Wide Local Excision Research Articles

ThTP4.10 Evaluating the Outcomes of Anal Squamous Cell Carcinoma with Curative Intent- a 10-Year Experience

Abstract Background Our 10-year experience in treating anal squamous cell carcinoma (ASCC) with curative intent. Patients and Method Clinical and pathological data were analysed between January 2011 and December 2019. All patients underwent the standard workup and treated accordingly. Patients were followed up according to the network protocol. The outcome measures were clinicopathological characteristics, treatment modalities, response, recurrence, disease-free and overall survival. Results 117 patients followed up over the 10-year. 26 patients treated palliatively, 11 patients with adenocarcinoma, 1 melanoma, 1 Paget disease and 1 carcinoid tumour were excluded. Median age was 65 (38-90) years with male to female ratio of 1:1.9. 3 patients were HIV+ve and 52% of the patients had AIN of varying degree of dysplasia. AJCC stages were I (16), II (18), IIIA (19), IIIB (9) and IV (15). 88.3%% of the patients had radical chemoradiotherapy while 9 patients with tumour ≤2cm underwent wide local excision. 81.8% of the patients treated with radical chemoradiotherapy had complete response while (5/77) had partial response.4 patients with incomplete response underwent salvage APER. Recurrence rate was 13%. The overall mean survival time was 99.82 (95% CI 88.87-110.77) months with 5-year survival rate 78.0%. Overall recurrence rate was 13.0%. Mean disease-free survival time in 77 patients was 111.82 (95% CI 102.38-121;26) months. 75.3% of the patients were alive at the end of the study with an overall mortality rate was 24.7% and disease-specific mortality was 15.6%. Conclusion Recurrent anal SCC occurred in 1 in 10 after radical chemo-radiotherapy with disease-specific mortality of 26%.

Use of the Superthin Superficial Circumflex Iliac Artery Perforator Flap for Reconstruction of Lower Lip Defects.

Wide local excision remains the main treatment modality for primary lip cancer, which often results in considerable deficits of lip tissue and requires reconstruction using free tissue transfer. Although diverse free flaps, such as radial forearm flap or anterolateral thigh flap, have been used, their application is often limited by their inherent limitations, including donor-site morbidity and flap bulkiness. This report details the authors' experience of lower lip reconstruction using a superthin superficial circumflex iliac artery perforator (SCIP) free flap. Two patients with primary squamous cell carcinoma spanning their lower lip underwent wide local excision with an 8-mm lateral excision margin and superficial layer of orbicularis oris muscle. To cover the shallow but extensive lower lip defects, a superthin SCIP flap elevating along the intraadipose tissue layer and a pure-skin perforator SCIP flap elevating along the subdermal layer were harvested. The thickness of the harvested SCIP flap was 3 mm in both cases. The microvascular anastomoses of pedicles were conducted using the inferior labial artery and adjacent subcutaneous vein as recipient vessels, and the flaps were inset. The flaps survived completely without any perfusion-related complications. The patients were discharged uneventfully. Postoperatively, both patients displayed complete functional recovery with good lip competence and aesthetically pleasing results, without requiring secondary debulking operations. The authors' results suggest that the superthin or pure-skin perforator SCIP flap might be considered as an effective method for covering shallow and wide lower lip defect.

