Ear lobule reduction using a sub-antitragal groove technique in patients with angiolymphoid hyperplasia with eosinophilia on the earlobe: a case report and literature review.

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare condition characterized by nodular lesions predominantly in the head and neck region, often causing discomfort or pain. Treatment remains challenging because of its rarity and the lack of established guidelines. This report presents a case of ALHE affecting the earlobes that was successfully managed using ear lobule reduction surgery and subsequent intralesional steroid injections. A 31-year-old woman with a history of recurrent earlobe masses underwent a partial excision to avoid the loss of the earlobe. Histopathological examination confirmed Kimura disease, a variant of ALHE. Subsequent local methylprednisolone injections effectively controlled the remaining lesions, resulting in significant size reduction without notching. Various treatment modalities have been attempted for this condition; however, recurrence rates remain high. Surgical resection combined with intralesional corticosteroid injections is the preferred approach. In this case, a sub-antitragal groove technique for earlobe reduction was employed to preserve the lateral edge of the ear lobule, minimize the risk of deformity, and achieve a predictable outcome. The sub-antitragal groove technique offers an approach to reduce earlobe size without compromising aesthetics. Further research is required to elucidate the pathogenesis of ALHE and establish standardized treatment protocols for this rare condition.

A Multidisciplinary Skull Base Board for Tumour and Non-Tumour Diseases: Initial Experiences.

The skull base is the area where various cancerous and non-cancerous diseases occur and represents the intersection of several medical fields. The key is an integrated treatment by specialists of multiple disciplines. We prospectively analysed patients with a skull base disease between August 2022 and 2023 and presented to the Multidisciplinary Skull Base Board (MDT-SB), which takes place once a month hybridly (in-person and remotely). Thirty-nine patients (median age of 58.2 years) were included, of which twelve (30.8%) had a benign tumour, twelve (30.8%) had a malignant tumour, five had an infection (12.8%), and ten (25.6%) had other diseases. For each patient, at least two otorhinolaryngologists, a neurosurgeon, and a neuroradiologist, as well as an infectious disease specialist, a paediatrician, an oculoplastic surgeon, a maxillofacial surgeon, and a pathologist were involved in 10%, 8%, 8%, 3%, and 3% of cases, respectively. In fifteen patients (38%), the MDT-SB suggested surgical treatment; in fourteen (36%), radiological follow-ups; in five (13%), non-surgical treatments; in two, conservative treatments (5%); in two (5%), surgical and conservative treatments; and in one (3%), a biopsy. Non-cancerous and cancerous diseases of the skull base in adults and children should be presented to the MDT-SB, which consists of at least an otolaryngologist, a neurosurgeon, and a neuroradiologist.

Dermal cylindroma of the external auditory canal.

External auditory canal (EAC) cylindroma is a rare tumor that mainly presents as a painless mass over the lateral aspect of the ear canal. They have been designated under different nomenclatures in the literature, and controversies persist about their etiology and histogenesis. Moreover, a clinical diagnosis of EAC cylindroma is often challenging because of their rarity and a close resemblance with other adnexal benign and malignant tumors. None of the previous authors have extensively reviewed the dermal cylindroma of the EAC. We provide an extensive review involving PubMed and Google Scholar and report by Preferred Reporting Items for Systematic Reviews and Meta-Analyses standards. A total of 8 cases are included in the current study. The mean age is 55.13 years. There are six females and two males. The left and right ear are involved in 62.50% and 37.50% of cases, respectively. The most common sign/symptom is painless mass (50%). Five authors reported a primary lesion (62.50%), while the remaining 3 reported a recurrent tumor (37.50%). Benign versus malignant cylindroma is reported in 87.50% and 12.50% of cases, respectively. All, except one case, reported a solitary swelling. Surgical excision was employed in all the cases. Primary defect closure versus defect closure with local/distant skin graft /flap is utilized in 37.50% and 62.50% of cases, respectively.

Late Recurrence of a Rare Middle Ear Neuroendocrine Tumor With an Intracranial Extension to the Temporal Fossa: A Case Report.

