ABSTRACT
Purpose: Local recurrence predicts dismal prognosis in eyelid sebaceous carcinoma (SC). Recurrence predictors vary across studies. Accurate recurrence estimation is essential for individualized therapy in eyelid SC. This study aims to identify recurrence predictors and develop a nomogram for personalized prediction in eyelid SC. Methods: We conducted a multicenter retrospective cohort study. Chart reviews were performed in 418 consecutive patients with eyelid SC. All patients were followed up after their initial surgery. Multivariate Cox regression was used to explore the independent predictors of recurrence. A nomogram for recurrence prediction was developed and validated with bootstrap resampling. The predictive accuracy and discriminative ability were compared with the Tumor, Node, Metastasis (TNM) staging system. Results: Over a median of 60-month follow-up, 167 patients (40%) had local recurrence. The median time from diagnosis to recurrence was 14 months. The 1-year cumulative recurrence rate was 18%. Diagnostic delay (hazard ratio [HR] = 1.01, 95% confidence interval [CI] = 1.00-1.01, P = 0.001), orbital involvement (HR = 4.47, 95% CI = 3.04-6.58, P < 0.001), Ki67 (HR = 1.01, 95% CI = 1.00-1.02, P = 0.008) and initial surgery of Mohs micrographic surgery with intraoperative frozen section control (HR = 0.53, 95% CI = 0.35-0.80, P = 0.003) were independent influencing factors of recurrence. A nomogram integrating these four factors combined with pagetoid spread displayed satisfactory discriminative ability (C-index = 0.80-0.83; area under the curve [AUC] = 0.82-0.84), which compared favorably than TNM staging (all P < 0.05). Conclusions: The recurrence rate is high in eyelid SC. Early detection and primary resection with Mohs micrographic surgery are recommended in controlling recurrence. Patients with orbital involvement, high Ki67 expression, and pagetoid spread may require adjuvant measures. This nomogram offers more accurate recurrence estimates, aiding in therapeutic decision making.
Subject(s)
Adenocarcinoma, Sebaceous , Eyelid Neoplasms , Neoplasm Recurrence, Local , Nomograms , Sebaceous Gland Neoplasms , Humans , Male , Female , Retrospective Studies , Eyelid Neoplasms/pathology , Eyelid Neoplasms/diagnosis , Sebaceous Gland Neoplasms/pathology , Sebaceous Gland Neoplasms/diagnosis , Middle Aged , Aged , Adenocarcinoma, Sebaceous/pathology , Adenocarcinoma, Sebaceous/diagnosis , Follow-Up Studies , Neoplasm Staging , Aged, 80 and over , Adult , PrognosisABSTRACT
PURPOSE: To report the outcomes of platinum-based neoadjuvant chemotherapy (NACT) for eyelid and periocular sebaceous gland carcinoma (eSGC). METHODS: Retrospective study of 25 patients. RESULTS: The mean age at presentation of eSGC was 59 years. The mean tumor basal diameter was 46 mm. By the 8th edition of AJCC classification, tumors belonged T2 (n = 2, 8%), T3 (n = 6, 24%), and T4 (n = 17, 68%); N1 (n = 12,48%); and M1 (n = 1, 4%). NACT with 5-fluorouracil (5-FU) and cisplatin/carboplatin was administered in 21 (84%)/4 (16%) patients, respectively. The mean number of cycles of neoadjuvant systemic chemotherapy per patient was 2 (median, 3). The mean percentage reduction of tumor basal volume after neoadjuvant chemotherapy was 65% (median, 60%). After NACT, 12 (48%) patients underwent surgical treatment, 6 (12%) patients underwent EBRT, and 4 (8%) underwent adjuvant chemotherapy. A total of 11 (44%) patients were lost to follow-up during the course of treatment, of whom 3 died from metastatic disease. In 16 patients followed up for ≥ 3 months, complete tumor control was achieved in 11 (69%) patients, local tumor control in 14 (88%), and globe salvage in 7 (44%) at a mean follow-up of 25 months (median, 7 months; range, 3 to 110 months). No tumor recurrence was seen in any case. One (4%) serious adverse event of cardiotoxicity was noted. CONCLUSION: Platinum-based NACT is a suitable option for eSGC with advanced tumors and locoregional metastasis. Adverse events are rare and in patients compliant with treatment, NACT-based combination therapy offers globe salvage and systemic tumor control.