Fibrosarcoma Presenting as Marjolin Ulcer – A Rare Case Report

Marjolin ulcer is a rare malignancy that arises in previously traumatised or chronically inflamed skin, particularly after burns, with an average latency period of 36 years. Nearly 75-90% marjolin ulcers are diagnosed as squamous cell carcinoma but other neoplasms like basal cell carcinoma, melanoma, fibrosarcoma etc have also been rarely reported. In this case, a 52-year-old male presented with a painless bleeding mass over lower chest wall rapidly increasing in size from last 2 years. He had suffered burn at the same site 20 years ago. Biopsy was reported as spindle cell neoplasm. Wide local excision revealed a poorly circumscribed subcutaneous spindle cell neoplasm exhibiting variable cellularity, fascicular to storiform architecture, mild to moderate nuclear atypia and scattered gaping thin-walled blood vessels. The neoplastic cells showed diffuse strong staining with CD34 in the less cellular area and patchy positivity in the cellular more mitotically active areas. The tumor cells were immunonegative for EMA, p63, SMA, CD99 and STAT6. The final diagnosis rendered was Dermatofibrosarcoma Protuberans dedifferentiating in to fibrosarcoma. Although sarcoma presenting as a Marjolin ulcer is exceedingly rare, it should be kept in mind in differential diagnosis of spindle cell malignancy arising at a burn site.

Rare cutaneous verrucous carcinoma in the femoral region: a case report

BackgroundVerrucous carcinoma (VC) is a well-differentiated variant of squamous cell carcinoma (SCC) characterized by slow growth and low metastatic potential. Cutaneous verrucous carcinoma (CVC) or papillomatosis cutis carcinoides is the term used for VC in other locations than the oral, anogenital, and plantar regions. This rare tumor has a contradictory “benign” histology, but markedly aggressive clinical behavior. We present this case as background knowledge to corroborate clinical course, diagnosis, histopathology, and treatment options for this infrequent disease. Case DescriptionWe report a verrucous carcinoma on a 79-year-old man’s left thigh. Clinical examination showed a fungating mass with cauliflower-like appearance measuring 5x3x1 cm. Excisional biopsy revealed exo-endophytic proliferation of well-differentiated squamous epithelium invading the dermis with marked hyperkeratosis, parakeratosis, acanthosis, papillomatosis, pushing margins, and minimal cellular atypia (T2N0M0). It was successfully treated by wide local excision, inguinal lymph node dissection, and adjuvant radiotherapy. Follow-up after 1 year showed good wound healing without any loss of function or recurrence. ConclusionCutaneous verrucous carcinoma is a subtype of a low-grade SCC and is not an SCC with a verrucous presentation. Identification of the clinicopathological features is essential to distinguish CVC from its mimics and to make an early diagnosis. Although slow growth and confusing early-stage appearances can lead to delay in diagnosis or to misdiagnosis, this case has not raised many diagnosis problems regarding the cardinal manifestations of CVC. Regardless of any available therapeutic methods, surgical excision with safety margins is still a priority and CVC requires aggressive treatment.

Spaghetti Technique Versus Wide Local Excision for Lentigo Maligna Affecting the Head and Neck Regions: Surgical Outcome and Descriptive Analysis of 79 Cases from a Single Practice Cohort.

Lentigo maligna is a subtype of melanoma in situ that typically affects the head and neck region with an increasing incidence. Margin-controlled techniques, such as spaghetti technique (ST), have gained popularity over wide local excision (WLE) with a margin of 5 mm. To evaluate the outcomes of lentigo maligna cases in the head and neck area treated by either WLE or ST in a tertiary referral hospital. The secondary goal was to describe the demographic and clinical characteristics of our series. Cohort study of patients diagnosed with lentigo maligna on the head and neck region between January 2014 and February 2022 in a tertiary hospital. In total, 79 lentigo maligna were studied, corresponding to 77 patients. Fifty-three lesions (67%) were treated with WLE and 26 (33%) with ST. The mean age of the patients was 73 years and 58% were men. Most of the tumors were located on the cheek (50%) and mean lesion diameter was 2.2 cm for the ST group and 1.2 cm for the WLE group. Mean duration follow-up was 44 months. There were two local recurrences in the WLE group (2/53; 3.7%) and none in the ST group. Both WLE and ST are appropriate surgical approaches for lentigo maligna. ST offers an efficient alternative to Mohs surgery for treating lentigo maligna in the head and neck area, especially when guided by reflectance confocal microscopy.

