Case Reports in Pathology

Case Reports in Pathology / 2018 / Article

Case Report | Open Access

Volume 2018 |Article ID 5971786 | 7 pages |

Nasal Chondromesenchymal Hamartoma: Rare Case Report in an Elderly Patient and Brief Review of Literature

Academic Editor: Stefan Pambuccian
Received29 May 2018
Accepted24 Sep 2018
Published14 Oct 2018


Hamartomas are considered a mixture of nonneoplastic tissue, which may be indigenous to a different location in the body. As such, they may be epithelial, mesenchymal, or mixed. In the sinonasal region, the following hamartomatous lesions are considered to lie on a spectrum and include respiratory epithelial adenomatoid hamartoma (REAH), chondro-osseous respiratory epithelial adenomatoid hamartoma (COREAH), and nasal chondromesenchymal hamartoma (NCMH). To our knowledge, less than 50 cases of sinonasal hamartomas have been reported in the English literature so far with NCMH being very rare and primarily a tumor in infancy, with only 2 cases reported in individuals older than 16 years of age. We report a highly unusual case of a NCMH in the right maxillary sinus of a 70-year-old female.

1. Case Report

A 70-year-old female presented with a two-year history of slowly growing, nonpainful maxillary sinus mass. She has a history of chronic maxillary sinusitis corresponding to presentation of the mass, with the first episode reported in 2014. Computed tomography (CT) imaging revealed an erosive right maxillary sinus mass (2.5 x 2.1 cm) with bony destruction.

Surgical excision of the right maxillary sinus mass revealed a fragmented, white, vaguely nodular, and whorled lesion. Histological examination revealed fragments of respiratory-type epithelium with focal cystic invagination and associated squamous metaplasia [Figure 1]. The underlying stroma consisted of a variably cellular, benign spindle cell proliferation with an associated background of hyalinization [Figure 2], calcification and ossification [Figure 3], and focal chondroid change [Figure 4] in a vague lobule-like arrangement. Focal areas of aneurysmal and cystic changes [Figure 5] were seen which would provide an explanation for the clinically noted enlargement since hamartomas by definition would be expected to have a much lower rate of growth. The intrinsic slow-growing nature is also supported by the deficit of mitotic activity even in the highly cellular/spindled regions of the lesion (less than 1/10 hpf). Areas with haphazard arrangement of nerve bundles within the collagenous stroma [Figure 6] were also noted. Immunohistochemical stains were positive for SMA [Figures 7(a) and 7(b)] in the spindle cells and negative for CK AE1/AE3, EMA, CD34, Stat6, ERG/FLI-1, Mucin 4, S-100, Sox-10, and desmin [Figure 8]; ruling out perineurioma, solitary fibrous tumor, a vascular neoplasm, Evans tumor, a benign peripheral nerve sheath tumor, or a myogenic neoplasm. The overall findings were suggestive of a hamartomatous lesion, most likely a nasal chondromesenchymal hamartoma. The absence of submucosal glandular proliferation, myxoid stroma, or mucinous metaplasia in the lining epithelium lowers the likelihood of other neoplastic hamartomatous lesions such as COREAH.

2. Discussion

Nasal chondromesenchymal hamartomas are most commonly seen in the nasal cavity of children less than 3 months old, with less common involvement of the paranasal sinuses [2]. As per one review [1], mean age for NCMH was 9.6 years. Review of the English PubMed literature reveals 43 cases [Table 1] of NCMH previously published, with our case being the oldest patient reported, and presenting with a tumor in an unusual location.

SitePertinent InformationStudyYear

5 daysM2 yearsNasal cavity-[2] McDermott1998

12 daysF< 16 monthsNasal cavityIntracranial extension[2] McDermott1998

14 daysM-Nasal cavity
Ethmoid Sinus
Intracranial extension
Residual tumor
[2] McDermott1998

2 monthsM18 monthsNasal cavityIntracranial extension[2] McDermott1998

3 monthsF2 yearsNasal cavity
Ethmoid Sinus
Intracranial extension
Residual tumor
[2] McDermott1998

3 monthsM4 yearsNasal cavity-[2] McDermott1998

7 yearsM-Nasal cavity Sphenoid sinusPPB, multiple recurrences[2] McDermott1998

4 monthsM13 yearsNasal cavityIntracranial extension[3] Kato1999

0 daysM5 yearsNasal cavity
Sphenoiod sinus
Ethmoid sinus
Orbital compression[4] Hsueh2001

