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A Masquerading Giant: Unprecedented Sebaceous Gland Carcinoma with Massive Orbito-facial Invasion and the Diagnostic and Therapeutic Challenges of an Extreme Presentation Rizki Dwayana Putra; Mardijas Efendi; Ardizal Rahman
Bioscientia Medicina : Journal of Biomedicine and Translational Research Vol. 9 No. 11 (2025): Bioscientia Medicina: Journal of Biomedicine & Translational Research
Publisher : HM Publisher

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.37275/bsm.v9i11.1434

Abstract

Background: Sebaceous gland carcinoma (SGC) is a rare, aggressive adnexal malignancy of the eyelid, notorious for masquerading as benign inflammatory conditions. Presentations involving massive, destructive orbito-facial invasion are exceptionally rare and pose profound diagnostic and therapeutic challenges, often reflecting a confluence of biological aggression and systemic delays in care. Case presentation: A 68-year-old male presented with a one-year history of a progressively enlarging mass on his left upper eyelid, which had evolved into a giant, 15 x 15 x 7 cm fungating tumor, causing complete destruction of the orbital contents and extensive invasion into adjacent facial structures. An initial incisional biopsy was paradoxically interpreted as benign sebaceous hyperplasia. Due to the stark clinico-pathological discordance, a repeat, deeper biopsy was performed. The subsequent histopathological examination revealed a high-grade carcinoma, and the diagnosis was definitively confirmed by a panel of immunohistochemical stains, including strong positivity for Epithelial Membrane Antigen (EMA) and Cytokeratin 7 (CK7). The disease was staged as T4dN1M0 and deemed unresectable. Conclusion: This case documents a catastrophic outcome of SGC, resulting from a combination of delayed patient presentation and initial diagnostic error. It highlights that giant SGC, while rare, must be considered in the differential of destructive facial tumors and underscores that immunohistochemistry is mandatory for resolving clinico-pathological paradoxes in ocular adnexal pathology. This case serves as a call to action for improved public health awareness and enhanced diagnostic acumen to prevent such devastatingly advanced presentations.
The Molecular and Epidemiological Atlas of Primary Orbital Lymphoma: A Global Meta-Analysis of 3,832 Cases and Pathophysiological Correlates Silvia Roza; Ardizal Rahman; Mardijas Efendi
Bioscientia Medicina : Journal of Biomedicine and Translational Research Vol. 9 No. 11 (2025): Bioscientia Medicina: Journal of Biomedicine & Translational Research
Publisher : HM Publisher

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.37275/bsm.v9i11.1425

Abstract

Background: Primary orbital lymphoma is the most common orbital malignancy in adults, yet its global distribution and the prevalence of its histopathological subtypes remain poorly defined by large-scale evidence. This study provides a comprehensive quantitative synthesis of the global landscape of orbital lymphoma to inform diagnostic frameworks and guide future research. Methods: Following PRISMA guidelines, we conducted a systematic review and meta-analysis of studies published between January 2015 and December 2023. We searched PubMed, Scopus, Web of Science, and Embase for observational studies reporting histopathological data on orbital lymphoma. Two independent reviewers performed study selection, data extraction, and quality appraisal using the Joanna Briggs Institute (JBI) checklist. Pooled prevalence for each lymphoma subtype was calculated using a random-effects model. Heterogeneity was explored via subgroup analyses and meta-regression, and the robustness of findings was confirmed with a sensitivity analysis. Results: Fifteen studies comprising 3,832 patients met the inclusion criteria. Extranodal marginal zone lymphoma (EMZL) was the most prevalent subtype globally, with a pooled prevalence of 57.1% (95% CI: 51.5-62.7%). This was followed by diffuse large B-cell lymphoma (DLBCL) at 16.5% (95% CI: 13.1-20.0%), follicular lymphoma (FL) at 10.2% (95% CI: 8.0-12.4%), mantle cell lymphoma (MCL) at 5.1% (95% CI: 3.6-6.6%), and small lymphocytic lymphoma (SLL) at 3.4% (95% CI: 2.2-4.5%). Subgroup analysis revealed a significantly higher prevalence of EMZL in Asia (61.3%) compared to Europe (54.2%) and North America (55.8%) (p=0.04), while FL was more common in North American (14.1%) and European (12.8%) cohorts versus Asian cohorts (4.5%) (p<0.01). Sensitivity analysis confirmed the stability of these estimates. Conclusion: This meta-analysis establishes EMZL as the predominant histopathological subtype of orbital lymphoma worldwide, while highlighting profound geographical disparities in the distribution of EMZL and FL. These findings provide a robust global benchmark critical for clinical practice and underscore the influence of distinct geographical, genetic, and microenvironmental factors in orbital lymphomagenesis.
Malignant Transformation of Primary Acquired Melanosis into Conjunctival Melanoma in an Adolescent Male: A Clinico-Pathological Case Report and Management Review Fitrah; Ardizal Rahman; Mardijas Efendi
Bioscientia Medicina : Journal of Biomedicine and Translational Research Vol. 9 No. 11 (2025): Bioscientia Medicina: Journal of Biomedicine & Translational Research
Publisher : HM Publisher

