Sinonasal Hamartoma and Chronic Laryngeal Edema Causing Severe Dyspnea

Abstract

Background

Sinonasal hamartomas, according to the 5th edition of the World Health Organisation classification of head and neck tumours are divided into respiratory epithelial adenomatoid hamartoma (REAH), seromucinous hamartoma and chondromesenchymal hamartoma. Seromucinous hamartoma are benign proliferations of small eosinophilic glands surrounded by fibrous stroma and cuboidal cells. Hamartomas of the nasal cavity and paranasal sinuses are rare entities, clinically presenting as sinonasal polyposis.

Case Presentation

A 79- year-old female patient was referred to our emergency room due to severe dyspnea. Anterior rhinoscopy revealed unilateral greyish polypoid mass obstructing the middle, inferior and common nasal meatus. Systemic corticosteroids and oxygen therapy were administered under observation. Computerized tomographic imaging of the paranasal sinuses with contrast on all three planes showed an opacified polypoid mass in all meatus and the maxillary, anterior ethmoidal and sphenoidal sinus posteriorly extending to the choanae. On the coronal plane a widening of the olfactory clefts about 12 mm was described. FESS visualized that the polypoid mass originated from the posterior septum and extended to all meatus anteriorly and to the choanae posteriorly. The polypoid lesion was endoscopically completely excised. Histopathological analysis revealed a seromucinous hamartoma.

Conclusion

Seromucinous hamartoma are rare benign tumors of the sinonasal region with potential of malignant alteration. Unfortunately, they share symptoms and clinical appearance with other benign conditions of the sinonasal region. Therefore, it is even more important to consider them as a differential diagnose.

Background

Sinonasal hamartomas, according to the 5th edition of the World Health Organisation classification of head and neck tumours are divided into respiratory epithelial adenomatoid hamartoma (REAH), seromucinous hamartoma and chondromesenchymal hamartoma. Respiratory epithelial adenomatoid hamartomas are benign glandular proliferations which arise from the multilayered ciliated epithelium of the sinonasal tract [1]. On the other hand, seromucinous hamartomas are benign proliferations of small eosinophilic glands surrounded by fibrous stroma and cuboidal cells [2]. Chondromesenchymal hamartomas are composed of mesenchymal elements including cartilage [3]. Generally, hamartomas of the nasal cavity and paranasal sinuses are rare entities, clinically presenting as sinonasal polyposis which can be easily misdiagnosed. Moreover, it is important to recognize potential precancerous changes [4].

Hereby we report a rare case of a patient with seromucinous hamartoma at the nasal cavity and the paranasal sinuses unilaterally.

Case Presentation

A 79- year-old female patient was referred to our emergency room due to severe dyspnea. The patient was sitting upright, with stridorous breathing and an oxygen saturation level of 78% on room air. Upon examination, anterior rhinoscopy revealed unilateral greyish polypoid mass obstructing the middle, inferior and common nasal meatus. Indirect laryngoscopy showed bilateral Reinke`s edema grade three. Immediately, the patient was admitted for resuscitation and systemic corticosteroids and oxygen therapy were administered under observation. After twenty minutes, breathing improved and the oxygen saturation level rose to 92%. Full medical history was taken. The patient complained about difficulty breathing through her nose and mouth about two years ago, which intensified a few days before. She also reported rhinorrhea and hoarseness lasting for several years. There was no history of anosmia and epistaxis. She had a 20 pack – year smoking history and comorbidities of hypertension and atrial arrythmia.

The blood count was within physiological ranges and the peripheral blood eosinophil ratio was not elevated. Computerized tomographic imaging of the paranasal sinuses with contrast on all three planes showed an opacified polypoid mass in all meatus and the maxillary, anterior ethmoidal and sphenoidal sinus posteriorly extending to the choanae. No bone erosion was visualized. (Fig. 1A and B) On the coronal plane a widening of the olfactory clefts of about 12 mm was described. (Fig. 1C)

Fig. 1.

(A and B) Axial and sagittal plane of contrast enhanced CT scan of the paranasal sinuses revealed an opacified polypoid mass in all meatus and the maxillary, anterior ethmoidal and sphenoidal sinus posteriorly extending to the choanae. (C) Coronal plane of contrast enhanced CT scan with olfactory cleft widening of 12 mm

After preoperative check-up, laryngomicroscopy and functional endoscopic sinus surgery (FESS) under general anesthesia were performed. The Reinke’s edema were microsurgically removed. FESS visualized that the polypoid mass originated from the posterior septum on its left side and extended to all meatus anteriorly and to the choanae posteriorly, in the absence of chronic rhinosinusitis. The polypoid lesion was completely excised endoscopically. Anterior nasal packing was inserted for three days. Histopathological analysis confirmed a seromucinous hamartoma. (Figure 2A and B)

Fig. 2.

