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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 11  |  Issue : 2  |  Page : 155-158

Respiratory epithelial adenomatoid hamartoma – A neglected entity in the nasal cavity


Department of Pathology, Mahatma Gandhi Institute of Medical Sciences, Wardha, Maharashtra, India

Date of Submission24-Apr-2022
Date of Decision25-May-2022
Date of Acceptance13-Jun-2022
Date of Web Publication22-Aug-2022

Correspondence Address:
Vitaladevuni Balasubramanyam Shivkumar
Department of Pathology, Mahatma Gandhi Institute of Medical Sciences, Sevagram - 442 102, Wardha, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/sjhs.sjhs_49_22

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  Abstract 


Respiratory epithelial adenomatoid hamartoma (REAH) is a rare subtype of hamartoma which consist of the proliferation of glandular spaces and is lined by ciliated epithelium or goblet cells. Here, we report the case of REAH in a 45-year-old male who presented with bilateral nasal blockage in the past 1 year. The bilateral polypoidal nasal mass was excised. Microscopically, the proliferation of glandular spaces lined by ciliated epithelium and goblet cells and thickened eosinophilic basement membrane was seen. Immunohistochemistry showed positivity for pancytokeratin. One should always consider REAH as one of the differential diagnoses of a symptomatic polypoidal sinonasal mass.

Keywords: Hamartoma, nasal polyposis, respiratory epithelial adenomatoid hamartoma


How to cite this article:
Vidwans TP, Deshmukh AV, Shivkumar VB. Respiratory epithelial adenomatoid hamartoma – A neglected entity in the nasal cavity. Saudi J Health Sci 2022;11:155-8

How to cite this URL:
Vidwans TP, Deshmukh AV, Shivkumar VB. Respiratory epithelial adenomatoid hamartoma – A neglected entity in the nasal cavity. Saudi J Health Sci [serial online] 2022 [cited 2022 Oct 3];11:155-8. Available from: https://www.saudijhealthsci.org/text.asp?2022/11/2/155/354164




  Introduction Top


Hamartoma is a benign focal malformation which resembles a benign neoplasm in its tissue of origin. It is comprised of disorganized cytologically normal mature cells and tissues of its origin.[1] These are commonly seen in the liver, spleen, kidney, lung, and intestine but rarely from the upper aerodigestive tract. Respiratory epithelial adenomatoid hamartoma (REAH) is a subtype of hamartoma which is rarely seen in the upper respiratory tract.[2] This entity is often neglected due to the nonspecific and vague presentation of a patient. Here, we present the case of REAH in a 45-year-old male.


  Case report Top


A 45-year-old male patient presented to the Otolaryngology Outpatient Department at Mahatma Gandhi Institute of Medical Sciences, Sevagram, Wardha, Maharashtra, a rural tertiary care hospital in Central India with complaints of nasal blockage in the past 1 year. It was gradual in onset and progressive in nature, aggravated during cold, and upper respiratory tract infections, and was relieved on medication. It was also associated with whitish, mucopurulent, sticky, and nonblood-stained nasal discharge. There were no complaints of epistaxis, foul-smelling discharge, visual disturbance, or associating headache. There was no history of trauma or infection, leading to the obstruction. On general examination, the patient was comfortable and there were no signs of respiratory distress. The cold spatula test was reduced bilaterally. Anterior rhinoscopy revealed the presence of a grayish mass in the bilateral nasal cavity. It was arising from the lateral wall of the nose, middle turbinate, and meatus. It was soft, nonfriable, nontender, and glistening in appearance and did not bleed on probing. His vital signs and systemic examination were within the normal range. Laboratory investigations were also within normal range with no signs of infection or coagulopathy. A preliminary diagnosis of bilateral chronic rhinosinusitis with nasal polyposis was made.

Computerized tomography of the paranasal sinus revealed polypoidal nonenhancing soft-tissue density lesions noted in the nasal cavity in the left middle and left inferior turbinate of size 15 mm × 11 mm × 10 mm with mucosal thickening of bilateral nasal turbinates. There was nonenhancing mucosal wall thickening of bilateral maxillary, ethmoid, sphenoid, and frontal sinuses with complete opacification [Figure 1]. It was reported as pansinusitis with a possibility of nasal polyp. Anterior functional endoscopic sinus surgery was done. Debridement of multiple nonfriable, pinkish-to-grayish sessile polypoidal masses from bilateral nasal, middle turbinate, uncinate process, septum, and choana region was done. Uncinectomy was done and the maxillary antrum was widened on both sides. Hemostasis was achieved and nasal packing was done.
Figure 1: CT paranasal sinus (a) coronal view and (b) sagittal view showing revealed polypoidal nonenhancing soft-tissue lesions in the left middle and left inferior turbinates with thickening of bilateral nasal turbinates along with nonenhancing mucosal wall thickening of bilateral maxillary, ethmoid, sphenoid, and frontal sinuses (red and green arrow). CT: Computed tomography

