Respiratory Epithelial Adenomatoid Hamartoma Research Articles

Respiratory epithelial adenomatoid hamartoma in a child with nasal polyposis. Case report

Benign and malignant formations of the nose and paranasal sinuses in the early stages of development are asymptomatic or manifest minor nonspecific signs, and therefore often remain unrecognized. Hamartoma is a tumor-like formation of a dysembriological nature, consisting of excessive tissues peculiar to the affected organ. Due to the rarity of the occurrence of hamartomas of the sinonasal region, especially in pediatric practice, their diagnosis may present certain difficulties, since they can mimic other formations, such as nasal polyps or inverted papilloma, may occur as an isolated formation in the nasal cavity, or in combination with chronic rhinosinusitis, nasal polyposis, allergic rhinosinusitis. Aim – to report a rare clinical case in pediatric practice of respiratory epithelial adenomatoid hamartoma associated with allergic rhinosinusitis and polyposis, display the relationship between the occurrence of respiratory epithelial adenomatoid hamartoma and chronic inflammation of the nasal cavity and paranasal sinuses, to carry out differential diagnosis with other exophytic aggressive lesions of the nasal cavity, combine and summarize available data on sinonasal hamartomas. The article describes a clinical case of respiratory epithelial adenomatoid hamartoma in a 17-year-old child with a previous 4-year follow-up with nasal cavity polyposis and a history of repeated polypotomy. The results of endoscopy and computed tomography of the nasal cavity and paranasal sinuses, the results of surgical intervention, as well as the conclusions of a histological examination of the tumor removed from the patient are presented. The authors analyzed the domestic and foreign literature, on the basis of which they presented a differential series of sinonasal hamartomas. Respiratory epithelial adenomatoid hamartoma is a rare pathological formation of the sinonasal region, which manifests itself as an isolated polypoid mass in the nasal cavity or as an accidental surgical finding in patients with chronic sinusitis. The association with nasal polyps supports the hypothesis that inflammation may be one of the inducing factors. Respiratory epithelial adenomatoid hamartoma, although rare, should be considered in the differential diagnosis of exophytic lesions of the nasal cavity. The tactics of treatment involves endoscopic removal of the formation within healthy tissues, which provides good long-term results, relapses are extremely rare.

Respiratory Epithelial Adenomatoid Hamartoma: An Uncommon Differential of Nasopharyngeal Tumor

Respiratory epithelial adenomatoid hamartoma (REAH) is an uncommon tumor-like lesion that has been reported within the nose, paranasal sinuses, and less frequently in the nasopharynx. While it is believed to be benign, its clinical presentation, radiological characteristics, and microscopic features may closely mimic more aggressive tumors of the upper respiratory tract, potentially leading to needless life-altering treatment. Prior to now, there had been no reported cases of this lesion in West Africa. We present a 35-year-old male with persistent bilateral nasal obstruction, difficulty with swallowing, and bilateral reduction in hearing, whose CT findings were highly suggestive of a nasopharyngeal tumor, but upon biopsy and histology showed features most consistent with REAH. Surgery completely alleviated his symptoms with no clinical evidence of recurrence after a 1-year follow-up period.

A Rare Case of Sinonasal Seromucinous Hamartoma Developing from the Nasal Septum.

A 45-year-old man presented with a history of chronic left nasal congestion. Nasal endoscopy revealed a pedunculated polypoid mass with glandular epithelium surface on the posterior nasal septum. Computed tomography revealed a 25-mm mass-like growth in the left posterior nasal cavity attached to the nasal septum with a stalk. The patient underwent transnasal endoscopic surgery, and the tumor was removed under a block with safety margin. The final pathological diagnosis was sinonasal seromucinous hamartoma (SH). Sinonasal SH is a rare tumor with only 31 reported cases. Transnasal endoscopic surgery is currently the first-line treatment for sinonasal SH. Differential diagnoses of this lesion include inflammatory polyps, respiratory epithelial adenomatoid hamartoma, and adenocarcinoma. Although SH is a benign tumor, its progression to adenocarcinoma has been reported. Therefore, unilateral posterior nasal tumors must be diagnosed precisely.

Sinonasal Hamartoma and Chronic Laryngeal Edema Causing Severe Dyspnea.

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. 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 12mm 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. 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.

Nasal Respiratory Epithelial Adenomatoid Hamartoma: Three Case Reports and Review of the Literature.