Deltopectoral flap reconstruction with primary repair in a patient with cutaneous neck metastasis of thyroid cancer; a case report

Background: Deltopectoral flap is the most commonly used fasciocutaneous, transpositional flap, which relies on the blood supply of intercostal perforators of the internal thoracic artery, usually requiring skin grafting to the donor site. Cutaneous metastasis of papillary thyroid carcinoma is rare. Here we report a case of deltopectoral flap reconstructive surgery in a patient with cutaneous neck metastasis of papillary thyroid carcinoma.
 Case Presentation: A 65 year old male who underwent a total thyroidectomy in 2013 for papillary thyroid carcinoma, presented with a skin neck metastasis for six months. It was an approximately 8cm diameter hemispherical lump over the left anterior root of the neck extending to the upper chest. Biopsy confirmed the diagnosis of cutaneous metastasis of thyroid papillary carcinoma. Wide local excision of the lump and deltopectoral flap reconstruction was performed without requiring skin grafting for the donor site.
 Conclusion: Effectiveness of use of deltopectoral flap as a reconstructive option for radical thyroid cancer surgeries which result in skin defect. 
 
 Keywords: Deltopectoral flap, Reconstruction, Papillary thyroid carcinoma, Cutaneous metastasis 

An unusual case of basal cell carcinoma in the scrotum

Basal cell carcinoma (BCC) is one of the most common cutaneous malignancies worldwide. Reports of BCC on non-exposed areas are relatively uncommon. In our study we present the case of a male patient with BCC in the scrotum, since 3 years, successfully treated with wide local excision. Histopathological examination confirms infiltrating BCC. Our objective is to remind specialists of the possibility of scrotal BCC when encountering scrotal lesions not responding to treatment.

Management of cutaneous melanoma: update on current treatment from surgical perspective

Incidence and mortality rate of cutaneous melanoma substantially varies across the globe depending upon early detection and management. Therapeutic developments have revolutionized the treatment. The aim of this paper is to discuss the treatment options for localized and advanced disease in the context of surgery, adjuvant, and neoadjuvant treatment. PubMed, Medline, Guidelines of European Society of Medical Oncology, National Institute of Clinical Excellence, American Joint Committee on Cancer on Melanoma, publications from 2012-2022 were searched. Low risk node negative disease (stage I and IIA) melanoma patients should have curative surgical wide local excision along with SLNB with no adjuvant therapy. High risk node negative disease (stage IIB and IIC) should be treated with curative surgery and SLNB followed by adjuvant immunotherapy. Low risk node positive disease (stage IIIA) surgical resection with SLNB followed by adjuvant systemic therapy, depending upon BRAF mutation status of tumour. High risk node positive microscopic disease (stage IIIB, IIIC, IIID) BRAF V600 mutation, primary resection with SLNB followed by nivolumab or combination of BRAF + MEK inhibitors. For BRAF wild-type tumours, adjuvant immunotherapy with programmed cell death-1 (PD-1) inhibitor. Patient with macroscopic disease that is resectable neoadjuvant combination immunotherapy followed by surgery with lymph node dissection. Metastatic disease (stage IV) regardless, adjuvant combination immunotherapy followed by maintenance nivolumab. Surgical excision is the treatment of choice for most patients with loco regional cutaneous melanoma and is curative in most cases. Checkpoint inhibitors and targeted therapies are important advances in adjuvant, neo adjuvant settings. Despite all the progress, melanoma remains challenging to treat.

Eccrine Porocarcinoma: Clinical and Histopathological Study of 14 Patients with Special Emphasis on Sentinel Lymph Node Biopsy.

Eccrine porocarcinoma is a rare skin adnexal tumour that affects elderly people. Most eccrine porocarcinomas are stage I or II according to the American Joint Committee on Cancer. The prognosis is good in early stages, but worsens when advanced. Since information on the use of sentinel lymph node biopsy in these patients is scarce, this study examined the records of all patients with eccrine porocarcinoma treated at Helsinki University Hospital during a 17-year period and focused on sentinel lymph node biopsy patients. The study identified 14 patients (9 male, 5 female). There were 2 metastases to the lymph nodes and 2 recurrences at initial referral to our institution. All primary tumours had wide local excision and 6 patients also had sentinel lymph node biopsy, of whom none had positive lymph nodes. There were no new metastases or recurrences during follow-up. Three patients died of causes other than eccrine porocarcinoma. When comparing the wide local excision only and wide local excision with sentinel lymph node biopsy groups, no parameters reached statistical significance. The decision process of the multidisciplinary tumour board meeting on whether to perform sentinel lymph node biopsy was not clear, perhaps due to the limited knowledge of eccrine porocarcinoma. Further studies and international collaboration are warranted.