We report a clinical case of an extremely rare neuroendocrine tumor of the right middle ear (MeNET) that recurred after 13 years with a local extension into the right temporal fossa. In the current medical literature, there are approximately 150 cases of MeNETs and even fewer cases with more than 10 years of follow-up, recurrence, and intracranial tumor progression. Therefore, we believe that this paper can make an important contribution to the existing and future knowledge about this disease. The purpose of this article is to present our experience in treating such a rare neoplasm in a 35-year-old woman. The patient initially complained of worsening hearing in her right ear over the past year. The final diagnosis was made based on the findings of computed tomography (CT), magnetic resonance imaging (MRI), and histological and immunohistochemical evaluation of excisional biopsies of the original and recurrent tumors. The primary tumor masses were removed with clear resection margins, and the ossicular chain was reconstructed. The patient has been monitored clinically and radiologically with temporal bone CTs every year and MRIs three times in general since then. A postoperative audiogram showed remaining mixed hearing loss in the right ear that eventually worsened as the tumor grew. Tumor recurrence and progression after 156 months (13 years) were seen on CT and MRI, requiring further treatment. After resection of the recurrent tumor, paresis of the right facial nerve developed, which was treated with dexamethasone. The surgical treatment caused the initial symptoms to disappear, but the facial nerve paresis persisted with mild functional improvement. The patient is not receiving adjuvant radiotherapy and is being monitored closely because the tumor may recur in the future.

Dermal cylindroma of the external auditory canal

ABSTRACT External auditory canal (EAC) cylindroma is a rare tumor that mainly presents as a painless mass over the lateral aspect of the ear canal. They have been designated under different nomenclatures in the literature, and controversies persist about their etiology and histogenesis. Moreover, a clinical diagnosis of EAC cylindroma is often challenging because of their rarity and a close resemblance with other adnexal benign and malignant tumors. None of the previous authors have extensively reviewed the dermal cylindroma of the EAC. We provide an extensive review involving PubMed and Google Scholar and report by Preferred Reporting Items for Systematic Reviews and Meta-Analyses standards. A total of 8 cases are included in the current study. The mean age is 55.13 years. There are six females and two males. The left and right ear are involved in 62.50% and 37.50% of cases, respectively. The most common sign/symptom is painless mass (50%). Five authors reported a primary lesion (62.50%), while the remaining 3 reported a recurrent tumor (37.50%). Benign versus malignant cylindroma is reported in 87.50% and 12.50% of cases, respectively. All, except one case, reported a solitary swelling. Surgical excision was employed in all the cases. Primary defect closure versus defect closure with local/distant skin graft /flap is utilized in 37.50% and 62.50% of cases, respectively.

An extensive squamous cell carcinoma of the auricle: From curative to reconstructive treatment. A case report.

Squamous cell carcinoma (SCC) of the auricle is a rare and aggressive entity of cell carcinomas. It is mostly identified in older males with history of sun exposure. After histopathological confirmation, the initial assessment which consists of clinical and radiological evaluation will determine the therapeutic strategy. We report the case of a neglected SCC of the left pinna with parotid and temporo-mandibular infiltration. After surgical resection, the patient underwent a two staged reconstructive surgery. This was followed later on with radiotherapy and chemotherapy. The evolution was favourable for our patient during an 18 months follow-up. This case report underlines the importance of both curative and reconstructive surgery in successfully treating locally advanced tumors of the temporal bone.

Immunohistochemical Expression Pattern of Theragnostic Targets SSTR2 and PSMA in Endolymphatic Sac Tumors: A Single Institution Case Series.

BACKGROUND: Endolymphatic sac tumors are rare neoplasia characterized by slow growth. However, their clinical impact should not be underestimated, considering their potential for local aggressive behavior and strong association with von Hippel-Lindau syndrome. Therefore, early detection with emerging theragnostic examinations such as 68Ga-DOTATATE-PET/CT might improve patient management and reduce morbidity. METHODS: We report the clinicopathological features of seven endolymphatic sac tumors. In this cohort, we performed immunohistochemical analysis of somatostatin receptor 2A (SSTR2A) and prostate specific membrane antigen (PSMA) protein expression patterns; two targets providing rationale for novel imaging modalities such as PSMA- or SSTR-targeted PET. RESULTS: The tumor cells of all cases were negative for prostate specific membrane antigen and somatostatin receptor 2A, however immunolabeling was consistently detected in intratumoral endothelial cells of endolymphatic sac tumors for PSMA (7/7 cases, 100%), and for SSTR2A (5/7 cases, 71%). CONCLUSIONS: Our results show a high rate of PSMA and SSTR2A expression in the tumor vasculature of endolymphatic sac tumors. PSMA and SSTR2A can be targeted with appropriate radioligands for diagnostic and therapeutic purposes. This finding provides a rationale for prospective clinical studies to test this approach as a sensitive screening tool for patients with suspected endolymphatic sac tumors including an improved management of von Hippel-Lindau syndrome.

