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

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.

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 longstanding 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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Kanish Mirchia (iD) and Rana Naous (iD)

Department of Pathology, SUNY Upstate Medical University, Syracuse, NY 13210, USA

Correspondence should be addressed to Kanish Mirchia;

Received 29 May 2018; Accepted 24 September 2018; Published 14 October 2018

Academic Editor: Stefan Pambuccian

Caption: Figure 1: Area of respiratory lining epithelium with squamous metaplastic change (arrowheads).

Caption: Figure 2: Focal areas of stroma displaying hyalinization (arrowheads).

Caption: Figure 3: Focal areas displaying calcification and ossification surrounded by variably spindled stroma.

Caption: Figure 4: Chondroid regions which support the hamartomatous nature of the lesion. Inset shows area at low-power with spatial relation of components, including surface ciliated epithelium (*) and bone (within dashed lines).

Caption: Figure 5: Variably dilated cystic regions within the lesion.

Caption: Figure 6: Disorganized bundles of nervous tissue interspersed within collagenous stroma.

Caption: Figure 7: Immunohistochemistry for smooth muscle actin (SMA) showing positive staining in the spindled lesionai cells.

Caption: Figure 8: Lesional cells are negative for pan-cytokeratin (ae1/ae3), desmin, Sox-10, and S100. CD34 and ERG/FLI-1 highlight vascular endothelial cells.
Table 1: Brief review of cases of nasal chondromesenchymal hamartomas
reported in the English literature. Some cases also reported in older
review articles [1].

Age         Sex     Follow-up                  Site

5 days       M       2 years               Nasal cavity

12 days      F     < 16 months             Nasal cavity

14 days      M          --          Nasal cavity Ethmoid Sinus

2 months     M      18 months              Nasal cavity

3 months     F       2 years        Nasal cavity Ethmoid Sinus

3 months     M       4 years               Nasal cavity

7 years      M          --          Nasal cavity Sphenoid sinus

4 months     M       13 years              Nasal cavity

0 days       M       5 years          Nasal cavity Sphenoiod
                                        sinus Ethmoid sinus

9 months     M       9 months              Nasal cavity

16 years     M       8 months              Nasal cavity

5 months     M          --                 Nasal cavity

11 years     M          --          Nasal cavity Ethmoid sinus

1 year       M          --                 Nasal cavity

11 years     M          --          Nasal cavity Ethmoid sinus

17 years     F          --                 Nasal cavity

25 years     M          --         Nasal cavity Maxillary sinus

69 years     F          --          Nasal cavity Ethmoid sinus

11 years     M       2 months              Nasal cavity

15 years     F       6 months              Nasal cavity

7 months     M      18 months              Nasal cavity

12 months    M          --                 Nasal cavity

19 months    M      10 months              Nasal cavity

2 cases previously reported, both
with PPB, multiple recurrences

10 years     F      21 months              Nasal cavity

11 years     M       4 months              Nasal cavity

11 years     M          --                      --

8 years      M       6 months      Sphenoid sinus Ethmoid sinus

14 years     M       4 years       Nasal cavity Maxillary sinus

23 years     M       3 months       Nasal cavity Ethmoid Sinus

40 years     F          --             Nasal cavity Ethmoid
                                       Sinus Maxillary sinus

9 months     F          --         Nasal cavity Maxillary sinus

14 years     M          --                 Nasal cavity

6 weeks      F      10 months              Nasal cavity

5 years      M       3 years        Nasal cavity Ethmoid sinus

10 months    M      18 months              Nasal cavity

49 years     M      2 years 4              Nasal cavity
                  years (phone)

                                         Systematic review

5 years      M          --                 Nasal cavity

13 years     F      12 months              Nasal cavity

3 years      M       3 years               Nasal cavity

Age           Pertinent Information          Study        Year

5 days                 --                [2] McDermott    1998

12 days      Intracranial extension      [2] McDermott    1998

14 days      Intracranial extension      [2] McDermott    1998
                 Residual tumor

2 months     Intracranial extension      [2] McDermott    1998

3 months     Intracranial extension      [2] McDermott    1998
                 Residual tumor

3 months               --                [2] McDermott    1998

7 years     PPB, multiple recurrences    [2] McDermott    1998

4 months     Intracranial extension        [3] Kato       1999

0 days         Orbital compression         [4] Hsueh      2001

9 months               --                  [4] Hsueh      2001

16 years         3-month history         [5] Alrawi M     2003

5 months       Orbital compression         [6] Kim B      2004

11 years         8-month history         [7] Norman ES    2004

1 year          Orbital extension          [8] ShetT      2004
                 Residual tumor

11 years               --                [9] Ozolek JA    2005

17 years               --                [9] Ozolek JA    2005

25 years         Bilateral NCMH          [9] Ozolek JA    2005
             Intracranial aneurysms

69 years               --                [9] Ozolek JA    2005

11 years               --                 [10] Low SE     2006

15 years       Bilateral NCMH PPB       [11] Johnson C    2007

7 months       Orbital compression      [12] Silkiss RZ   2007

12 months      Orbital compression      [13] Finitsis S   2009

19 months     Intracranial, orbital       [14] Kim JE     2009

2 cases previously reported, both       [15] Priest JR    2010
with PPB, multiple recurrences

10 years       Bilateral NCMH PPB       [15] Priest JR    2010

11 years               PPB              [15] Priest JR    2010

11 years               PPB              [16] Behery RE    2012

8 years          4-month history        [17] Uzomefuna    2012

14 years               --                 [18] Cho YC     2013

23 years        Orbital extension         [19] Li GY      2013

40 years           Malignant               [20] Li Y      2013
            transformation Recurrence

9 months       Orbital compression        [21] Moon S     2014

14 years       Bilateral NCMH PPB       [22] Obidan AA    2014

6 weeks                --                 [23] Wang T     2014

5 years          4-year history           [23] Wang T     2014

10 months        6-month history          [24] Lee CH     2015

49 years         5-year history          [1] Mason AK     2015

                                         [1] Mason AK     2015

5 years             Previous              [25] Avci H     2016
                  in remission

13 years         6-month history          [26] Unal A     2016

3 years                --                [27] Nakaya M    2017

Index. Cases older than 1 year of age at presentation. Bilateral/
cases associated with pleuropulmonary blastoma.
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Title Annotation:Case Report
Author:Mirchia, Kanish; Naous, Rana
Publication:Case Reports in Pathology
Date:Jan 1, 2018
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