Nasal Chondromesenchymal Hamartoma: Rare Case Report in an Elderly Patient and Brief Review of Literature.
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.
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 . As per one review , 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  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 . 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.
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.
 K. A. Mason, A. Navaratnam, E. Theodorakopoulou, and P. G. Chokkalingam, "Nasal Chondromesenchymal Hamartoma (NCMH): A systematic review of the literature with a new case report," Journal of Otolaryngology - Head and Neck Surgery, vol. 44, no. July, 2015.
 M. B. McDermott, T. B. Ponder, and L. P. Dehner, "Nasal chondromesenchymal hamartoma: An upper respiratory tract analogue of the chest wall mesenchymal hamartoma," The American Journal of Surgical Pathology, vol. 22, no. 4, pp. 425-433, 1998.
 K. Kato, R. Ijiri, Y. Tanaka, M. Hara, and K. Sekido, "Nasal chondromesenchymal hamartoma of infancy: The first Japanese case report," Pathology International, vol. 49, no. 8, pp. 731-736, 1999.
 C. Hsueh, "Nasal chondromesenchymal hamartoma in children: report of 2 cases with review of the literature," Archives of Pathology & Laboratory Medicine, vol. 125, no. 3, p. 400,2001.
 M. Alrawi, M. McDermott, D. Orr, and J. Russell, "Nasal chondromesynchymal hamartoma presenting in an adolescent," International Journal of Pediatric Otorhinolaryngology, vol. 67, no. 6, pp. 669-672, 2003.
 B. Kim, S.-H. Park, H. S. Min, J. S. Rhee, and K. C. Wang, "Nasal chondromesenchymal hamartoma of infancy clinically mimicking meningoencephalocele," Pediatric Neurosurgery, vol. 40, no. 3, pp. 136-140, 2004.
 E. S. Norman, S. Bergman, and J. K. Trupiano, "Nasal chondromesenchymal hamartoma: Report of a case and review of the literature," Pediatric and Developmental Pathology, vol. 7, no. 5, pp. 517-520,2004.
 T. Shet, A. Borges, C. Nair, S. Desai, and R. Mistry, "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?" International Journal of Pediatric Otorhinolaryngology, vol. 68, no. 3, pp. 359-364,2004.
 J. A. Ozolek, "Nasal chondromesenchymal hamartoma in older children and adults: series and immunohistochemical analysis," Archives of Pathology & Laboratory Medicine, vol. 129, no. 11, pp. 1444-50, 2005.
 S. E. Low, R. K. Sethi, E. Davies, and J. S. Stafford, "Nasal chondromesenchymal hamartoma in an adolescent," Histopathology, vol. 49, no. 3, pp. 321-323, 2006.
 C. Johnson, U. Nagaraj, J. Esguerra, D. Wasdahl, and D. Wurzbach, "Nasal chondromesenchymal hamartoma: Radiographic and histopathologic analysis of a rare pediatric tumor," Pediatric Radiology, vol. 37, no. 1, pp. 101-104, 2007.
 R. Z. Silkiss, S. S. Mudvari, and D. Shetlar, "Ophthalmologic presentation of nasal chondromesenchymal hamartoma in an infant," Ophthalmic Plastic & Reconstructive Surgery, vol. 23, no. 3, pp. 243-244, 2007.
 S. Finitsis, C. Giavroglou, S. Potsi et al., "Nasal chondromesenchymal hamartoma in a child," CardioVascular and Interventional Radiology, vol. 32, no. 3, pp. 593-597, 2009.
 J.-E. Kim, H.-J. Kim, H. K. Ji, Y.-H. Ko, and S.-K. Chung, "Nasal chondromesenchymal hamartoma: CT and MR imaging findings," Korean Journal of Radiology, vol. 10, no. 4, pp. 416-419, 2009.
 J. R. Priest, G. M. Williams, W. A. Mize, L. P. Dehner, and M. B. McDermott, "Nasal chondromesenchymal hamartoma in children with pleuropulmonary blastoma-A report from the International Pleuropulmonary Blastoma Registry registry," International Journal of Pediatric Otorhinolaryngology, vol. 74, no. 11, pp. 1240-1244, 2010.
 R. El Behery, J. Bedrnicek, A. Lazenby et al., "Translocation t(12;17)(q24.1;q21) as the sole anomaly in a nasal chondromesenchymal hamartoma arising in a patient with pleuropulmonary blastoma," Pediatric and Developmental Pathology, vol. 15, no. 3, pp. 249-253, 2012.
 V. Uzomefuna, F. Glynn, J. Russell, and M. McDermott, "Nasal chondromesenchymal hamartoma with no nasal symptoms," BMJ Case Reports, 2012.