Comparison of Wire and Non-Wire Localisation Techniques in Breast Cancer Surgery: A Review of the Literature with Pooled Analysis.

Background and Objectives: Wide local excision is a common procedure in the treatment of breast cancer. Wire-guided localisation (WGL) has been the gold standard for many years; however, several issues have been identified with this technique, and therefore, wire-free techniques have been developed. This scoping review synthesises the available literature comparing wire-guided localisation with the wire-free techniques used in breast-conserving cancer surgery. Materials and Methods: Multiple databases including Pubmed and MEDLINE were used to search articles between 1 January 2000 and 31 December 2022. Terms included "breast neoplasms", "margins of excision", and "reoperation". In total, 34/256 papers were selected for review. Comparisons were made between positive margins and re-excision rates of WGL with wire-free techniques including SAVI SCOUT, Magseed, ROLL, and RSL. Pooled p-values were calculated using chi-square testing to determine statistical significance. Results: Pooled analysis demonstrated statistically significant reductions in positive margins and re-excision rates when SAVI SCOUT, RSL, and ROLL were compared with WGL. When SAVI SCOUT was compared to WGL, there were fewer re-excisions {(8.6% vs. 18.8%; p = 0.0001) and positive margins (10.6% vs. 15.0%; p = 0.0105)}, respectively. This was also the case in the ROLL and RSL groups. When compared to WGL; lower re-excision rates and positive margins were noted {(12.6% vs. 20.8%; p = 0.0007), (17.0% vs. 22.9%; p = 0.0268)} for ROLL and for RSL, respectively {(6.8% vs. 14.9%),(12.36% vs. 21.4%) (p = 0.0001)}. Magseed localisation demonstrated lower rates of re-excision than WGL (13.44% vs. 15.42%; p = 0.0534), but the results were not statistically significant. Conclusions: SAVI SCOUT, Magseed, ROLL, and RSL techniques were reviewed. Pooled analysis indicates wire-free techniques, specifically SAVI SCOUT, ROLL, and RSL, provide statistically significant reductions in re-excision rates and positive margin rates compared to WGL. However, additional studies and systematic analysis are required to ascertain superiority between techniques.

Recurrent cervical adenofibroma progressing to adenosarcoma: a rare case report.

Cervical adenofibroma is a rare form of mixed mesodermal tumor that can present as cervical polyps with a tendency for local recurrence and progression. Few cases progressing to adenosarcoma have previously been reported. We report a case of cervical adenofibroma progressing to adenosarcoma, and we seek to remind clinicians of the method and importance of the differential diagnosis of this disease. A fertile woman was admitted in our department for the eighth recurrence of a cervical polypoidal mass which for the past 10 years. Recurrence of cervical adenofibroma was confirmed by ultrasound and MRI. A wide local excision under hysteroscopy was performed due to her strong desire to preserve the uterus. Surgical pathology and immunohistochemical interpretation revealed cervical adenosarcoma. A hysterectomy with conservation of the ovaries was recommended, with regular follow-ups for evidence of disease recurrence. Differential diagnoses of cervical adenofibroma are hard to prove. Adenosarcoma should be ruled out, especially in women presenting with recurrent cervical polypoidal masses. A combined histological/immunohistochemical investigation is mandatory.

Primary Cutaneous Adenoid Cystic Carcinoma Of The Chest Wall With Axillary Lymph Node Metastases And Its Management: A Case Report.