Hamartomas, as non-true tumors, are commonly found in the lungs, digestive tract, and kidneys, and there are few clinical reports on nasal cases. Nasal histiocytosis accompanied by lymphatic lesions is a common feature of nasal hamartoma. Three patients with nasal cavity hamartoma were treated in our department, including 1 male and 2 females, with nasal obstruction or epistaxis. The imaging (computed tomography, magnetic resonance imaging) of 2 patients showed a soft tissue mass in the nasal cavity and no erosion of bone, while in 1 patient, the imaging was inconclusive. The excised material in all patients was found to be a soft mass. The 3 cases showed the same pattern under histological examination, and all 3 patients were diagnosed with nasal cavity respiratory epithelial adenomatoid hamartoma (REAH). Nasal hamartoma is a rare disease with nonspecific clinical manifestations and imaging features and is often misdiagnosed. Only by a better understanding of this disease can the rate of correct clinical diagnosis be improved.

Seromucinous Hamartoma of the Sinonasal Cavity.

Benign tumors or malignant neoplasms must be evaluated in patients with unilateral nasal cavity mass lesions. The 3 most prevalent unilateral benign mass lesions in such individuals are nasal polyps (NPs) and inverted papillomas (IPs). Although rare, it should be kept in mind that sinonasal hamartomas can be occasionally diagnosed as well. Among sinonasal hamartomas, respiratory epithelial adenomatoid hamartoma is more prevalent, with seromucinous hamartoma (SMH) being the second most common. Unlike NPs, sinonasal hamartomas are benign tumors with growth potential, which means it should not be undertreated and warrants surgical removal for treatment. However, sinonasal hamartomas do not have local invasion or malignant transformation potential like IPs; hence, it is vital not to overtreat them. Therefore, understanding the histopathology of SMH and thereby establishing proper surgical planning prior to the surgery remains crucial in such cases. Here, we present a successfully treated case of SMH with a distinctive radiographic, gross, and pathological clinical image of SMH.

[Clinical characteristics of nasal respiratory epithelial adenomatoid hamartoma].

〓 Objectives: To analyze the pathological and clinical features of nasal respiratory epithelial adenomatoid hamartoma（REAH）, and summarize the diagnostic points, to improve the experience of diagnosis and treatment. Methods:The clinical data of 16 patients with REAH were analyzed retrospectively. The clinical manifestations, pathological features, imaging features, surgical treatment and prognosis were summarized. Results:16 cases of REAH were studied, 10 cases（62.50%） were associated with sinusitis, 1 case（6.25%） was associated with inverted papilloma, 1 case（6.25%） was associated with hemangioma. 5 cases（31.25%） had a history of nasal sinus surgery, including 1 case with 3 times of nasal sinus surgery, 1 case with 2 times of nasal sinus surgery, 3 cases with 1 time of nasal sinus surgery; 10 cases（62.50%） occurred in the bilateral olfactory cleft, 2 cases（12.50%） in the unilateral olfactory cleft, 3 cases（18.75%） in the unilateral middle turbinate, 1 case（6.25%） in the nasopharynx. All 16 patients were pathologically diagnosed as REAH. In the patients with lesions located in bilateral olfactory fissures, symmetrical widening of olfactory fissures and lateral displacement of middle turbinate were observed on preoperative sinus CT. The average width of bilateral olfactory fissures was （9.9±2.70） mm. The ratio of wide to narrow olfactory cleft was 1.21 ± 0.19. There was no significant difference in Lund-Mackay score between the two sides（P>0.05）. All patients underwent surgery under general anesthesia and nasal endoscopy. The follow-up period ranged from 1 to 66 months, and no recurrence occurred. Conclusion:Preoperative diagnosis of REAH is facilitated by the combination of clinical manifestations and endoscopic and imaging features. Endoscopic complete resection can achieve a good therapeutic effect.

Predictive significance of computed tomography in bilateral sinonasal respiratory epithelial adenomatoid hamartoma.

Respiratory epithelial adenomatoid hamartoma (REAH) is easily confused with nasal polyps (NP). The typical manifestation of REAH on CT is the enlargement of bilateral olfactory clefts (OCs). The widening of the OCs in the CT scan is a biomarker for diagnosing REAH associated with NP.

Analysis of KRAS, BRAF, and EGFR mutational status in respiratory epithelial adenomatoid hamartoma (REAH).