Subject(s)
Eyelid Neoplasms , Neoadjuvant Therapy , Sebaceous Gland Neoplasms , Humans , Middle Aged , Male , Female , Retrospective Studies , Eyelid Neoplasms/drug therapy , Eyelid Neoplasms/diagnosis , Eyelid Neoplasms/pathology , Sebaceous Gland Neoplasms/drug therapy , Sebaceous Gland Neoplasms/surgery , Sebaceous Gland Neoplasms/diagnosis , Sebaceous Gland Neoplasms/pathology , Neoadjuvant Therapy/methods , Aged , Adult , Adenocarcinoma, Sebaceous/drug therapy , Adenocarcinoma, Sebaceous/diagnosis , Adenocarcinoma, Sebaceous/pathology , Adenocarcinoma, Sebaceous/surgery , Follow-Up Studies , Cisplatin/administration & dosage , Cisplatin/therapeutic use , Aged, 80 and over , Fluorouracil/administration & dosage , Fluorouracil/therapeutic use , Chemotherapy, Adjuvant/methods , Carboplatin/administration & dosage , Carboplatin/therapeutic use , Neoplasm Staging , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Treatment OutcomeSubject(s)
Antibodies, Monoclonal, Humanized , Antineoplastic Agents, Immunological , Sebaceous Gland Neoplasms , Humans , Antibodies, Monoclonal, Humanized/therapeutic use , Sebaceous Gland Neoplasms/drug therapy , Sebaceous Gland Neoplasms/pathology , Antineoplastic Agents, Immunological/therapeutic use , Male , Adenocarcinoma, Sebaceous/drug therapy , Adenocarcinoma, Sebaceous/secondary , Adenocarcinoma, Sebaceous/pathology , Aged , FemaleABSTRACT
INTRODUCTION: Sebaceous gland carcinoma (SGC) of the eyelid is an aggressive tumour with the ability to metastasise and an increased morbidity. Controversies regarding the epidemiology of this malignant eyelid tumour is widespread in the scientific literature. Western reports repeatedly describes eyelid SGC as a rare occurring tumour in general, accounting for 1%-3% of all eyelid tumours, however studies from Asia have uncovered a higher frequency of eyelid SGC including 54% of all eyelid tumours in Japan, and 43%-56% in India. We wish to retrieve observational data of eyelid SGC prevalence in proportion to total eyelid tumours, from pathological studies published worldwide to resolve this controversy. METHODS AND ANALYSIS: We will search Ovid Medline, EMBASE, Cochrane Central Register of Controlled Trials, Scopus and Google Scholar to identify published reports on eyelid SGC prevalence proportions, aiming to clarify the incidence of the tumour. We will include observational clinicopathological studies reporting prevalence with confirmed histopathology. No limitations on publication date or language will be applied. Data from the individual studies and study quality will be extracted by two individual reviewers. Study quality will be assessed using the JBI Critical Appraisal Instrument for Studies Reporting Prevalence Data. Raw proportions will be transformed and pooled using a random effects model for meta-analysis. And subgroup analysis according to geography will be performed. If data are deemed unsuitable for a meta-analysis, a narrative synthesis will be presented. We will judge the certainty of evidence and present whether this has an overall effect on the results. The results may shed light on a long-standing academic disparity of the scientific literature. ETHICS AND DISSEMINATION: This systematic review does not require ethical approval. The results of this proposed review will be the subject to a publication in an international peer-reviewed journal within the ophthalmic or pathological specialty. PROSPERO REGISTRATION NUMBER: CRD42023487141.