 Y. C. Cho, I. Y. Sung, J. H. Son, and R. Ord, "Nasal chondromesenchymal hamartoma: Report of a case presenting with intraoral signs," Journal of Oral and Maxillofacial Surgery, vol. 71, no. 1, pp. 72-76,2013.
 G.-Y. Li, B. Fan, and Y.-Y. Jiao, "Endonasal endoscopy for removing nasal chondromesenchymal hamartoma extending from the lacrimal sac region," Canadian Journal of Ophthalmology, vol. 48, no. 2, pp. e22-e23,2013.
 M. Sharif and Abdul Jawad, "Interacting generalized dark energy and reconstruction of scalar field models," Modern Physics Letters A, vol. 28, no. 38, Article ID 1350180, 15 pages, 2013.
 S. H. Moon and M. M. Kim, "Nasal chondromesenchymal hamartoma with incomitant esotropia in an infant: A case report," Canadian Journal of Ophthalmology, vol. 49, no. 1, pp. e30-e32, 2014.
 A. A. Obidan and M. M. Ashoor, "Nasal chondromesenchymal hamartoma in an adolescent with pleuropulmonary blastoma," Saudi Medical Journal, vol. 35, no. 8, pp. 876-878, 2014.
 T. Wang, W. Li, X. Wu et al., "Nasal chondromesenchymal hamartoma in young children: CT and MRI findings and review of the literature," World Journal of Surgical Oncology, vol. 12, no. 1, p. 257,2014.
 C. H. Lee, Y. H. Park, J. Y. Kim, and J. H. Bae, "Nasal chondromesenchymal hamartoma causing sleep-disordered breathing in an infant," International Journal of Clinical and Experimental Pathology, vol. 8, no. 8, pp. 9643-9646, 2015.
 H. Avci, ?. Comoglu, E. Ozturk, B. Bilgic, and O. E. Kiyak, "Nasal chondromesenchymal hamartoma: a rare nasal benign tumor," Kulak burun bogaz ihtisas dergisi : KBB = Journal of ear, nose, and throat, vol. 26, no. 5, pp. 300-303,2016.
 A. Unal, R. O. Kum, Y. Avci, and D. T. Unal, "Nasal chondromesenchymal hamartoma, a rare pediatric tumor: Case report," The Turkish Journal of Pediatrics, vol. 58, no. 2, pp. 208-211, 2016.
 M. Nakaya, S. Yoshihara, A. Yoshitomi, and S. Baba, "Endoscopic endonasal excision of nasal chondromesenchymal hamartoma with intracranial extension," European Annals of Otorhinolaryngology, Head and Neck Diseases, vol. 134, no. 6, pp. 423-425, 2017.
 D. A. Hill, J. Ivanovich, J. R. Priest et al., "DICER1 mutations in familial pleuropulmonary blastoma," Science, vol. 325, no. 5943, p. 965, 2009.
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; email@example.com
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 . Age Sex Follow-up Site (Asymptomatic) 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 --  McDermott 1998 12 days Intracranial extension  McDermott 1998 14 days Intracranial extension  McDermott 1998 Residual tumor 2 months Intracranial extension  McDermott 1998 3 months Intracranial extension  McDermott 1998 Residual tumor 3 months --  McDermott 1998 7 years PPB, multiple recurrences  McDermott 1998 4 months Intracranial extension  Kato 1999 0 days Orbital compression  Hsueh 2001 9 months --  Hsueh 2001 16 years 3-month history  Alrawi M 2003 5 months Orbital compression  Kim B 2004 11 years 8-month history  Norman ES 2004 1 year Orbital extension  ShetT 2004 Residual tumor 11 years --  Ozolek JA 2005 17 years --  Ozolek JA 2005 25 years Bilateral NCMH  Ozolek JA 2005 Intracranial aneurysms 69 years --  Ozolek JA 2005 11 years --  Low SE 2006 15 years Bilateral NCMH PPB  Johnson C 2007 7 months Orbital compression  Silkiss RZ 2007 12 months Orbital compression  Finitsis S 2009 19 months Intracranial, orbital  Kim JE 2009 extension 2 cases previously reported, both  Priest JR 2010 with PPB, multiple recurrences 10 years Bilateral NCMH PPB  Priest JR 2010 11 years PPB  Priest JR 2010 11 years PPB  Behery RE 2012 8 years 4-month history  Uzomefuna 2012 14 years --  Cho YC 2013 23 years Orbital extension  Li GY 2013 40 years Malignant  Li Y 2013 transformation Recurrence 9 months Orbital compression  Moon S 2014 14 years Bilateral NCMH PPB  Obidan AA 2014 6 weeks --  Wang T 2014 5 years 4-year history  Wang T 2014 10 months 6-month history  Lee CH 2015 49 years 5-year history  Mason AK 2015  Mason AK 2015 5 years Previous  Avci H 2016 rhabdomyosarcoma in remission 13 years 6-month history  Unal A 2016 3 years --  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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