Respiratory epithelial adenomatoid hamartoma (REAH) is a sinonasal glandular overgrowth arising from the surface respiratory epithelium and invaginating into the stroma. Clinically, it appears as a polypoid mass that may cause nasal obstruction, anosmia, and epistaxis. The presence of cartilaginous and/or osseous areas move the lesion to a chondro-osseous respiratory epithelial (CORE) hamartoma subtype. Scattered small seromucinous glands may be observed between typical REAH glands and when it is the only feature, it represents seromucinous hamartoma (SH). The molecular pathogenesis of REAH has been poorly explored and remains unclear. Given that KRAS, BRAF, and EGFR mutations have been detected in a variety of sinonasal tumors, we aimed to assess these mutations in REAH and SH. Ten REAH (including one CORE subtype), in addition to two SH cases, were Sanger sequenced by standard techniques. The targeted regions included KRAS exons 2-4 (encompassing hotspots codons 12, 13, 61, and 146), BRAF exons 11 and 15 (spanning the V600 codon), and EGFR exons 19 and 20. All REAH and SH samples showed wild-type sequences for KRAS, BRAF, and EGFR genes. Our results demonstrate a lack of KRAS, BRAF, or EGFR pathogenic variants with further evaluation of REAH and SH needed to elucidate driver genetic events.

Simultaneous Nasal Septal Schneiderian Papilloma and Respiratory Epithelial Adenomatoid Hamartoma.

Schneiderian papilloma is typically found on the lateral nasal wall, but, in rare cases, can be located at the mucosa of the nasal septum. Respiratory epithelial adenomatoid hamartoma (REAH) is rare, non-neoplastic lesion characterized by an abnormal proliferation of glandular part of the respiratory nasal mucosa. We present a case of a 42-year-old man with five-year history of bilateral nasal obstruction, rhinorrhea, epistaxis, and hyposmia. On endoscopic examination, we found lobulated, yellowish-pink soft tissue masses on both sides of the nasal septum. The results of histopathological analyses showed the characteristics of Schneiderian papilloma in the lesion excised from the right and REAH in the lesion excised from the left side of the nasal cavity. To our knowledge, cases of simultaneous presentation of Schneiderian papilloma and REAH on opposite sides of the nasal septum have not been published so far. An appropriate cooperation of rhinologist and pathologist allows for a correct diagnosis of these rare and unusual lesions.

Predictors and prognosis of respiratory epithelial adenomatoid hamartoma in sinonasal cavities.

ObjectiveRespiratory epithelial adenomatoid hamartoma (REAH) is classified as a histopathologic diagnosis and often identified in sinus surgery for chronic rhinosinusitis (CRS). The purpose of this study was to clarify the frequency and predictors of REAH and prognosis of CRS with REAH in CRS cases.MethodsIn the first study, we histologically reviewed sinonasal polyps and mucosal tissue specimens obtained from patients who underwent endoscopic sinus surgery (ESS) for CRS to reveal how many REAH were involved in ESS cases. We compared REAH and non‐REAH groups in terms of preoperative symptoms and endoscopic, imaging and blood examination findings to elucidate predictors of REAH genesis. In the second study, we compared the data 3 months after surgery such as endoscopic and imaging findings and olfactory test to evaluate prognosis of CRS with REAH.ResultsThe prevalence of REAH was 15.5% of all 304 cases in the first and second studies combined. Higher polyp score in the middle meatus was an independent predictor of the presence of REAH (p = .02). Presence of REAH was significantly associated with the enlargement of olfactory cleft polyps (p < .01), increasing postoperative scores of standard olfactory tests (p = .03), and decline of ratio of improvement (p < .01) measured using T&T olfactometry.ConclusionsHigher polyp score in the middle meatus is an independent predictor of REAH. Olfactory function is difficult to recover after surgery in REAH patients because it is associated with recurrent polyps in the olfactory cleft.

Respiratory epithelial adenomatoid hamartomas of the sinonasal tract: A histopathological analysis of 50 patients.