Subject(s)
Eyelid Neoplasms , Meta-Analysis as Topic , Research Design , Sebaceous Gland Neoplasms , Systematic Reviews as Topic , Humans , Eyelid Neoplasms/epidemiology , Eyelid Neoplasms/pathology , Prevalence , Sebaceous Gland Neoplasms/pathology , Sebaceous Gland Neoplasms/epidemiology , Adenocarcinoma, Sebaceous/epidemiology , Adenocarcinoma, Sebaceous/pathologyABSTRACT
Ovarian mature teratomas (OMTs) originate from post-meiotic germ cells. Malignant transformation occurs in approximately 1-2% of OMTs; however, sebaceous carcinoma arising from OMTs is rare. This is the first report of a detailed genomic analysis of sebaceous carcinoma arising from an OMT. A 36-year-old woman underwent evaluation for abdominal tumors and subsequent hysterectomy and salpingo-oophorectomy. Pathologically, a diagnosis of stage IA sebaceous carcinoma arising from an OMT was established. Eight months post-surgery, the patient was alive without recurrence. Immunohistochemically, the tumor was negative for mismatch repair proteins. A nonsense mutation in TP53 (p.R306*) and a deletion in PIK3R1 were identified. Single nucleotide polymorphisms across all chromosomes displayed a high degree of homozygosity, suggestive of uniparental disomy. Herein, the OMT resulting from the endoreduplication of oocytes underwent a malignant transformation to sebaceous carcinoma via TP53 as an early event and PIK3R1 as a late event.
Subject(s)
Ovarian Neoplasms , Teratoma , Tumor Suppressor Protein p53 , Humans , Female , Adult , Ovarian Neoplasms/genetics , Ovarian Neoplasms/pathology , Ovarian Neoplasms/surgery , Teratoma/genetics , Teratoma/pathology , Tumor Suppressor Protein p53/genetics , Class Ia Phosphatidylinositol 3-Kinase/genetics , Adenocarcinoma, Sebaceous/genetics , Adenocarcinoma, Sebaceous/pathology , Polymorphism, Single Nucleotide , Cell Transformation, Neoplastic/geneticsSubject(s)
Marital Status , Neoplasm Staging , SEER Program , Sebaceous Gland Neoplasms , Humans , SEER Program/statistics & numerical data , Male , Female , Sebaceous Gland Neoplasms/mortality , Sebaceous Gland Neoplasms/pathology , Sebaceous Gland Neoplasms/epidemiology , Middle Aged , Marital Status/statistics & numerical data , Aged , United States/epidemiology , Adult , Adenocarcinoma, Sebaceous/pathology , Adenocarcinoma, Sebaceous/mortality , Adenocarcinoma, Sebaceous/epidemiology , Survival Rate , Aged, 80 and overABSTRACT
Sebaceous gland carcinomas are rare malignant cutaneous adnexal tumors with sebocytic differentiation. The typical predilection area is the head and neck region, where sebaceous gland carcinomas are the most common malignant adnexal tumors of the skin. According to their localization a distinction is made between periocular and extraocular sebaceous gland carcinomas. Muir-Torre syndrome (MTS) should always be ruled out if it is suspected. In terms of prognosis, sebaceous gland carcinomas are potentially aggressive tumors with a clear tendency to recur and metastasize. Only small extraocular sebaceous gland carcinomas that have been completely resected have a very good prognosis. Sebaceous gland carcinomas most frequently metastasize lymphogenously to regional or distant lymph nodes; organ metastasis occurs less frequently. Periocular sebaceous gland carcinomas have a higher metastasis rate (up to 15%) than extraocular sebaceous gland carcinomas (up to 2%). Complete micrographically controlled surgery (MCS) of the primary tumor is the therapy of first choice, regardless of periocular or extraocular localization. Adjuvant or therapeutic radiotherapy may be considered. There is currently no established standard therapy for advanced, inoperable, or metastatic sebaceous gland carcinomas. Local procedures and systemic therapies such as chemotherapy or immunotherapy can be considered. The procedure should be determined individually by an interdisciplinary tumor board. Close follow-up care is recommended for these potentially aggressive carcinomas.