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.37275/bsm.v9i11.1426

Abstract

Background: Conjunctival malignant melanoma (CMM) is a rare but potentially lethal ocular surface malignancy, especially uncommon in adolescents. It often arises from a precursor lesion known as primary acquired melanosis (PAM) with atypia. We present a case of CMM developing from long-standing PAM in an 18-year-old male, highlighting the diagnostic and therapeutic challenges in this unusual demographic. Case presentation: An 18-year-old male presented with a pigmented conjunctival lesion in his right eye, which had been present for over a decade but had recently shown progressive enlargement and darkening. Slit-lamp biomicroscopy revealed a 5x2 mm, variegated, hyperpigmented lesion on the bulbar conjunctiva with a prominent feeding vessel. The patient underwent an excisional biopsy using a "no-touch" technique with 4 mm margins and adjunctive double freeze-thaw cryotherapy. Histopathological analysis confirmed an invasive malignant melanoma, Breslow thickness of 1.8 mm, arising from PAM with severe atypia. Surgical margins were clear of the tumor. Conclusion: This case underscores that malignant transformation of conjunctival melanocytic lesions can occur even in young patients. The presence of a changing pigmented lesion, regardless of patient age, necessitates a high index of suspicion and a low threshold for excisional biopsy. Meticulous surgical technique combined with adjuvant therapy and vigilant long-term surveillance is paramount for optimizing patient outcomes.
A Masquerading Giant: Unprecedented Sebaceous Gland Carcinoma with Massive Orbito-facial Invasion and the Diagnostic and Therapeutic Challenges of an Extreme Presentation Rizki Dwayana Putra; Mardijas Efendi; Ardizal Rahman
Bioscientia Medicina : Journal of Biomedicine and Translational Research Vol. 9 No. 11 (2025): Bioscientia Medicina: Journal of Biomedicine & Translational Research
Publisher : HM Publisher

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.37275/bsm.v9i11.1434

Abstract

Background: Sebaceous gland carcinoma (SGC) is a rare, aggressive adnexal malignancy of the eyelid, notorious for masquerading as benign inflammatory conditions. Presentations involving massive, destructive orbito-facial invasion are exceptionally rare and pose profound diagnostic and therapeutic challenges, often reflecting a confluence of biological aggression and systemic delays in care. Case presentation: A 68-year-old male presented with a one-year history of a progressively enlarging mass on his left upper eyelid, which had evolved into a giant, 15 x 15 x 7 cm fungating tumor, causing complete destruction of the orbital contents and extensive invasion into adjacent facial structures. An initial incisional biopsy was paradoxically interpreted as benign sebaceous hyperplasia. Due to the stark clinico-pathological discordance, a repeat, deeper biopsy was performed. The subsequent histopathological examination revealed a high-grade carcinoma, and the diagnosis was definitively confirmed by a panel of immunohistochemical stains, including strong positivity for Epithelial Membrane Antigen (EMA) and Cytokeratin 7 (CK7). The disease was staged as T4dN1M0 and deemed unresectable. Conclusion: This case documents a catastrophic outcome of SGC, resulting from a combination of delayed patient presentation and initial diagnostic error. It highlights that giant SGC, while rare, must be considered in the differential of destructive facial tumors and underscores that immunohistochemistry is mandatory for resolving clinico-pathological paradoxes in ocular adnexal pathology. This case serves as a call to action for improved public health awareness and enhanced diagnostic acumen to prevent such devastatingly advanced presentations.
A Diagnostic Rarity: Apocrine Hidrocystoma Presenting as a Medial Canthal Mass in Adolescence Atika Syafendra; Mardijas Efendi; Ardizal Rahman
Bioscientia Medicina : Journal of Biomedicine and Translational Research Vol. 9 No. 11 (2025): Bioscientia Medicina: Journal of Biomedicine & Translational Research
Publisher : HM Publisher