[Fibroproliferative trichoepithelioma grows behind the ear: a case report].

Trichoepithelioma is a kind of rare benign tumor of the skin that occurs in direct sunlight and is usually small. Large solitary trichoepithelioma behind the ear is rare. The pathological result of this patient was fibroproliferative trichoepithelioma. This type of tumor is rare and has no specific tumor markers. Diagnosis can be decided based on its histological characteristics. Combined with the appearance and histological characteristics of other types of trichoepithelioma, it can be concluded that this tumor is a special type of connective tissue hyperplastic trichoepithelioma.

[Clinical features and prognostic factors for early-stage external auditory canal carcinoma].

Objective:To analyze the clinical features and the prognostic factors of early-stage external auditory canal carcinoma. Methods:Data from 36 patients with early-stage external auditory canal carcinoma（T1, T2） treated in Department of Otolaryngology, Xijing Hospital, Air Force Military Medical University from January 2008 to June 2020 were reviewed retrospectively, including clinical manifestations, surgical and treatment methods, pathological types and disease status. The relationship between survival rate and the prognostic factors was compared using Kaplan-Meier method, and the independent risk factors were analyzed by Cox proportional hazards model. Results:There were 36 patents with early-stage external auditory canal carcinoma. The common initial symptoms were otalgia（66.7%）, otorrhea（41.7%） and hearing loss（30.6%）. The most common histopathologic types were adenoid cystic carcinoma（50.0%） and squamous cell carcinoma（33.3%）. Among the patients, 21 patients（58.3%） were initially treated, 9 patients（25.0%） were treated with salvage therapy, and 6 patients（16.7%） were re-surgery after recurrence. The 5-year disease-specific survival（DSS）, disease-free survival（DFS） and relapse-free survival（RFS） were 82.3%, 64.0% and 73.0% respectively. Seven cases （19.4%） relapsed after surgery. For 5-year survival rate, the lateral temporal bone resection with superficial parotidectomy（DSS 91.7%, DFS 83.9%） is higher than the lateral temporal bone resection only（DSS 77.8%, DFS 55.6%） and sleeve resection（DSS 75.0%, DFS 56.0%）, but there was no significant difference（P>0.05）. In these patients, the postoperative radiotherapy and disease status had no significant impact on the survival rate. Additionally, there was no obvious correlation between recurrence and age, gender, stage, histopathologic types, operation methods and postoperative radiotherapy（P>0.05）. But there were significant differences between histopathologic types and DSS or DFS（P<0.05）. Multivariate regression analysis showed that histopathologic type was an independent prognostic factor for DFS. Conclusion:There are no specific clinical manifestations for early-stage external auditory canal carcinoma, such as otalgia and otorrhea. Histopathologic types have a direct impact on the patients'prognosis. Thus, individualized treatment should be applied based on pathologic findings to improve the survival rate.

Auricular reconstruction after Mohs micrographic surgery: analysis of 101 cases

Abstract Background: The ear is a region that has a high prevalence of cutaneous carcinomas and several guidelines indicate Mohs micrographic surgery as the first-choice treatment in such cases. Although the technique allows maximum preservation of healthy tissue, many auricular surgical wounds constitute a challenge due to the peculiar local anatomy, with evident curves and reliefs. Auricular reconstruction should prioritize function before aesthetics, but without leaving the latter aside, since postoperative distortions can have a significant psychological impact. Objective: To describe the authors' experience in auricular reconstruction after Mohs surgery and to evaluate the most frequently used repair methods. Methods: Retrospective study of consecutive cases submitted to Mohs surgery and auricular reconstruction, over a period of 3 years. Results: One hundred and one cases were included and the most common repair method was primary closure (n = 35), followed by full-thickness skin graft (n = 30) and flaps (n = 24). In thirty cases, reconstruction methods were associated. Seven patients had complications (partial graft necrosis, postoperative bleeding or infection). Study limitations: Retrospective design and the absence of long-term follow-up of some cases. Conclusions: The dermatologic surgeon should be familiarized with different options for auricular reconstruction. Primary closure and skin grafts were the most frequently used repair methods.