Subject(s)
Sebaceous Gland Neoplasms , Sebaceous Gland Neoplasms/pathology , Sebaceous Gland Neoplasms/therapy , Sebaceous Gland Neoplasms/diagnosis , Humans , Muir-Torre Syndrome/pathology , Muir-Torre Syndrome/diagnosis , Muir-Torre Syndrome/therapy , Prognosis , Adenocarcinoma, Sebaceous/pathology , Adenocarcinoma, Sebaceous/therapy , Adenocarcinoma, Sebaceous/diagnosis , Dermatology/standards , Germany , Mohs Surgery , Practice Guidelines as TopicABSTRACT
BACKGROUND: Sebaceous carcinoma is a very rare malignant skin adnexal tumor that is occasionally aggressive. We have not seen a case of sebaceous carcinoma in our center in the last 10 years. It is extremely rare in Black Africans. CASE PRESENTATION: We described the case of a 55-year-old man African man who presented to our ophthalmologist with complaints of growth on the right upper eyelid for 8 months. He had surgery and chemotherapy for rectal carcinoma 6 years prior to presentation and received his last dose of chemotherapy 5 years before seeing our ophthalmologist. There was a history of spontaneous unprovoked bleeding from the lesion. He subsequently underwent surgical excision under general anesthesia. Histology of the mass showed an effaced architecture due to proliferating malignant epithelial cells disposed as trabecules, solid nests, and tongues. The microscopic features of widespread multivacuolated cytoplasm of the neoplastic cells led us to conclude that the tumor was a sebaceous carcinoma. The patient is alive and well. CONCLUSION: Sebaceous carcinoma is a rare malignant skin adnexal tumor in Black Africans. It can present as an eyelid mass with spontaneous bleeding. It can follow cancer chemotherapy either because of its association with other tumors in Muir-Torre syndrome or because of mutagenic effects of chemotherapeutic agents.
Subject(s)
Adenocarcinoma, Sebaceous , Blepharoptosis , Muir-Torre Syndrome , Sebaceous Gland Neoplasms , Male , Humans , Middle Aged , Adenocarcinoma, Sebaceous/pathology , Adenocarcinoma, Sebaceous/surgery , Sebaceous Gland Neoplasms/pathology , Sebaceous Gland Neoplasms/surgeryABSTRACT
ABSTRACT: Cutaneous sebaceous neoplasia comprises a spectrum of disease ranging from benign adenomas to malignant carcinomas. The hallmark of these lesions is sebaceous differentiation. However, poorly-differentiated sebaceous carcinoma (SC), which lacks significant overt sebaceous differentiation, can show morphologic overlap with a variety of other basaloid cutaneous neoplasms. The accurate classification of SC is essential not only for diagnosis, but also because of the potential association with Muir-Torre syndrome. Androgen receptor (AR) is a sensitive, but not entirely specific immunohistochemical marker that has been used for the diagnosis of SC. PReferentially expressed Antigen in MElanoma (PRAME) demonstrates strong cytoplasmic labeling of mature sebocytes and has been reported to be expressed in a variety of sebaceous neoplasms, including in the basaloid cell component. Therefore, we sought to compare the diagnostic use of cytoplasmic PRAME expression with that of AR for the distinction of SC from a cohort of basaloid cutaneous mimics; namely basal cell carcinoma, basaloid squamous cell carcinoma, pilomatricoma, cutaneous lymphadenoma, and extra-mammary Paget disease. We report that cytoplasmic PRAME expression is uncommon in poorly differentiated SC, and although specific, it shows very low sensitivity (22%). In contrast, AR was moderately sensitive (66%) and highly specific (92%) for the distinction of SC from basaloid mimics. These attributes, in addition to the nuclear expression of AR in the sebocytic and basaloid components of SC, suggest that AR is superior to PRAME for the diagnosis of SC.
Subject(s)
Adenocarcinoma, Sebaceous , Carcinoma, Basal Cell , Hair Diseases , Muir-Torre Syndrome , Sebaceous Gland Neoplasms , Humans , Immunohistochemistry , Receptors, Androgen , Adenocarcinoma, Sebaceous/diagnosis , Adenocarcinoma, Sebaceous/pathology , Sebaceous Gland Neoplasms/pathology , Carcinoma, Basal Cell/pathology , Antigens, NeoplasmABSTRACT
Sebaceous carcinoma of the breast is an extremely rare histological subtype of breast cancer, with fewer than 30 cases reported to date. Because of its extremely rare histological presentation, there are few case reports that highlight its cytological findings. In this case report, the cytomorphological features of a sebaceous carcinoma of the breast are described in detail. Cytomorphological analysis revealed atypical cells presenting predominantly as loose clusters. No tubular or papillary structures were evident in the clusters and no mucin production was observed. The diagnosis of sebaceous carcinoma of the breast requires prominent sebaceous differentiation of cells. In Papanicolaou-stained smears, the differentiated tumor cells were found within the yellowish clusters. When these yellowish clusters were observed at high magnification and shifted out of focus, the sebaceous differentiation of tumor cells could be recognized. This finding is an advantage of observing Papanicolaou-stained specimens. Like previous reports, some individual cells showing sebaceous differentiation were also observed. In cases where many yellowish clusters appear, close observation of the interior of the clusters can confirm the presence of sebaceous differentiation of tumor cells and serve as a diagnostic clue for the cytological diagnosis of sebaceous carcinoma of the breast.