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.37275/bsm.v9i11.1438

Abstract

Background: Apocrine hidrocystomas are benign adnexal tumors of the glands of Moll, typically diagnosed in adults. Their presentation in adolescence is an exceptional clinical finding that challenges standard diagnostic paradigms for periocular masses in this age group, necessitating a broad and meticulous differential diagnosis. Case presentation: A 15-year-old female presented with a two-month history of a stable, asymptomatic, 7×5×1 mm cystic mass at the inferomedial canthus of the left eye. The patient's primary concern was cosmetic. A comprehensive ophthalmologic examination was unremarkable. Orbital computed tomography confirmed a simple, preseptal subcutaneous cyst. A complete excisional biopsy was performed, and histopathological analysis revealed a unilocular cyst lined by a double layer of epithelium with inner columnar cells demonstrating pathognomonic decapitation secretion, confirming an apocrine hidrocystoma. Conclusion: This case highlights the necessity of including apocrine hidrocystoma in the differential diagnosis of periocular masses in adolescents. The primary lesson is that a patient's age should broaden, not narrow, the diagnostic possibilities. Definitive diagnosis relies on histopathology, not demographic probability, and complete surgical excision remains the gold standard for both diagnosis and curative therapy, yielding an excellent prognosis.
Clinicopathological Spectrum and Management of Lacrimal Gland Tumors: A Five-Year Retrospective Analysis from an Indonesian Tertiary Center Agustin Virajati Negoro; Mardijas Efendi; Ardizal Rahman
Bioscientia Medicina : Journal of Biomedicine and Translational Research Vol. 9 No. 12 (2025): Bioscientia Medicina: Journal of Biomedicine & Translational Research
Publisher : HM Publisher

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.37275/bsm.v9i12.1456

Abstract

Background: Lacrimal gland tumors are a rare and heterogeneous group of neoplasms, representing a significant diagnostic and therapeutic challenge. Regional data, particularly from Southeast Asia, is sparse. This study aims to define the clinicopathological spectrum, radiological features, and management strategies for lacrimal gland tumors at a major tertiary referral center in Indonesia. Methods: A retrospective, cross-sectional analysis was conducted on all patients with histopathologically confirmed lacrimal gland tumors treated between January 2019 and June 2024. Data extracted from medical records included demographics, detailed clinical presentations (visual acuity, proptosis, pain scores), radiological findings from computed tomography (CT) and magnetic resonance imaging (MRI), definitive histopathological diagnoses with immunohistochemical profiles, and treatment modalities with short-term outcomes. Descriptive statistics and comparative analyses were performed. Results: A total of 35 patients (19 male, 16 female; mean age 48.2 ± 16.5 years) were included. Non-epithelial lesions (88.6%) were more common than epithelial tumors (11.4%). The most prevalent diagnosis was idiopathic orbital inflammation (IOI) (n=12, 34.3%), followed by lymphoproliferative disorders (n=11, 31.4%). Adenoid cystic carcinoma (ACC) was the most frequent malignant epithelial tumor (n=3, 8.6%). Superior eyelid edema was the hallmark of non-epithelial lesions (83.9%), whereas proptosis (mean 6.2mm) and severe pain (mean VAS 7.3/10) were characteristic of ACC. Radiological findings were highly correlative, with IOI showing diffuse gland enhancement and ACC demonstrating bone erosion and perineural invasion. Management was tailored to histology, with corticosteroids for IOI, radiotherapy for lymphomas, and radical surgery for ACC. Conclusion: In this Indonesian cohort, lacrimal gland tumors are predominantly non-epithelial, with inflammatory and lymphoproliferative conditions being most common. A high index of suspicion for malignancy, particularly ACC, is warranted in patients presenting with pain and proptosis. Integrating clinical, radiological, and pathological data is paramount for accurate diagnosis and effective management.
A Diagnostic Rarity: Apocrine Hidrocystoma Presenting as a Medial Canthal Mass in Adolescence Atika Syafendra; Mardijas Efendi; Ardizal Rahman
Bioscientia Medicina : Journal of Biomedicine and Translational Research Vol. 9 No. 11 (2025): Bioscientia Medicina: Journal of Biomedicine & Translational Research
Publisher : HM Publisher