Primary cutaneous adenoid cystic carcinoma (PCACC) is a rare form of adenoid cystic carcinoma (ACC) arising commonly from the salivary gland. Less often they originate outside the head and neck region, with the scalp being the commonest cutaneous site in 40% of the cases. The presentation on the chest wall is rare, with no reports to date on axillary lymph node metastases. Here we report a case of a 65-year-old female with previously operated PCACC of the chest wall at a different center, showing uptake on positron emission tomography imaging at the site of surgical scar that w as inconclusive on needle biopsy metastasized to the axillary lymph node confirmed by needle biopsy managed with wide local excision, axillary lymph node dissection, and chest wall reconstruction with keystone island flap. The postoperative outcome was uneventful with no recurrence or axillary complications at one year's follow-up. She was advised to receive adjuvant radiotherapy; however, she refused. To conclude, though PCACC is rare, they can have an aggressive presentation, and a multidisciplinary approach is necessary for a better outcome.

Surgical management of giant skin tumor – A case report

Malignant non-melanoma skin cancers (NMSC) are of two main types: basal cell carcinoma (BCC) and squamous cell carcinoma (SCC). In rare occasions, some of the malignant skin lesions show histopathological characteristics of both BCC and SCC and are known as basosquamous carcinomas (BSC). In some cases of large tumors, extensive reconstructive surgery might be needed to correct the skin defect after the primary excision. We report a case of a 76-year-old Bulgarian male patient who presented with a neglected giant cutaneous tumor with more than a 15-year history of a growing mass in the right deltoid area. On physical exam an enormous exophytic ulcerated and crusted skin lesion measuring around 11×11cm was found. Wide local excision of the lesion with 10-mm resection margins and partial resection of the underlying deltoid muscle were performed due to signs of infiltration. A full-thickness total skin graft from the left inguinal area was harvested to cover the skin defect. Final histopathological examination showed metatypical carcinoma with mixed characteristics of SCC and BCC-BSC, with infiltration of the fatty tissue, deltoid muscle and clear margins of resection, staged as T4R0. Two and a half years after surgery there are no signs of upper arm motor dysfunction and no evidence of local recurrence and distant metastasis on a follow-up PET/CT. Following current National Comprehensive Cancer Network's guidelines for primary treatment of BCC, surgical candidates should undergo standard excision with wider surgical margins, postoperative margin assessment and second intention healing, linear repair, or skin graft. Therapeutic strategy for non-operable cases includes administration of radiotherapy or system therapy in the face of Hedgehog pathway inhibitors and programmed cell death protein 1 inhibitor. They can provide an alternative solution to unresectable or difficult-to-treat locally advanced cases of BSC. Similarly to BCC and SCC, the first-line treatment option for BCS is surgical excision, but surgical margins should be wider than those for low-risk BCC due to the infiltrative growth pattern of this tumor. Favorable esthetic outcome requires precise planning of the reconstructive technique.

Bedah Mikrografi Mohs

Mohs micrographic surgery is a specialized form of skin cancer surgery that has the highest cure rates for several cutaneous malignancies. Certain skin cancers can have small extensions or ‘‘roots’’ that may be missed if an excised tumor is serially cross-sectioned in a ‘‘bread-loaf’’ fashion, commonly performed on excision specimens. The method of Mohs micrographic surgery is unique in that the dermatologist (Mohs surgeon) acts as both surgeon and pathologist, from the preoperative considerations until the reconstruction. Since Dr. Mohs’s initial work in the 1930s, the practice of Mohs micrographic surgery has become increasingly widespread among the dermatologic surgery community worldwide and is considered the treatment of choice for many common and uncommon cutaneous neoplasms. Mohs micrographic surgery spares the maximal amount of normal tissue and is a safe procedure with very few complications, most of them managed by Mohs surgeons in their offices. Mohs micrographic surgery is the standard of care for high risks basal cell carcinomas and cutaneous squamous cell carcinoma and is commonly and increasingly used for melanoma and other rare tumors with superior cure rates. This review better familiarizes the dermatologists with the technique, explains the difference between Mohs micrographic surgery and wide local excision, and discusses its main indications.

