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  • 2.5-year-old male intact Great Pyrenees presented for a six-month history of chronic epistaxis, excessive mucoid nasal discharge, and recent difficulty breathing.
  • On physical examination, two firm subcutaneous masses were palpable over the nasal bone.
  • Four months prior to presentation, a head CT and nasal biopsies were performed at another institution, which showed a heterogenous soft-tissue, fluid, and mineral-attenuating, contrast-enhancing mass within the right nasal passage. Biopsies were consistent with nasal polypoid tissue.
  • Complete blood count and chemistry panel were within normal limits.
  • Treatment had been initiated previously with antibiotics and steroids with no clinical improvement.
  • Large heterogeneous, generally hypoenhancing (with some areas of rim enhancement and heterogeneous enhancement) soft-tissue attenuating mass with amorphous central mineralization arising from the right nasal passage. The mass/soft tissue mostly fills the nasal cavity, with leftward deviation of the nasal septum and extension into the left nasal passage. Lysis of a large portion of the nasal turbinates with some remaining turbinates in the left caudal and right caudodorsal nasal passages.
  • Lysis of the right nasal bones with extension into the soft tissues dorsal to the nasal cavity. Smooth to mildly irregular periosteal reaction along the dorsal right nasal bone.
  • Multifocal lysis of the right hard palate/palatine bone with mild extension of the mass ventrally and rostrally, with extension into/filling of the choanae.
  • Lysis of the medial wall of the right orbit with mild extension into the retrobulbar space.
  • Lysis of the ventral wall of the right frontal sinus with extension of mostly nonenhancing soft tissue into and filling both frontal sinuses. Few areas of rounded rim enhancement within the sinuses as well as lysis of the septum between the frontal sinuses.
  • Lysis of the right cribriform plate with extension into the cranial vault. Mild leftward midline shift at this level, but no abnormal contrast enhancement appreciated within the adjacent brain parenchyma.
  • Caudal extension of the mass into the rostral nasopharynx, where it occupies the entire nasopharyngeal lumen. Heterogeneous contrast enhancement is most pronounced within this part of the mass.
  • Very small mineral foci within both tympanic bullae, consistent with incidental tympanic bone spicules.
  • Mild mineralization of the external ear canals bilaterally, likely incidental.
  • Mild amount of fluid within the cervical esophagus.
  • Normal medial retropharyngeal and mandibular lymph nodes.
  • No significant findings on thoracic CT (not included).

• Large nasal mass with widespread regional extension and turbinate/adjacent osseous lysis as described, including extension into/occlusion of the rostral nasopharynx, extension into the cranial vault, and extension into the frontal sinuses with suspect concurrent obstructive frontal sinusitis. Given the reported previous biopsy results consistent with polyps, large areas of hypoenhancement within the mass, and young age of the patient, severe nasal polyposis is considered; however, neoplastic etiologies such as carcinoma, sarcoma, or hamartoma are also considered.

  • Rhinotomy and debulking of a large portion of the nasal mass was performed.
  • Histopathology of the ethmoid and maxillary turbinates was consistent with a chondro-osseous respiratory epithelial adenomatoid hamartoma (COREAH) with concurrent nasal polyps.

Hamartomas are neoplasms characterized by disordered proliferation of tissues specific to an anatomic region. Respiratory epithelial adenomatoid hamartomas (REAH) are well-classified in the human literature and are generally thought to be benign, although the pathogenesis is debated.1 Chondro-osseous respiratory epithelial adenomatoid hamartoma (COREAH) is an extremely rare upper respiratory neoplasm in humans, with only 11 reported cases as of 2019.2 A study in the veterinary literature3 describes three cases in dogs with nasal masses histopathologically diagnosed as COREAH. The ages of these dogs were variable (2, 8, and 10 years old). Similar clinical signs as the patient in our case were seen, including epistaxis and difficulty inspiring. Reverse sneezing, hyporexia, and lethargy were also noted. The duration of the clinical signs prior to diagnosis was 2, 6, and 18-months. In the two cases that underwent CT scan, masses were predominantly soft-tissue attenuating with internal mineralization. Both were located unilaterally within a caudal nasal passage with bilateral extension into the frontal sinuses. Both masses exhibited regional osteolysis and multifocal periosteal new bone formation. Following CT scan, the primary differential for both masses was malignant neoplasia (e.g. carcinoma, sarcoma). The concurrent histopathologic finding of nasal polyps in this patient is of note. Patients with nasal polyposis may present with similar clinical signs (reverse sneezing, nasal discharge, epistaxis) and may share overlapping CT features with COREAH or other nasal tumors (e.g. turbinate and/or septal destruction, regional osteolysis).4 Nasal polyps have been documented to occur in conjunction with nasal neoplasms, primarily nasal adenocarcinoma, in the veterinary literature.5 Nasal polyps have also been associated with rhinosporidiosis in dogs4 but in many instances, the etiology of nasal polyposis is unknown. Although theoretically benign, human literature has suggested this tumor may have more aggressive biological behavior than previously thought. Two cases in the veterinary study were euthanized within 9 months of diagnosis; the third underwent rhinotomy with no evidence of recurrence months postoperatively. Although uncommon, COREAH is a potential differential diagnosis in patients with destructive, partially mineralized nasal passage masses, which may otherwise be presumed to represent carcinoma or other malignancy.

Case Follow-up: One month following rhinotomy, the patient continued to exhibit persistent difficulty breathing. Follow-up rhinoscopy revealed numerous polypoid masses throughout the nasal cavity intermixed with several solid-appearing masses. Nasal biopsies were consistent with regrowth of polypoid tissue. Repeat rhinotomy was not recommended due to the extent of tissue regrowth in the short interim period. Consultation with a radiation oncology service was recommended, extrapolated from documented human treatments with low-dose radiation in benign cartilaginous proliferative diseases.6 Ultimately, the owners did not elect to proceed with further treatment.

References:

  1. Fedda F, Boulos F, Sabri A. Chondro-osseous respiratory epithelial adenomatoid hamartoma of the nasal cavity. Int Arch Otorhinolaryngol. 2013 Apr;17(2):218-21. doi: 10.7162/S1809-97772013000200017. PMID: 25992017; PMCID: PMC4423336.
  2. Daniel A, Wong E, Ho J, Singh N. Chondro-Osseous Respiratory Epithelial Adenomatoid Hamartoma (COREAH): Case Report and Literature Review. Case Rep Otolaryngol. 2019 Jul 25;2019:5247091. doi: 10.1155/2019/5247091. PMID: 31428496; PMCID: PMC6683791.
  3. LaDouceur EEB, Michel AO, Lindl Bylicki BJ, Cifuentes FF, Affolter VK, Murphy BG. Nasal Cavity Masses Resembling Chondro-osseous Respiratory Epithelial Adenomatoid Hamartomas in 3 Dogs. Veterinary Pathology. 2016;53(3):621-624.
  4. Holt DE, Goldschmidt MH. Nasal polyps in dogs: five cases (2005 to 2011). J Small Anim Pract. 2011 Dec;52(12):660-3. doi: 10.1111/j.1748-5827.2011.01152.x. PMID: 22136450.
  5. Tarrant JC, Holt DE, Durham AC. Co-occurrence of Nasal Polyps and Neoplasms of the Canine Nasal Cavity. Vet Pathol. 2019 Nov;56(6):885-888. doi: 10.1177/0300985819854438. Epub 2019 Jun 6. PMID: 31170873.
  6. Chong CC, Kneebone A, Kirsh G. Radiotherapy in the management of recurrent synovial chondromatosis. Australasian Radiology. 2007 Feb;51(1):95-8.