka Y, Usuda J, Ohira T, Kato H, et al. Pneumothorax caused by costal exostosis. Ann Thorac Cardiovasc Surg 2012:1-4 [Epub ahead of print].
15.	Takata K, Suzuki K, Kurosaki Y. Spontaneous hemothorax in hereditary multiple exostosis involving the ribs. Radiat Med 2008;26:39-41.
16.	Simansky DA, Paley M, Werczberger A, Bar Ziv Y, Yellin A. Exostosis of a rib causing laceration of the diaphragm: Diagnosis and management. Ann Thorac Surg 1997;63:856-7.
17.	Buchan KG, Zamvar V, Mandana KM, Nihal E, Kulatilake P. Juxtacardiac costal osteochondroma presenting as recurrent haemothorax. Eur J Cardiothorac Surg 2001;20:208-10.
18.	Hajjar WM, El-Medany YM, Essa MA, Rafay MA, Ashour MH, Al-Kattan KM. Unusual Presentation of Rib Exostosis. Ann Thorac Surg 2003;75:575-7.
19.	Bernstein SC, Lim KK, Brodland DG, Heidelberg KA. The many faces of squamous cell carcinoma. Dermatol Surg 1996;22:243-54.
20.	Petter G, Haustein UF. Squamous cell carcinoma of the skin - histopathological features and their significance for the clinical outcome. J Eur Acad Dermatol Venereol 1998;11:37-44.