Vulvar basal cell carcinoma: A case report and literature review

Basal cell carcinoma (BCC), is the most prevalent skin cancer with favorable prognosis, and lymphatic or hematogenous metastases are quite rare. Here we report an uncommon case of vulvar BCC. A 68-year-old woman with 5-year history of an asymptomatic lump on the right side of the vulva. The patient complained of progressive itching, pain, and color changes to brown for 3 months before visiting the clinic. Excisional biopsy revealed the lesion to be a BCC. After thorough negative metastasis work up the patient underwent a bilateral inguinofemoral lymphadenectomy and a wide local excision with a one-centimeter margin and final histology report confirmed the diagnosis of BCC. (HPV DNA and viral markers were negative, the bimanual exam was normal, both colposcopy and vaginoscopy were normal. There was no acetowhite lesion). To avoid delayed diagnosis, any persistent lesion even asymptomatic, in the vulvar region, especially when pigmented, irritating, or expanding in size, should be biopsied and investigated histologically, regardless of the patient's age. Any persistent lesion in the vulvar region, especially when pigmented, irritating, or expanding in size, should be biopsied and investigated histologically, regardless of the patient's age.

Primary Extrathoracic Extraskeletal Osteosarcoma of the Chest Wall: A Rare Entity

Extraskeletal osteosarcoma (ESOS) is a rapidly growing malignant neoplasm-producing osteoid, located in the soft tissues other than bones or periosteum. Primary ESOS presenting in extrathoracic locations is very rare. The optimal treatment of ESOS is largely unknown. The most common location is the soft tissues of the thigh (46%), upper extremity (20%), intrathoracic (rare), and retroperitoneum (17%). These tumors are usually large while diagnosis, typically measures 10 cm in diameter. The diagnosis is by core biopsy and microscopy sections reveal spindle cells with high NC ratio, myxoid stroma, osteoid matrix, and necrosis. Immunohistochemistry reveals that the tumor cells are positive for Desmin (40%), CD99 (25%), NKX2.2 (30%), and myogenin (15%). The radiological differentials include posttraumatic myositis ossificans. The treatment consists of wide surgical resection and reconstruction depends on the defect. Patients with localized disease were treated with an osteosarcoma-type adjuvant chemotherapy regimen consisting of doxorubicin, and cisplatin. The prognosis is poor when compared with intra-osseous osteosarcoma. More than 90% of patients develop local and distant metastasis and recurrence is between 2 months and 10 years. We hereby describe the case of giant ESOS of the chest wall, which was managed successfully at our center. A 58-year-old male presented with rapid progressive malignant ESOS in the right side chest 20 cm × 20 cm. He was managed by a multidisciplinary team of oncosurgery, plastic surgeon, and onco medicine by neoadjuvant chemotherapy and wide local excision with latissimus dorsi pedicle reconstruction. On a 3-year follow-up, he had no recurrence and doing well.

Use of NovoSorb Biodegradable Temporizing Matrix in the Management of Complex Scalp Defects

Summary: Dermal substitutes have emerged as a potential option in complex wound management. PolyNovo NovoSorb Biodegradable Temporizing Matrix has shown promise in managing partial and full-thickness wounds. We discuss here the successful use of this product in the management of a scalp defect complicated by exposed bone, following wide local excision of a desmoplastic melanoma of the scalp.

A Rare Case of Malignant Phyllodes Tumor in a Young Female

Phyllodes tumours are rare breast tumours that represent less than 1% of all breast tumours. They can be classified into three categories based on their histological features: benign, borderline, and malignant. Malignant phyllodes tumours are the most aggressive and can metastasize to distant organs. Here we report a case of a 26-year-old female with a malignant phyllodes tumour of the breast. The patient underwent wide local excision followed by adjuvant radiotherapy. We discuss the diagnostic and therapeutic challenges of managing this rare malignancy.