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The specimen was then sent to the surgical pathology section for examination. Grossly, the specimen consisted of multiple gray‒white-to-gray‒red tissue pieces aggregating 1.5 cm × 1 cm × 0.8 cm. Microscopically, hematoxylin and eosin-stained sections showed proliferation of glands. These glands had a thick eosinophilic basement membrane. The glandular spaces were lined by goblet cells. The glands were surrounded by chronic inflammatory cells and vascularization [Figure 2] and [Figure 3]. No dysplasia or atypia was seen in epithelial cells. No mitosis or necrosis was present. Immunohistochemistry was done using a formalin-fixed and paraffin-embedded tissue block. The glandular elements showed strong positive staining for pan-cytokeratin, (1:100, Mouse cocktail antibody, clone: AE1 & AE3, Cell Marque) [Figure 4]. A diagnosis of REAH was made.
Figure 2: Proliferation of glandular elements (H and E, ×100)

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Figure 3: Glands lined by goblet cells and were surrounded by chronic inflammatory cells (H and E, ×400)

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Figure 4: Positivity for pan-cytokeratin (IHC, ×100)

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  Discussion Top


The term hamartoma was coined by Albrecht in 1904 and originated from ancient Greek word ”hamartia” meaning error and ”-oma” meaning benign growth.[3] Hamartoma is a benign neoplasm having focal malformation, comprising disorganized but morphologically normal mature cells and tissues of its origin and it has no tendency to regress spontaneously.[4]

REAH, mostly, is an incidental finding and due to its low prevalence, can often be mistaken with other sinonasal masses ranging from benign inflammatory polyp to truly inverted papilloma till grave diagnosis of sinonasal adenocarcinoma.[2],[5] However, grossly and radiologically, it resembles an inflammatory polyp, therefore, often is a neglected entity. Only histopathology is the key to the diagnosis of this entity.

REAH is most commonly found in posterior aspect of the nasal septum, followed by middle meatus, inferior turbinate, maxillary sinus and ethmoidal sinus. The incidence of frontal hamartomas in the head and neck is very rare. The common sites in the head and neck where hamartomas can occur are the soft tissue of the cheek, neck, maxilla, gum margin, nose, and tongue.[5],[6]

A subgroup of hamartoma, REAH, of the upper aerodigestive tract was described first by Wenig and Heffner in 1995.[7] The diagnosis of REAH is rare as it is underdiagnosed.[8] The incidence of REAH ranges from the third to ninth decades with a median age group of the sixth decade. It is more commonly observed in males as compared to females.[4] The patients usually present with vague and nonspecific symptoms such as unilateral obstruction of the nasal cavity, epistaxis, and rhinorrhea.[4] RHEA in the nasal cavity is mostly observed in the posterior nasal septum and is usually unilateral. Our case presented with bilateral nasal mass, leading to obstruction.

The exact etiology of REAH is not known to date. As this entity is usually observed in patients with inflammatory polyposis, rhinosinusitis, and sinus surgery, the inflammatory process is considered the probable mechanism.[9] It can also be attributed to the presence of inflammatory cells in microscopy. Few authors have also linked this etiology with smoking and asthma.[9] Our patient was a middle-aged male who presented with rhinosinusitis with nasal polyposis. As the soft-tissue mass occupies the nasal cavity, there are no typical pathognomonic features on radiography for an exact diagnosis.