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.37275/bsm.v9i11.1438

Abstract

Background: Apocrine hidrocystomas are benign adnexal tumors of the glands of Moll, typically diagnosed in adults. Their presentation in adolescence is an exceptional clinical finding that challenges standard diagnostic paradigms for periocular masses in this age group, necessitating a broad and meticulous differential diagnosis. Case presentation: A 15-year-old female presented with a two-month history of a stable, asymptomatic, 7×5×1 mm cystic mass at the inferomedial canthus of the left eye. The patient's primary concern was cosmetic. A comprehensive ophthalmologic examination was unremarkable. Orbital computed tomography confirmed a simple, preseptal subcutaneous cyst. A complete excisional biopsy was performed, and histopathological analysis revealed a unilocular cyst lined by a double layer of epithelium with inner columnar cells demonstrating pathognomonic decapitation secretion, confirming an apocrine hidrocystoma. Conclusion: This case highlights the necessity of including apocrine hidrocystoma in the differential diagnosis of periocular masses in adolescents. The primary lesson is that a patient's age should broaden, not narrow, the diagnostic possibilities. Definitive diagnosis relies on histopathology, not demographic probability, and complete surgical excision remains the gold standard for both diagnosis and curative therapy, yielding an excellent prognosis.
Clinicopathological Spectrum and Management of Lacrimal Gland Tumors: A Five-Year Retrospective Analysis from an Indonesian Tertiary Center Agustin Virajati Negoro; Mardijas Efendi; Ardizal Rahman
Bioscientia Medicina : Journal of Biomedicine and Translational Research Vol. 9 No. 12 (2025): Bioscientia Medicina: Journal of Biomedicine & Translational Research
Publisher : HM Publisher

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.37275/bsm.v9i12.1456

Abstract

Background: Lacrimal gland tumors are a rare and heterogeneous group of neoplasms, representing a significant diagnostic and therapeutic challenge. Regional data, particularly from Southeast Asia, is sparse. This study aims to define the clinicopathological spectrum, radiological features, and management strategies for lacrimal gland tumors at a major tertiary referral center in Indonesia. Methods: A retrospective, cross-sectional analysis was conducted on all patients with histopathologically confirmed lacrimal gland tumors treated between January 2019 and June 2024. Data extracted from medical records included demographics, detailed clinical presentations (visual acuity, proptosis, pain scores), radiological findings from computed tomography (CT) and magnetic resonance imaging (MRI), definitive histopathological diagnoses with immunohistochemical profiles, and treatment modalities with short-term outcomes. Descriptive statistics and comparative analyses were performed. Results: A total of 35 patients (19 male, 16 female; mean age 48.2 ± 16.5 years) were included. Non-epithelial lesions (88.6%) were more common than epithelial tumors (11.4%). The most prevalent diagnosis was idiopathic orbital inflammation (IOI) (n=12, 34.3%), followed by lymphoproliferative disorders (n=11, 31.4%). Adenoid cystic carcinoma (ACC) was the most frequent malignant epithelial tumor (n=3, 8.6%). Superior eyelid edema was the hallmark of non-epithelial lesions (83.9%), whereas proptosis (mean 6.2mm) and severe pain (mean VAS 7.3/10) were characteristic of ACC. Radiological findings were highly correlative, with IOI showing diffuse gland enhancement and ACC demonstrating bone erosion and perineural invasion. Management was tailored to histology, with corticosteroids for IOI, radiotherapy for lymphomas, and radical surgery for ACC. Conclusion: In this Indonesian cohort, lacrimal gland tumors are predominantly non-epithelial, with inflammatory and lymphoproliferative conditions being most common. A high index of suspicion for malignancy, particularly ACC, is warranted in patients presenting with pain and proptosis. Integrating clinical, radiological, and pathological data is paramount for accurate diagnosis and effective management.
The Clinical Spectrum and Management Patterns of Orbital Meningioma: A 6-Year Single-Institution Case Series from Indonesia Anggia Dwi Kora; Mardijas Effendi; Ardizal Rahman
Bioscientia Medicina : Journal of Biomedicine and Translational Research Vol. 10 No. 1 (2025): Bioscientia Medicina: Journal of Biomedicine & Translational Research
Publisher : HM Publisher