Subject(s)
Adenocarcinoma, Sebaceous , Breast Neoplasms , Female , Humans , Adenocarcinoma, Sebaceous/pathology , Adenocarcinoma, Sebaceous/diagnosis , Breast Neoplasms/pathology , Breast Neoplasms/diagnosis , Sebaceous Gland Neoplasms/pathology , Sebaceous Gland Neoplasms/diagnosisABSTRACT
Here we present the first case of sebaceous carcinoma of the middle ear. We discuss the treatment course and post treatment results after 11 years of follow up. We further summarize the available literature of sebaceous carcinoma of the temporal bone, which prior to this case was exclusively limited to the external auditory canal. Laryngoscope, 134:3769-3772, 2024.
Subject(s)
Adenocarcinoma, Sebaceous , Ear Neoplasms , Ear, Middle , Humans , Ear Neoplasms/pathology , Ear Neoplasms/surgery , Ear Neoplasms/diagnosis , Ear, Middle/pathology , Ear, Middle/diagnostic imaging , Adenocarcinoma, Sebaceous/pathology , Adenocarcinoma, Sebaceous/diagnosis , Adenocarcinoma, Sebaceous/surgery , Sebaceous Gland Neoplasms/pathology , Sebaceous Gland Neoplasms/diagnosis , Sebaceous Gland Neoplasms/surgery , Male , Aged , Female , Middle AgedABSTRACT
Periocular sebaceous carcinoma (PSC) is a rare, aggressive, and potentially metastatic adnexal malignancy. Due to the ability of PSC to resemble several benign and malignant conditions, diagnosis is often delayed or mistaken. In addition, even with a known diagnosis, choosing the right treatment is still an open debate. For this reason, we decided to review the most up-to-date literature on PSC and propose a dedicated procedural protocol to help clinicians when dealing with PSC. A PubMed search was carried out using the terms "Sebaceous Carcinoma", "Adnexal Periocular Cancer", "Sebaceous Carcinoma AND eyelid", "Periocular Sebaceous Carcinoma", and "Ocular Adnexa". Pertinent studies published in English from 1997 up to December 2022 were compared to the selection criteria and if suitable, included in this review. Through the initial search, 84 articles were selected. Of these, 36 were included in the final study. Several papers explored different diagnostic and therapeutic strategies regarding PSC diagnosis and management. In light of the current literature review and the multidisciplinary experience of three clinical centers, a dedicated procedural protocol is proposed. PSC diagnosis may be achieved through accurate clinical evaluation, but it requires histopathologic confirmation, which can be challenging. Dermoscopy, in vivo reflectance confocal microscopy, and optical coherence tomography may facilitate PSC clinical examination, while immunohistochemistry stains may support histological diagnosis. Appropriate disease staging is necessary before choosing the treatment, as local disease requires radically different treatment compared to advanced disease. In addition, recent innovations in nonsurgical treatments, including radio-chemotherapy, immunotherapy, and targeted therapy, may be a viable option in the most challenging cases.