Auricular reconstruction after Mohs micrographic surgery: analysis of 101 cases.

BACKGROUND: The ear is a region that has a high prevalence of cutaneous carcinomas and several guidelines indicate Mohs micrographic surgery as the first-choice treatment in such cases. Although the technique allows maximum preservation of healthy tissue, many auricular surgical wounds constitute a challenge due to the peculiar local anatomy, with evident curves and reliefs. Auricular reconstruction should prioritize function before aesthetics, but without leaving the latter aside, since postoperative distortions can have a significant psychological impact. OBJECTIVE: To describe the authors' experience in auricular reconstruction after Mohs surgery and to evaluate the most frequently used repair methods. METHODS: Retrospective study of consecutive cases submitted to Mohs surgery and auricular reconstruction, over a period of 3 years. RESULTS: One hundred and one cases were included and the most common repair method was primary closure (n = 35), followed by full-thickness skin graft (n = 30) and flaps (n = 24). In thirty cases, reconstruction methods were associated. Seven patients had complications (partial graft necrosis, postoperative bleeding or infection). STUDY LIMITATIONS: Retrospective design and the absence of long-term follow-up of some cases. CONCLUSIONS: The dermatologic surgeon should be familiarized with different options for auricular reconstruction. Primary closure and skin grafts were the most frequently used repair methods.

Malignancies requiring temporal bone resection: An Australian single-institution experience.

BACKGROUND: Malignancies in and around the temporal bone are aggressive and difficult to manage. In Queensland (Australia), where skin cancer rates are exceedingly high, tumours extending to the temporal bone from surrounding structures occur more commonly than primary cancers. Yet, a paucity of evidence exists as to their management and outcomes. This study aimed to review an Australian centre's experience of managing temporal and peritemporal bone malignancies, reporting on patient and tumour characteristics, treatment, and survival outcomes. METHODS: Retrospective analysis of patients with primary temporal bone cancer and cancers extending to the temporal bone managed by the Queensland Skull Base Unit (Princess Alexandra Hospital) between 2000 and 2019. RESULTS: A total of 222 patients were identified, of which 203 (91.4%) had cutaneous primaries, with 167 (75.2%) being squamous cell carcinoma (SCC). 73.9% presented with locoregionally recurrent or residual disease. Secondary tumours (92.8%) were 12 times more frequent than primary malignancies (7.2%), with the preauricular subsite the most common (45.5%). In the 201 patients (90.5%) who underwent curative intent surgery, 5-year overall survival, disease-free survival (DFS), and disease-specific survival was 46.6%, 52.2%, and 65.9%, respectively. The preauricular subsite (p = 0.004), melanoma (vs. SCC, p = 0.027), involved margins (p < 0.001), and pathologically involved nodes (p < 0.001) were associated with significantly worse DFS. CONCLUSION: This is one of the largest studies of temporal bone malignancy in the literature, comprised primarily of secondary cutaneous malignancies. Although clear differences in epidemiological characteristics exist around the world, survival remains poor. Treatment should focus on achieving a clear margin of resection to optimize outcomes.

Retalho retroauricular: um relato de caso / Retroauricular flap: case report

O carcinoma basocelular (CBC) pode acometer a região auricular em diversas localizações e tamanhos variados, com ou sem comprometimento de cartilagem. Algumas vezes, as ressecções são pequenas, e as reconstruções são simples. Outras vezes, em lesões maiores, as reconstruções são mais complexas, considerando-se a restrita quantidade de pele do local e a peculiaridade das estruturas envolvidas. Relatamos o caso de um retalho retroauricular estagiado em dois tempos, após a exérese de um CBC na face posterior do pavilhão auricular direito com comprometimento parcial da cartilagem, evoluindo com ótimo resultado estético e funcional, tanto do pavilhão auricular quanto da área doadora

Basal cell carcinoma (BCC) can affect the auricular region in several locations and sizes, with or without cartilage involvement. Sometimes resections are small and reconstructions are simple. Other times, when the lesions are more extensive, reconstructions are more complex, considering the limited amount of skin in the area and the peculiarity of the structures involved. We report a case of a retroauricular flap staged in two stages, after the excision of a BCC on the posterior face of the auricle, with partial involvement of the cartilage, showing a great aesthetic and functional result both in the recipient and donor area.