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.37275/bsm.v10i1.1482

Abstract

Background: Orbital meningiomas (OMs) are rare, complex tumors whose clinical course and management are debated. Data from Southeast Asian populations, particularly Indonesia, is scarce. This study aims to define the clinical spectrum, radiological features, and management trends of OMs at a single Indonesian tertiary referral center, with a focus on hormonal factors in a predominantly female cohort. Methods: A retrospective, descriptive case series was conducted on all patients diagnosed with orbital meningioma at Dr. M. Djamil General Hospital, Padang, Indonesia, from January 2018 to December 2023. Data on demographics, detailed clinical presentation (visual acuity, proptosis, extraocular movement), radiological findings (location, size, hyperostosis), and hormonal risk factors were collected. Management strategies, histopathological results (WHO grade), and follow-up outcomes were analyzed. Results: Twenty patients were included, with a significant female predominance (n=16, 80%; F:M ratio 4:1). The median age at diagnosis was 47 years (range 28-71). A strong association with hormonal factors was noted; 50% (8/16) of female patients had a history of exogenous hormonal contraceptive use. The most common presenting symptoms were progressive proptosis (n=15, 75%) and visual loss (n=12, 60%). The mean proptosis was 5.5 ± 2.1 mm. The most frequent tumor location was retrobulbar (intraconal) (n=11, 55%), followed by optic nerve sheath (n=5, 25%). A multidisciplinary, conservative-led approach was the primary management strategy, with 75% (n=15) of patients managed with active observation and neurosurgical consultation. Surgical intervention was performed in 25% (n=5) of cases, primarily for severe symptoms or aggressive radiological features. Histopathology (n=5) confirmed WHO Grade I in four cases (80%) and WHO Grade II (atypical) in one case (20%). Conclusion: Orbital meningiomas in this Indonesian cohort demonstrate a striking female predominance and a strong association with hormonal contraceptive use, suggesting a significant role for hormonal pathways in their pathophysiology. The management paradigm has shifted towards a multidisciplinary, observation-first approach, reserving surgical intervention for cases with documented progression, severe vision loss, or high-grade pathological features.
The Profile of Sinonasal Tumors with Orbital Involvement in Dr. M. Djamil General Hospital, Padang, Indonesia: A 10-Year Overview Pohan, Dwi Lestari; Ardizal Rahman; Mardijas Efendi
Bioscientia Medicina : Journal of Biomedicine and Translational Research Vol. 8 No. 6 (2024): Bioscientia Medicina: Journal of Biomedicine & Translational Research
Publisher : HM Publisher

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.37275/bsm.v8i6.1014

Abstract

Background: Sinonasal tumors are rare tumors, either malignant or benign, that vary greatly based on the origin and primary tumor location in the sinonasal tract. The close anatomical relationship between the sinonasal tract and the orbital cavity makes orbital involvement very common (50%-80%) in sinonasal tumors, and this indicates a negative prognostic factor in sinonasal malignancy. The purpose of this study is to demonstrate the profile of sinonasal tumors with orbital involvement in our center. Methods: Retrospective study from oncology subdivision files and medical records from January 2011 – December 2020. Collected data including age, gender, the clinical manifestations of orbital involvement, primary location, histopathology, and management of sinonasal tumors. Results: There were 35 patients examined by the oncology subdivision either directly came or consulted from the ENT department, consisting of 18 (51.4%) males and 17 (48.6%) females. The age of patients ranged from 11 to 83 years old. with the most common age groups being 41-50 and 51-60 (25.7% in each age group). The majority of the patients (77.1%) had presented with proptosis. Maxillary sinus was the most common (45.7%) primary site of tumors, and squamous cell carcinoma was the most common (51.4%) histopathology found. Most of the patients (37.1%) were treated with a combination of chemotherapy and radiotherapy. Conclusion: Orbital involvement commonly occurs in malignant sinonasal tumors with proptosis as a major clinical presentation. A combination of chemotherapy and radiotherapy was the most common practice management because the tumors were unresectable at the time of presentation.