360

ABSTRACT

Nasal Chondromesenchymal Hamartoma (NCMH) was first described by McDermott et al., in 1998. It is a very uncommon benign mass arising in the sinonasal area. Forty-eight cases of NCMH have been reported including 6 cases of adult presentation. It mostly occurs in younger age group with median age of 7 years. In this article, we report a rare case of Nasal Chondromesenchymal hamartoma (NCMH) in 17-years-old male who presented to us with persistent headache. The tumor was surgically excised in toto by using lateral rhinotomy approach with no recurrence.KEY WORDS: Nasal Chondromesenchymal Hamartoma, Sinonasal, Lateral Rhinotomy.

HOW TO CITE THIS:Ahmed J, Saqulain G, Shahzad J. Nasal Chondromesenchymal Hamartoma – A Rare Case Report. Isra Med J. 2017; 9(5): 360-62.

This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 International License (http://creativecommons.org/licenses/by-nc/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

ISRA MEDICAL JOURNAL | Volume 9 - Issue 5 | Sep - Oct 2017

increased insidiously causing facial disfigurement, till 6 months before presentation, when patient developed persistent throbbing headache. Examination revealed a bony swelling on nasal bridge and medial to the left orbit with tele-canthus (Figure - 1). Anterior rhinoscopy, revealed a firm mass on the left side, pushing the septum towards the right. The differential diagnosis considered were osteoma, chondroma and fibrous dysplasia.CT scan revealed a well-defined rounded osseous and soft tissue density lesion involving the left ethmoid sinuses and left nasal cavity measuring 4.8×4.0×4.3 cm with irregular ossification (Figure - 2). It had a broad-based attachment to the ethmoid with expansion of the left nasal cavity with septal deviation towards the right and medial wall of left maxilla and orbit towards left. Intranasal punch biopsy revealed a Chondroma. Surgical removal under general anesthesia through a lateral rhinotomy approach with extended Moure's incision revealed a well-defined mass with attachment to ethmoids including cribriform plate, which was freed to remove the mass in toto. It was 6x5x4 cm, well encapsulated, hard and giving a gritty feel (Figure - 3). The Cavity was packed trans-nasally and wound closed in layers. Post operatively patient suffered from conjunctivitis, excessive crusting and epiphora in the first month, which gradually improved. Follow up was maintained for 2 years with no recurrence. Histo-pathology (Figure - 4) revealed well delineated nodules of hyaline cartilage within the stroma showing variable cellularity with giant cells of osteoclastic appearance. Stroma also showed erythrocytes filled spaces. No significant pleomorphism and mitotic activity seen, however some nodules were ossified. Lining epithelium was normal respiratory epithelium. Thus, the diagnosis of Nasal Chondromesenchymal Hamartoma was made.

INTRODUCTION

Hamartomas are very rare sinonasal lesions, usually identified 1in children . Nasal Chondromesenchymal Hamartoma (NCMH)

2is a term coined by McDermott et al., in 1998 . It is a very uncommon benign sinonasal pathology of unknown etiology with characteristic clinicopathological features. 48 cases have

3been reported in literature . NCMH's have a mixed morphology including mesenchymal and cartilaginous components. It

2,4usually occurs in children with a median age of 7 years .

Although usually asymptomatic, symptoms when occur depends on the tumor size and location and commonly include nasal symptoms like nasal obstruction, rhinorrhea, epistaxis, anosmia, headache; ophthalmic symptoms like epiphora, diplopia, proptosis, decreased vision, hypotropia and intra-oral

1,3symptoms .We report a rare case of NCMH of a 17-year-male, the first case from Pakistan, with a large mass in the left nasal cavity with tele-canthus which presented to us due to persistent headache.

CASE REPORT

A 17-years-old male, presented in ENT outpatients with swelling over the nasal bridge and nasal obstruction since birth which

1 2 3Jawwad Ahmed , Ghulam Saqulain , Junaid Shahzad ,

CASE REPORT

Nasal Chondromesenchymal Hamartoma – A Rare Case Report

1. Associate Surgeon 2. Head of Department 3. Postgraduate Trainee Department of ENT Capital Hospital, Islamabad, Pakistan

Correspondence to:Jawwad AhmadAssociate Surgeon of ENT Capital Hospital, Islamabad, PakistanEmail drjawwad@hotmail.com:

Received for Publication: 20-05-17Accepted for Publication: 10-08-17

ISRA MEDICAL JOURNAL | Volume 9 - Issue 5 | Sep - Oct 2017Jawwad Ahmad et al.