9 monthsM9 monthsNasal cavity-[4] Hsueh2001

16 yearsM8 monthsNasal cavity3-month history[5] Alrawi M2003

5 monthsM-Nasal cavityOrbital compression[6] Kim B2004

11 yearsM-Nasal cavity Ethmoid sinus8-month history[7] Norman ES2004

1 yearM-Nasal cavityOrbital extension
Residual tumor
[8] Shet T2004

11 yearsM-Nasal cavity Ethmoid sinus-[9] Ozolek JA2005

17 yearsF-Nasal cavity-[9] Ozolek JA2005

25 yearsM-Nasal cavity Maxillary sinusBilateral NCMH
Intracranial aneurysms
[9] Ozolek JA2005

69 yearsF-Nasal cavity Ethmoid sinus-[9] Ozolek JA2005

11 yearsM2 monthsNasal cavity-[10] Low SE2006

15 yearsF6 monthsNasal cavityBilateral NCMH
[11] Johnson C2007

7 monthsM18 monthsNasal cavityOrbital compression[12] Silkiss RZ2007

12 monthsM-Nasal cavityOrbital compression[13] Finitsis S2009

19 monthsM10 monthsNasal cavityIntracranial, orbital extension[14] Kim JE2009

2 cases previously reported, both with PPB, multiple recurrences[15] Priest JR2010

10 yearsF21 monthsNasal cavityBilateral NCMH
[15] Priest JR2010

11 yearsM4 monthsNasal cavityPPB[15] Priest JR2010

11 yearsM--PPB[16] Behery RE2012

8 yearsM6 monthsSphenoid sinus Ethmoid sinus4-month history[17] Uzomefuna2012

14 yearsM4 yearsNasal cavity Maxillary sinus-[18] Cho YC2013

23 yearsM3 monthsNasal cavity Ethmoid SinusOrbital extension[19] Li GY2013

40 yearsF-Nasal cavity Ethmoid Sinus
Maxillary sinus
Malignant transformation Recurrence[20] Li Y2013

9 monthsF-Nasal cavity
Maxillary sinus
Orbital compression[21] Moon S2014

14 yearsM-Nasal cavityBilateral NCMH
[22] Obidan AA2014

6 weeksF10 monthsNasal cavity-[23] Wang T2014

5 yearsM3 yearsNasal cavity
Ethmoid sinus
4-year history[23] Wang T2014

10 monthsM18 monthsNasal cavity6-month history[24] Lee CH2015

49 yearsM2 years
4 years (phone)
Nasal cavity5-year history[1] Mason AK2015

Systematic review[1] Mason AK2015

5 yearsM-Nasal cavityPrevious rhabdomyosarcoma in remission[25] Avci H2016

13 yearsF12 monthsNasal cavity6-month history[26] Unal A2016

3 yearsM3 yearsNasal cavity-[27] Nakaya M2017

Index. Cases older than 1 year of age at presentation. Bilateral/cases associated with pleuropulmonary blastoma.

Our case would lend support to extending the age range for NCMH and considering it in the differential diagnosis of all sinonasal region tumors, irrespective of age, and location in the head and neck region. Despite primarily being a benign lesion, these tumors can present with areas of necrosis and local destruction, including bony invasion. The tumors can be aggressive appearing on imaging, extending into bony structures, including the cranium and/or the orbital cavity, which should not lead away from the diagnosis of this benign lesion. Detailed CT or preferably MRI prior to surgical excision should be performed.

NCMH has been associated with development of pleuropulmonary blastoma (PPB) during infancy. A recent [28] report highlighted the association of NCMH and PPB with DICER1 mutation and various associated entities such as lung cysts, cystic nephroma, renal sarcoma, Wilms tumor, thyroid hyperplasia, and CNS tumors. NCMH in isolation however is a benign lesion with follow-up in patients up to 16 years after excision, except for one reported case with malignant transformation in the literature [20]. Etiologically, it would make sense that cases in adults, such as ours, represent a tissue response to insult, such as chronic sinusitis rather than an inborn germline error (such as a DICER1 mutation).

Whether the presentation of a NCMH at a later age predisposes to malignant transformation due to the long-standing nature of the lesion is up for debate. It could represent a somatic DICER1 mutation rather than a germline mutation, causing the hamartoma to form later in age. Longer follow-up results from the adult cases and routine genetic testing in all NCMH will help provide an answer to these questions.

3. Conclusion

We report an unusual case of NCMH eroding the right maxillary sinus of a 70-year-old female. Although, NCMH is a rare entity with predilection for pediatric age groups, it is important to consider NCMH in the differential diagnosis of nasal/sinonasal masses in adult patients in order to avoid diagnostic errors.

Conflicts of Interest

The authors declare that there are no conflicts of interest regarding the publication of this paper.


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Copyright © 2018 Kanish Mirchia and Rana Naous. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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