Chondrosarcoma of the anterior chest wall: surgical resection and reconstruction, our institutional experience

Primary chest wall tumours are not very common. Chondrosarcomas is most common tumour arising from the chest wall. It occurs more often during the third and fourth decade of life. Chondrosarcomas are resistant to conventional chemotherapy and radiotherapy. Wide margin surgical excision remains the best available treatment approach. For chondrosarcomas involving the chest wall, surgical excision may result in chest wall defects that may require reconstruction to obliterate dead space, restore chest wall rigidity, preserve respiratory mechanics, maintain pulmonary function, protect intrathoracic organs, provide soft tissue coverage and minimize deformity. In this article we present a series of 3 cases of chondrosarcoma of anterior chest wall managed at government Royapettah hospital, Kilpauk medical college, Chennai. A 71-year-old male patient, a case of 22×20 cm giant chondrosarcoma arising from anterior left chest wall involving 2nd to 8th ribs. We did wide local excision and reconstruction of chest wall with a synthetic bone cement (methyl methacrylate) construct, sandwiched between two layers of polypropylene mesh. A 38-year-old male patient, a case of 8×6 cm chondrosarcoma of left anterior chest wall involving 9th rib, we did wide excision of tumor along with 8th, 9th, 10th ribs and defect reconstructed with prolene mesh. A 37-year-old male patient, a case of 5×4 cm chondrosarcoma arising from left 4th rib. We did wide excision along with 4th rib and primary closure. Patients with chondrosarcomas generally have a good prognosis when optimally diagnosed and treated. Our case series is interesting due to the different sizes of chondrosarcomas at presentation, which are managed differently. Complete resection with wide surgical margin remains the best available treatment, but post resection chest wall reconstruction is posing a great surgical challenge.

Clinical and Histopathologic Analyses of Nasopharyngeal Hyalinizing Clear Cell Carcinoma: A Series of 26 Cases With Molecular Confirmation.

The aim of this study was to evaluate the clinicopathologic features, molecular characteristics, treatment strategy, and prognosis of nasopharyngeal hyalinizing clear cell carcinoma (HCCC). Retrospective observational case series. Institutional pathology records between 2006 and 2022 were searched for all cases of nasopharyngeal HCCC. We included 10 male and 16 female patients aged 30 to 82 years (median: 60.5y, mean: 54.6y). The most common symptoms were blood-stained rhinorrhea and nasal obstruction. Tumors most often involved the lateral wall of the nasopharynx, followed by the superior posterior wall. Microscopically, all tumor cells were arranged in sheets, nests, cords, and single cells in a hyaline/myxoid/fibrous stroma. The tumor cells were polygonal, with or without distinct cell borders, and displayed abundant clear-to-eosinophilic cytoplasm. All 26 cases were positive for pancytokeratin, CK7, p40, and p63 but negative for myoepithelial differentiation markers. Ki-67 labeling was low and ranged from 1% to 10%. All 26 cases demonstrated EWSR1 and EWSR1-ATF1 rearrangements, and no case demonstrated MAML2 rearrangement. Complete follow-up data were available for 23 patients: 14 patients underwent endoscopic surgery alone, 5 underwent radiation therapy followed by endoscopic surgery, 3 underwent radiation therapy followed by biopsy, and 1 underwent cisplatin chemotherapy before endoscopic surgery. Clinical follow-up ranged from 6 to 195 months; 13 patients (56.5%) were alive without tumor, 5 patients (21.7%) died of disease, 5 patients (21.7%) survived with tumor. HCCCs of the nasopharynx are rare tumors. The definitive diagnosis depends on histopathology, immunohistochemistry, and molecular studies. The optimal treatment for patients with nasopharyngeal HCCC is wide local excision. Radiation and chemotherapy might be good options for managing locally advanced cases. Nasopharyngeal HCCC is less indolent than previously thought. Tumor stage and the choice of treatment are key factors affecting the prognosis of nasopharyngeal HCCC patients.