[Basal squamous cell carcinoma of external auditory meatus: a case report].

Summary The patient developed repeated itching and scabbing at the mouth of the left external auditory canal 5 years ago. In the last two years, the tumor is enlarged. After admission, the left external auditory canal can be seen as a reddish mass, brittle and easy to bleed. CT of temporal bone showed that the soft tissue shadow of left external auricle and external auditory canal was thickened. Postoperative pathological findings: (left external auditory canal) basal cell squamous cell carcinoma. According to the history, physical examination and laboratory examination, the diagnosis is considered as basal squamous cell carcinoma of the external auditory meatus.

Reconstrução da hélice: tripla transposição / Helix reconstruction: triple transposition

O câncer da pele não melanoma é a neoplasia maligna mais comum em humanos, sendo os carcinomas basocelulares responsáveis por aproximadamente 80% dos casos. A exposição cumulativa à radiação ultravioleta é o principal fator de risco associado ao carcinomas basocelulares, ocorrendo maior incidência nas áreas fotoexpostas, incluindo a região auricular. A região auricular é uma unidade cosmética nobre, cujas peculiaridades anatômicas tornam as cirurgias, com necessidade de excisão de grande quantidade de tecido, um desafio para o cirurgião dermatológico. Os autores apresentam caso de reconstrução da hélice e região retroauricular por meio de retalho com tripla transposição.

Non-melanoma skin cancer is the most common neoplasia in humans, and basal cell carcinomas (BCC) account for approximately 80% of cases. Cumulative exposure to ultraviolet radiation (UV) is the main risk factor associated to BCC, with a higher incidence in photoexposed areas, including the auricular region. The auricular region is a noble cosmetic unit, with anatomical peculiarities that lead surgeries to require the excision of a large amount of tissue, presenting a challenge for the dermatological surgeon. The authors present a case of helix reconstruction with a triple transposition flap.

Retalho pré-auricular tunelizado insular de base superior para reconstrução de defeitos auriculares / Superiorly-based, preauricular tunneled insular flap for reconstruction of auricular defects

O complexo auricular representa sede frequente de lesões, especialmente de origem tumoral ou resultante de eventos traumáticos. Diversas são as opções cirúrgicas para restaurar a integridade anatômica da orelha. Relatamos o caso de um paciente que apresentou lesões tumorais em mais de uma ocasião na face anterior da orelha esquerda e que precisou de intervenção cirúrgica para a correção do defeito gerado pela excisão tumoral. Como procedimento proposto, planejamos e executamos um retalho insular de base superior, que por meio de um túnel criado na projeção da fossa triangular foi rodado para a fossa escafoide, reparando-a em um único tempo cirúrgico, apresentando resultados estético e funcional satisfatórios ao paciente.

The auricular complex is commonly affected by tumors or traumatic events. Several surgical options are available for restoration of ear anatomy. We report the case of a patient who presented with tumor lesions on more than one occasion in the anterior left ear and required surgical intervention to correct the defect generated by excision. As a proposed procedure, we designed and executed a superiorly-based insular flap that was rotated to the scaphoid fossa through a tunnel created in the projection of the triangular fossa; this was performed in a single stage, and the patient was satisfied with the aesthetic and functional results.

Reconstrução da hélice da orelha sem triângulo de compensação / Ear's helix reconstruction without the compensation triangle

Os cânceres da pele na região da cabeça e do pescoço correspondem a 70-75% dos tumores cutâneos malignos, e, destes, 80% são do tipo carcinoma basocelular. O pavilhão auricular é a localização dos tumores cutâneos malignos em percentual que varia de três a 6% dos casos. Relatamos técnica alternativa para reconstrução da hélice após exérese de tumores sem a utilização do triângulo de compensação clássico, de modo a proporcionar cicatriz na dobra da hélice, com melhor resultado estético, sem retrações inestéticas ou cicatrizes transversais à hélice.