Histopathological examination is the gold standard for the exact diagnosis of REAH. Microscopy shows the proliferation of glandular spaces which are lined by ciliated epithelium and goblet cells along with the presence of chronic inflammatory cells and vascularization. It should be differentiated from other polypoidal masses of the nasal cavity such as an inflammatory polyp, inverted papilloma, seromucinous hamartoma, and low-grade sinonasal adenocarcinoma.[10] Inflammatory polyp, on microscopy, shows edematous, fibrotic, or loosely myxoid stroma which is covered by respiratory epithelium. The stroma is infiltrated with mixed inflammatory cells, along with absent or decreased submucosal glands, unlike REAH. Inverted papilloma shows multilayering of the epithelium and decreased seromucinous glands in lamina propria, whereas in REAH, there is thickened basement membrane with increased seromucinous glands.[10] Seromucinous hamartoma is another important differential and is distinguished from REAH due to its proliferation of glands arranged in a lobular, clustered, or haphazard arrangement. Both are lined by ciliated respiratory-type epithelium, but no thickening of the basement membrane is seen in seromucinous hamartoma. Low-grade sinonasal adenocarcinoma (SNAC), which is also a rare entity to arising from the sinonasal tract, shows back-to-back glands, occasional nuclear pleomorphism, mitotic activity, and desmoplastic stroma. On immunohistochemistry, REAH, inverted papilloma, and seromucinous hamartoma stains are positive for CK20+, CK 7+, and CD X 2–; whereas SNAC is positive for CK 7+, CK 20±, and CKX 2.[11]

Complete total excision is the treatment of choice for REAH. There has been no evidence of recurrence or aggressive behavior of this hamartoma to date.


  Conclusion Top


REAH, although a rare lesion, should always be considered one of the differential diagnoses of a symptomatic polypoidal sinonasal mass. The treatment of choice is conservative surgical resection and it has no recurrence or metastatic change in the lesion is noted to date. Therefore, otolaryngologists should always consider this rare entity to avoid aggressive surgical intervention in such cases.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal his identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Al-Musaileem N, Qazi IM, Bastaki JM, Ebrahim MK. An atypical presentation of a Respiratory Epithelial Adenomatoid Hamartoma, a case report. Ann Med Surg 2019;46:27-30.  Back to cited text no. 1
    
2.
Jeyasakthy S, Shukri N, Ramli RR, Wahab WA, Zawawi N. Sinonasal respiratory epithelial adenomatoid hamartoma: An overlooked entity. Egypt J Ear Nose Throat Allied Sci 2017;18:191-3.  Back to cited text no. 2
    
3.
Avilés Jurado FX, Guilemany Toste JM, Alobid I, Alós L, Mullol I Miret J. The importance of the differential diagnosis in rhinology: Respiratory epithelial adenomatoid hamartoma of the sinonasal tract. Acta Otorrinolaringol Esp 2012;63:55-61.  Back to cited text no. 3
    
4.
Di Carlo R, Rinaldi R, Ottaviano G, Pastore A. Respiratory epithelial adenomatoid hamartoma of the maxillary sinus: Case report. Acta Otorhinolaryngol Ital 2006;26:225-7.  Back to cited text no. 4
    
5.
Kumar SA, O'Meara C, Fredericks S, Havas T. Beware the respiratory epithelial adenomatoid hamartoma – A malignant masquerador. J Surg Case Rep 2021;2021:rjab007.  Back to cited text no. 5
    
6.
Garry S, Corbett M, Elsafty N, Keogh IJ. Nasal respiratory epithelial adenomatoid hamartoma. Otolaryngol Case Rep 2020;6:1-3. [doi: 10.1016/j.xocr. 2020.100167].  Back to cited text no. 6
    
7.
Wenig BM, Heffner DK. Respiratory epithelial adenomatoid hamartomas of the sinonasal tract and nasopharynx: A clinicopathologic study of 31 cases. Ann Otol Rhinol Laryngol 1995;104:639-45.  Back to cited text no. 7
    
8.
Cascio F, Basile GC, Felippu AW, Felippu AW, Militi D, Portaro S, et al. Diagnosis and Treatment of Bilateral Respiratory Epithelial Adenomatoid Hamartomas With and Without Sinonasal Polyposis. Ear Nose Throat J 2021;100:495S-497S.  Back to cited text no. 8
    
9.
Liang J, O'Malley BW Jr., Feldman M, Newman JG. A case of respiratory epithelialadenomatoid hamartoma. Am J Otolaryngol 2007;28:277-9.  Back to cited text no. 9
    
10.
Shanbag R, Patil P, Rani SH, Kulkarni S. Respiratory Epithelial Adenomatoid Hamartoma (REAH) in the olfactory cleft: Often masked by bilateral nasal polyps. Indian J Otolaryngol Head Neck Surg 2019;71:2121-6.  Back to cited text no. 10
    
11.
Ozolek JA, Barnes EL, Hunt JL. Basal/myoepithelial cells in chronic sinusitis, respiratory epithelial adenomatoid hamartoma, inverted papilloma, and intestinal-type and nonintestinal-type sinonasal adenocarcinoma: An immunohistochemical study. Arch Pathol Lab Med 2007;131:530-7.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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