(A and B) Pathological features of sinonasal SH (hematoxylin-eosin staining). Sermomucionous ducts surrounded by fibrous stroma and single lined cuboidal cells with eosinophillic cytoplasm. Invagination of the respiratory epithel

The postoperative course was uneventful, and the patient was discharged on the 3rd postoperative day. Oral and local antibiotics were prescribed. We followed up with the patient after six months, during which she neither reported nasal obstruction nor rhinorrhea nor anosmia. Her hoarseness had improved. On clinical examination there was no evidence of recurrence.

Discussion

Despite seromucinous hamartoma being classified as a new subtype of sinonasal hamartoma according to the 5th edition of the World Health Organisation classification of head and neck tumors, they are indeed rare [1]. First mentioned in the literature by Baillie and Batsakis in 1974, there have been approximately 39 cases of seromucinous hamartoma published in the English literature to date [5–7]. The incidence of seromucinous hamartoma is moderately higher in females (1.4:1) according to previous literature reviews and case reports [8]. The age ranged from 14 to 85 years [8, 9]. However, Alokby et al. published a case report of seromucinous hamartoma of the ethmoid sinus in a two -year old female patient, which was clinically presented as a mass of the medial canthal area [10].

The etiology of seromucinous hamartoma remains largely unknown. Similiar to REAH, there have been reported cases of seromucinous hamartoma coexisting with chronic rhinosinusitis with or without nasal polyposis [2, 5, 11]. A Brazilian cross - sectional study by Issa et al. revealed that inflammatory nasal polyposis and CT verified olfactory cleft enlargement, were histopathologically confirmed with REAH in 47.4% of the cases [11]. Thus, the most acceptable hypothesis is that chronic inflammation can contribute to the development of hamartoma [12].

The most common symptoms are unilateral nasal obstruction followed, by purulent rhinorrhea, hyposmia, and epistaxis [2, 6]. Some asymptomatic cases are described, where the lesions have been found incidentally [6, 13]. On clinical examination unilateral polypoid mass in the nasal cavity without bleeding is usually found [2, 5, 6].

Imaging features, such as CT or MR scans, can aid in the diagnosis of hamartoma. According to an imaging review from 2019, the appearence of seromucinous hamartoma is similiar to respiratory epithelial adenomatoid hamartoma (REAH). On CT scan typically a homogenous mass with olfatory cleft widening without bone erosion is described like in our case. On sagittal images, a half- moon appearance is observed [14, 15]. Interestingly, the width of the olfactory cleft has been analyzed since 2006 in order to differentiate between REAH and other conditions. In a retrospective study, the medians of olfactory cleft enlargement in REAH, nasal polyposis and normal CT scans were compared. The median olfactory cleft enlargement was found to be 12.1 mm on the coronary plane for REAH, 5.4 mm for nasal polyposis and 4.2 mm for normal individuals [16].

Despite these imaging findings, establishing a definitive diagnosis often requires considering a range of differential diagnoses based on symptoms, clinical examinations, and radiological evaluations. Some of the most important conditions to consider include inflammatory polyps, juvenile angiofibroma, olfactory neuroblastoma, REAH, and low-grade non-intestinal adenocarcinoma [6].

Ultimately, histopathological and immunohistochemical analysis are the most precise methods for diagnosing seromucinous hamartoma. Since seromucinous hamartoma consist of small eospinophilic glands surrounded by stroma, the imunohistochemical staining is positive for CK7, CK19 and S100, which indicate the serous component. The absence of basal cells is confirmed by a negative p63 staining [17]. However, we should address that low-grade tubolopappilary adenocarcinoma (LGTA) can share the same imunohistochemical profile. Baneckova et al. demonstrated the morphologically and imunohistochemically overlapping between REAH, seromucinous hamartoma and LGTA. Those findings support the hypothesis that seromucinous and respiratory epithelial hamartoma can be the precursor lesion of LGTA [18].

Last but not least, the treatment of choice is complete transnasal endoscopically excision if seromucinous hamartoma is previously confirmed [4, 6].

Conclusion

Seromucinous hamartoma are rare benign tumors of the sinonasal region with potential of malignant alteration. Unfortunately, they share symptoms and clinical appearance with other benign conditions of the sinonasal region. Therefore, it is even more important to consider them as a differential diagnose.

Funding

The authors received no financial support for the research, authorship, and/or publication of this article.

Data Availability

Not applicable.

Declarations

Ethics Approval

Not applicable.

Informed Consent

Written informed consent was obtained from the patient for publication of this case report and accompanying images. Written consent is available for review by the Editor-in-Chief of this journal on request. All procedures performed in studies involving human participants were in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments.

Competing Interests

The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.