361

Presentation depends on size and location of the tumor. Usually it is asymptomatic, but may present with nasal obstruction, rhinorrhea, epistaxis, anosmia, headache, epiphora, diplopia,

1,10-12proptosis, decreased vision, hypotropia, and intra-oral 3symptoms .

NCMH have a mixed morphology. Mesenchymal hamartomas 4being more common . Aetiology and origin of NCMH is not well

understood. It may be caused by an underlying genetic predisposition. Some consider it a variant of chest wall

13mesenchymal hamartoma , others link it to blastoma of 10,14pleuropulmonry (PPB) origin . Microscopy reveals a variety of

DISCUSSION

NCMH is uncommon, slow growing, benign sinonasal pathology 3with 48 reported cases including 6 cases of adult presentation .

This is the first case of NCMH being reported from Pakistan. This lesion is mostly reported in younger children (median age 7

2,4years) . This is locally destructive with only one reported case 6of malignant transformation . These lesions have delayed

presentation being slow growing and sometimes asymptomatic 6-9in infancy , which was true in our case. It grew insidiously, over

17 years, and ultimately presented with persistent headache.

FIGURE-1: PREOPERATIVE CLINICAL PHOTOGRAPHSHOWING A SWELLING AND WIDENING INVOLVING

NASAL BRIDGE UP TO LEFT ORBIT WITH TELE CANTHUS

FIGURE-3: GROSS PHOTOGRAPH SHOWING 6 X 5x4 CMMASS WHICH HAD AN IRREGULAR HARD SURFACE

AND GIVING A GRITTY FEELING.

FIGURE 2: CT SCAN A: AXIAL VIEW SHOWING A WELL-DEFINED ROUNDED OSSEOUS AND SOFT TISSUE DENSITYLESION WITH IRREGULAR OSSIFICATION IN LEFT NASAL

CAVITY WITH SEPTAL DEVIATION TO RIGHT SIDE.B: CORONAL VIEW SHOWING LESION WITH A BROAD-

BASED ATTACHMENT TO THE ETHMOIDS.

FIGURE-4: PHOTOMIROGRAPH SHOWING MESENCHYMALCOMPONENTS IN THE FORM OF FOLLICLES.

THE BACKGROUND IS MYXOID WITH A POPULATION OFRELATIVELY BLAND AND COMPACT SPINDLE CELLS WITH

QUITE VARIABLE CELLULARITY. (H&E: 100X)

ISRA MEDICAL JOURNAL | Volume 9 - Issue 5 | Sep - Oct 2017Jawwad Ahmad et al.

362

Chokkalingam PG. Nasal Chondromesenchymal Hamartoma (NCMH): a systematic review of the literature with a new case report. J Otolaryngol Head Neck Surg. 2015;44(1):28.

4. Hsueh C, Hsueh S, Gonzalez-Crussi F, Lee T, Su J. Nasal Chondromesenchymal hamartoma in children: report of 2 cases with review of the literature. Arch Pathol Lab Med. 2001;125(3):400-03

5. Tabatabaei A, Naeimi M, Ghanadan AR, Ayatollahi H. Nasal chondromesenchymal hamartoma in an adolescent: a report of case and review of literature. Iran J Otorhinolaryngol. 2006;17(4):19-22

6. Li Y, Yang QX, Tian XT, Li B, Li Z. Malignant transformation of nasal chondromesenchymal hamartoma in adult: a case report and review of the literature. Histol Histopathol 2013; 28(3): 337-44

7. Ozolek JA, Carrau R, Barnes EL, Hunt JL. Nasal chondromesenchymal hamartoma in older children and adults: series and immune histochemical analysis. Arch Pathol Lab Med. 2005;129 (11):1444–50.

8. Li GY, Fan B, Jiao YY. Endonasal endoscopy for removing nasal chondromesenchymal hamartoma extending from the lacr imal sac reg ion. Can J Ophtha lmol . 2013;48(2):22–23.