Skin cancers in the head and neck region account for 70-75% of malignant cutaneous tumors, of which 80% are basal cell carcinomas. Between 3-6% of malignant cutaneous tumors occur in the auricular pavilion. The authors of the present paper report an alternative technique for reconstructing the ear helix after excision of tumors, without the use of the classic compensation triangle. This method allows that the scar be located in the helical sulcus, resulting in a better aesthetic result, without unaesthetic retractions or scars located transversely to the helix.

Prognostic Factors Affecting Surgical Outcomes in Squamous Cell Carcinoma of External Auditory Canal.

OBJECTIVES: Carcinomas of the external auditory canal (EAC) are rare, and management remains challenging. Previous studies seeking prognostic factors for EAC cancers included cancers other than carcinomas. In this study, we analyzed the treatment outcomes of, prognostic factors for, and survival rates associated with specifically squamous cell carcinoma (SCC) of the EAC. METHODS: A retrospective review of 26 consecutive patients diagnosed with SCCs of the EAC in a 10-year period was performed in terms of clinical presentation, stage, choice of surgical procedure, and adjunct therapy. Overall survival (OS) and recurrence-free survival (RFS) were calculated and univariate analysis of prognostic factors was performed. RESULTS: The median age of the 26 patients with SCCs of the EAC was 63 years (range, 40 to 72 years), and 16 males and 10 females were included. According to the modified University of Pittsburgh staging system, the T stages were T1 in 11, T2 in six, T3 in four, and T4 in five cases. The surgical procedures employed were wide excision in three cases, lateral temporal bone resection (LTBR) in 17, and extended LTBR in four, and subtotal temporal bone resection in two. Two patients underwent neoadjuvant chemotherapy, and two underwent adjuvant chemotherapy. One patient received preoperative radiation therapy, and eleven received postoperative radiation therapy. Of the possibly prognostic factors examined, advanced preoperative T stage and advanced overall stage were significant predictors of RFS, but not of OS. CONCLUSION: The advanced T stage and overall stage were associated with decreased survival after surgical treatment in patients with SCC of the EAC, highlighting the importance of clinical vigilance and early detection.

A rare development of tumoral calcinosis of the ear auricle.

Tumoral calcinosis is a condition characterized by deposition of calcium salts in the skin and sub- cutaneous tissue, commonly found around the joints. However, tumoral calcinosis of the auricle is extremely rare. We present the case of a 13-year-old boy with tumoral calcinosis of the helix of the ear auricle. A 13-year-old boy presented with a 10-year history of an enlarging mass on the left auricle. The mass was hard, non-tender, and non-compressible. The patient had no history of trauma. Complete surgical excision and pathological examination of the specimen was performed. The final diagnosis of the excised mass was tumoral calcinosis. After 9 months of follow-up, there were no signs of recurrence of the tumor and the patient was satisfied with the surgical results. Tumoral calcinosis of the auricle is extremely rare and may be misdiagnosed as other tumors. Pathological examination is essential for definitive diagnosis and complete surgical excision should be considered as the treatment of choice.

A rare development of tumoral calcinosis of the ear auricle

Tumoral calcinosis is a condition characterized by deposition of calcium salts in the skin and sub- cutaneous tissue, commonly found around the joints. However, tumoral calcinosis of the auricle is extremely rare. We present the case of a 13-year-old boy with tumoral calcinosis of the helix of the ear auricle. A 13-year-old boy presented with a 10-year history of an enlarging mass on the left auricle. The mass was hard, non-tender, and non-compressible. The patient had no history of trauma. Complete surgical excision and pathological examination of the specimen was performed. The final diagnosis of the excised mass was tumoral calcinosis. After 9 months of follow-up, there were no signs of recurrence of the tumor and the patient was satisfied with the surgical results. Tumoral calcinosis of the auricle is extremely rare and may be misdiagnosed as other tumors. Pathological examination is essential for definitive diagnosis and complete surgical excision should be considered as the treatment of choice.