Respiratory epithelial adenomatoid hamartoma (REAH) is a benign lesion of the nasal cavity and paranasal sinuses. Here, we report the clinicopathological characteristics of REAH identified in 2065 cases with nasal/paranasal polypoid lesions treated with endoscopic sinus surgery (ESS) at our hospital from 2008 to 2021. Cases including the olfactory area were reviewed and 50 patients of REAH were identified pathologically (50/2065, 2.4%). The average age was 58.9 years old and the male/female ratio was 45/5. Grossly, REAH showed a whitish surface and elastic firm consistency. The histopathological characteristics included proliferation of small to medium-sized glands composed of ciliated respiratory epithelium containing goblet cells; thickening of the basement membrane compared to that for inverted papilloma (9.6 ± 2.4 vs. 1.3 ± 1.6 µm, p < 0.001); and no intra-epithelial neutrophilic infiltration. Among the REAH cases, 81% were associated with sinonasal inflammatory polyps. Many olfactory cleft polyps were REAH (38/98, 39%). The rate of REAH found in ESS in the last 7 years was higher than that in the first 7 years (3.17% vs. 1.62%, p = 0.032). Our results in Japanese patients are similar to those found in other countries, including male predominance. REAH is relatively common and that 39% of polyps taken from olfactory clefts are REAH.

CD163 Immunohistochemical "Circle Sign" Staining Pattern Differentiates Sinonasal Papillomas From Morphologically Similar Non-neoplastic Lesions.

Sinonasal papillomas are a diverse group of benign epithelial neoplasms of the sinonasal tract. Inverted papilloma, in particular, must be distinguished from other lesions with no malignant potential. The aim of this study was to distinguish sinonasal papillomas from morphologically similar lesions using CD163 immunostaining. Cases from a 19-year period were identified. These included 49 inverted, 10 exophytic, and 12 oncocytic papillomas, 21 chronic sinusitides with squamous metaplasia, 27 inflammatory polyps, 5 verrucae vulgares, 5 respiratory epithelial adenomatoid hamartomas, and 6 DEK::AFF2 carcinomas of the sinonasal tract. A subset of biopsy cases (8 inverted papillomas, 5 inflammatory polyps) was separately analyzed. CD163 immunohistochemistry (IHC) was performed. A unique "circle" staining pattern was identified in the surface epithelium. After locating a hotspot, circles were quantified in 10 consecutive high-power fields. Circles were present in 66/71 (93%) cases of sinonasal papilloma, with a mean of 35 circles/10HPF (range: 0 to 160/10HPF) and a median of 19 circles/10HPF. Circles were present in 20/58 (34%) non-neoplastic cases, with a mean of 2 circles/10HPF (range: 0 to 27/10HPF) and a median of 0. Considering all resection and biopsy cases, performance for distinguishing papillomas from non-neoplastic lesions was best at a cutoff of 10 circles/10HPF (2-tailed P <0.0001) with sensitivity, specificity, positive predictive value, and negative predictive value of 66.2%, 93.1%, 92.1%, and 69.2%, respectively. The results were similar in the biopsy subset. One other neoplastic entity, the DEK::AFF2 carcinomas, also showed prominent CD163 circle staining. In summary, sinonasal papillomas demonstrate extensive CD163 "circle" staining in the epithelium compared with the non-neoplastic lesions studied. As such, the "circle sign" on CD163 IHC may be helpful in distinguishing between diagnoses, particularly on small biopsies or equivocal specimens.

A case report of adolescent respiratory epithelial adenomatoid hamartoma.

Hamartomas are common in the lung, kidney, liver, spleen, and, but rare, in the sinonasal tract. Respiratory epithelial adenomatoid hamartomas (REAHs) are benign lesions common in men aged 30 to 90years. Approximately 70% of REAHs in the head and neck region originate from the posterior nasal septum. We present an unusual case of REAH originating from the maxillary sinus and extending to the nasopharynx of an adolescent boy.A 17-year-old boy without any salient medical history presented to our department with nasal obstruction that had persisted for 7years as well as greenish nasal discharge, hyposmia, and a complaint of fetid smell. Sinoscopy of the osteomeatal complex (OMC) revealed bilateral mucopus and a large right polypoid tumor extending into the nasopharynx. Computed tomography of the paranasal sinuses revealed soft-tissue opacification around the right OMC, frontal sinus, ethmoid sinus, maxillary sinus, and nasopharynx. We performed bilateral endoscopic sinus surgery. REAH and chronic rhinosinusitis with nasal polyps were diagnosed on the basis of a pathology report. No evidence of recurrence was observed by 6months after surgery, and his hyposmia, nasal obstruction, and purulent nasal discharge were alleviated considerably. Accurate diagnosis based on pathology is essential for determining the optimal treatment, which for REAH is complete surgical excision.