9. Alrawi M, McDermott M, Orr D, Russell J. Nasal chondromesynchymal hamartoma presenting in an a d o l e s c e nt . I n t J Pe d i at r O to r h i n o l a r y n go l . 2003;67(6):669–72.

10. Finitsis S, Giavroglou C, Potsi S, Constantinidis I, Mpal tatz id i s A , Rachov i tsas D, et a l . Nasa l chondromesenchymal hamartoma in a child. Cardiovasc Intervent Radiol 2008;32(3):593-97

11. Silkiss RZ, Mudvari SS, Shetlar D. Ophthalmologic presentation of nasal chondromesenchymal hamartoma in an infant. Ophthal Plast Reconstr Surg 2007;23(3):243-44

12. Norman ES , Bergman S , Trup iano JK . Nasa l chondromesenchymal hamartoma: report of a case and review of the literature. Pediatr Dev Pathol 2004; 7(5): 517-20

13. Kim B1, Park SH, Min HS, Rhee JS, Wang KC. Nasal chondromesenchymal hamartoma of infancy clinically mimicking meningoencephalocele. Pediatr Neurosurg. 2004;40(3):136-40.

14. Obidan AA1, Ashoor MM. Nasal chondromesenchymal hamartoma in an adolescent with pleuropulmonary blastoma. Saudi Med J. 2014;35(8):876-78.

15. Johnson C, Nagaraj U, Esguerra J, Wasdahl D, Wurzbach D. Nasal chondromesenchymal hamartoma: radiographic and histopathologic analysis of a rare pediatric tumor. Pediatr Radiol 2007;37(1):101-104

16. Shet T, Borges A, Nair C, Desai S, Mistry R. Two unusual lesions in the nasal cavity of infants - a nasal chondromesenchymal hamartoma and an aneurysmal bone cyst like lesion. More closely related than we think? Int J Pediatr Otorhinolaryngol 2004; 68(3): 359-64.

mesenchymal components in follicles including irregular islands of mature and immature hyaline cartilaginous components with bi-nucleated chondrocytes. Stroma shows well delineated cartilaginous islands with myxoid background with a population of relatively bland and compact spindle cells with variable

1, 4cellularity . Immune reactivity may show positive for smooth muscle actin, KP-1, Leu-7, S-100, vimentin, and negative for

1, 4epithelial membrane antigen, cytokeratin, and desmin .CT Scan, reveals an un-capsulated lesion with some cystic areas

1,4,10,15and calcifications . This was true in our case. On MRI, it is homogeneously iso-intense to the cerebral cortex on T1 images and heterogeneously hyper-intense on T2. This is attributed to

1abundance of myxoid stroma with less number of cells .4,10 Complete Surgical Excision is the mainstay of treatment and

approach depends on the lesion's site and size. Endoscopic surgery can be useful for tumors restricted to the nasal cavity, however, infiltrative nature, makes it difficult to get a tumor free

15margin . Tumor recurrence occurs, especially in cases with inadequate resections or where small deposits of tumor are left

5,15behind . We were successful in doing a complete resection of tumor without any recurrence. Radiotherapy and combination chemotherapy are other modes of treatment reported by

16Shet.et.al . Therefore, Careful detailed preoperative assessment including imaging is essential to see the extent of lesion and status of the neighboring structures. Magnetic resonance imaging may be required in aggressive cases with suspicion of malignancy and to see involvement of important neighboring structures. Complete Surgical Excision is the mainstay of treatment.

CONCLUSION

NCMH may be rarely encountered when dealing with masses in the sinonasal area and should be considered in differential diagnosis.

Contribution of author:Ahmed J: Manuscript writingSaqulain G: Literature ReviewShahzad J: Data Collection, Histopathology slides

Disclaimer: None.Conflict of Interest: None.Source of Funding: None.

REFERENCES

1. Kim JE, Kim HJ, Kim JH, Ko YH, Chung SK. Nasal chondromesenchymal hamartoma: CT and MR imaging findings. Korean J Radiol. 2009; 10 (4): 416-19.

2. McDermott MB, Ponder TB, Dehner LP. Nasal chondromesenchymal hamartoma: an upper respiratory tract analogue of the chest wall mesenchymal hamartoma. Am J Surg Pathol.1998;22(4):425-33

3. Mason KA, Navaratnam A, Theodorakopoulou E,