A bony nasal polyp: Chondroosseous respiratory epithelial hamartoma – A case report

Chondroosseous respiratory epithelial hamartoma (CORE hamartoma), also known as chondroosseous respiratory epithelial adenomatoid hamartoma (COREAH), is a rare benign growth arising from sinonasal cavity and nasopharynx. It occurs in all age groups, including children and older adults. It is related to respiratory epithelial adenomatoid hamartoma (REAH) histologically. REAH is a polypoid or exophytic lesion with proliferation of glands, lined by respiratory epithelium, often mixed with goblet cells.

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

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.

Prevalence of respiratory epithelial adenomatoid hamartomas (REAH) associated with nasal polyposis: an epidemiological study – how to diagnose

ObjectivesTo highlight the prevalence of respiratory epithelial adenomatoid hamartomas in the olfactory cleft of patients with nasal polyposis. To demonstrate characteristics indicative of hamartoma on the CT scans of paranasal sinuses during surgery and in histopathological exams. MethodsCross-sectional study carried out in Hospital das Clínicas da UFMG and Núcleo de Otorrino BH. We performed 114 nasal endoscopic surgeries for polyposis, between February 2015 and November 2019. We assessed the olfactory cleft width in all preoperative CT scans. Upon seeing an indication of hamartoma on the CT scan, we took a tissue sample from the olfactory cleft during the surgery and sent for histopathological exam. We referred the samples to a pathologist experienced in the anatomopathological diagnosis of respiratory epithelial adenomatoid hamartomas. ResultsOf the 114 patients with polyposis, 54 (47.4%) had olfactory cleft enlargement and, 100% of them had tissue with a dense and hardened polypoid aspect, with a slight cerebriform appearance in this region during the surgery. Histology confirmed a respiratory epithelial adenomatoid hamartoma. ConclusionThis observation suggests that the presence of hamartomas in polyposis is common, but underdiagnosed. Level of evidenceStep 3 (Level 3).

Not just another nasal polyp: Chondro-osseous respiratory epithelial adenomatoid hamartomas of the sinonasal tract.

ObjectivesChondro‐osseous respiratory epithelial adenomatoid hamartomas (COREAH) are extremely rare benign lesions in the sinonasal cavity. We aim to (a) report two cases of COREAH and (b) perform a literature review with a summary on previously published cases of COREAH till August 2019.MethodsA literature review identified 16 cases, but only 12 cases from publications from 2005 to 2019 were included in analysis. In addition, we report two cases of COREAH from our tertiary academic medical center.ResultsCOREAH is found in patients from 3 to 83 years old. It originates from various sites in the nasal cavity and sinuses but the most common location is the lateral nasal wall. Calcification on imaging is a common finding.ConclusionCOREAH is an uncommon entity that is important to recognize and distinguish from other sinister nasal masses. It is a slow growing benign lesion which lacks aggressive features on imaging. Future studies are needed to evaluate the possibility of an underlying genetic predisposition.Level of Evidence4.

ХОНДРО-КОСТНАЯ РЕСПИРАТОРНАЯ ЭПИТЕЛИАЛЬНАЯ АДЕНОМАТОИДНАЯ ГАМАРТОМА

Hamartomas of sinonasal tract are a very rare and poorly studied phenomenon, even within the framework of world medicine. In the classification of tumors of the head and neck according to the WHO, in the section of nasal papillomas, only respiratory epithelial adenomatoid hamartoma is mentioned, there is no information about the other types of nasal hamartomas. The peculiarity of this clinical case is the difficulty in verifying the type of hamartoma of the sinonasal tract (respiratory epithelial adenomatoid, seromucinous, chondromesenchymal or chondro-osseous respiratory epithelial adenomatoid), respectively, and in determining the tactics of subsequent treatment, due to the rarity of this type in the world and the possibility of malignant transformation. A 15-year-old patient was diagnosed with chondro-osseous respiratory epithelial adenomatoid hamartoma. According to the literature, only 12 such clinical cases were recorded in the world. The absence of specific radiological and laboratory markers, the clinical picture of the disease similar to other diseases of the nose and paranasal sinuses - all this complicates early diagnosis, and emphasizes the need to include this type of neoplasm in the differential diagnostic range in children and adults with masses of the nasal cavity.

Two cases of respiratory epithelial adenomatoid hamartoma diagnosed by postoperative pathology

Two cases of respiratory epithelial adenomatoid hamartoma diagnosed by postoperative pathology