Subject(s)
Adenocarcinoma, Sebaceous , Eyelid Neoplasms , Neoplasm Staging , Sebaceous Gland Neoplasms , Humans , Sebaceous Gland Neoplasms/therapy , Sebaceous Gland Neoplasms/diagnosis , Sebaceous Gland Neoplasms/pathology , Eyelid Neoplasms/therapy , Eyelid Neoplasms/diagnosis , Eyelid Neoplasms/pathology , Adenocarcinoma, Sebaceous/therapy , Adenocarcinoma, Sebaceous/diagnosis , Adenocarcinoma, Sebaceous/pathology , Dermoscopy , Diagnosis, DifferentialABSTRACT
Sebaceous gland carcinoma is a rare and aggressive skin cancer derived from the sebaceous glands. Sebaceous carcinomas are divided into those occurring in ocular (75%) and extra-ocular locations. A 45-year-old female patient presented with rapidly growing swelling over the upper back region. It was provisionally diagnosed as an infected sebaceous cyst, and an excision biopsy was received in the pathology department. Histopathology was reported as sebaceous carcinoma, Grade II, Stage P T3 Nx. Immunohistochemistry was positive for epithelial membrane antigen. Sebaceous carcinoma accounts for 0.2-4.6% of all malignant cutaneous neoplasms, and the estimated rate of occurrence is only 1-2 per 1 million individuals per year. These tumors frequently present with a painless sub-cutaneous nodule, but they can also present as pedunculated lesions, irregular mass, or diffuse thickening of the skin. Hence, they are misinterpreted as other benign tumors or inflammatory conditions, thereby leading to delay in diagnosis, inappropriate treatment, increased morbidity, and mortality.
Subject(s)
Adenocarcinoma, Sebaceous , Sebaceous Gland Neoplasms , Skin Neoplasms , Female , Humans , Middle Aged , Adenocarcinoma, Sebaceous/diagnosis , Adenocarcinoma, Sebaceous/pathology , Adenocarcinoma, Sebaceous/surgery , Sebaceous Gland Neoplasms/diagnosis , Sebaceous Gland Neoplasms/pathology , Sebaceous Gland Neoplasms/surgery , Skin Neoplasms/pathology , Skin/pathology , Sebaceous Glands/pathologySubject(s)
Adenocarcinoma, Sebaceous , Sebaceous Gland Neoplasms , Skin Neoplasms , Humans , Keratin-7 , Mohs Surgery , Adenocarcinoma, Sebaceous/surgery , Adenocarcinoma, Sebaceous/pathology , Skin Neoplasms/surgery , Skin Neoplasms/pathology , Sebaceous Gland Neoplasms/surgery , Sebaceous Gland Neoplasms/pathologySubject(s)
Adenocarcinoma, Sebaceous , Eyelid Neoplasms , Sebaceous Gland Neoplasms , Humans , Adenocarcinoma, Sebaceous/diagnostic imaging , Adenocarcinoma, Sebaceous/pathology , Eyelid Neoplasms/diagnostic imaging , Eyelid Neoplasms/pathology , Eyelids , Sebaceous Gland Neoplasms/diagnostic imaging , Sebaceous Gland Neoplasms/pathology , Retrospective StudiesABSTRACT
Sebaceous carcinoma (SC) is a very rare and aggressive form of skin cancer that arises from the sebaceous glands. SC can occur anywhere on the body, but most commonly affects the head and neck, especially the upper eyelid. SC is the third most common malignancy of the eyelid and has the potential to metastasize and be fatal; therefore, it is vital for dermatologists to remain acquainted with this malignancy and its most current treatment options. Most commonly presenting as a painless lump or thickening of skin on the eyelid, SC has an insidious progression that may not prompt the patient to seek medical attention immediately. To avoid the potential of metastasis, early diagnosis and treatment is paramount. To assess if the cancer has spread, ophthalmology, imaging, and sentinel lymph node biopsy are recommended. This article provides a comprehensive review of SC's pathogenesis, current diagnostic methods, and treatments, including wide local excision, Mohs micrographic surgery, orbital exenteration, radiation, and other topicals. The prognosis of SC depends on several factors, including size, location, stage, and treatment method. After treatment of the neoplasm, diligent post-treatment surveillance remains the cornerstone of patient care. Continued dermatologic follow-ups are essential for early detection of reoccurrence, ensuring timely intervention and optimal long-term outcomes. In conclusion, this comprehensive review aims to equip dermatologists and other physicians with a nuanced understanding of SC, enabling them to provide effective care to support patients encountering this malignancy.
Subject(s)
Adenocarcinoma, Sebaceous , Sebaceous Gland Neoplasms , Humans , Adenocarcinoma, Sebaceous/diagnosis , Adenocarcinoma, Sebaceous/therapy , Adenocarcinoma, Sebaceous/pathology , Sebaceous Gland Neoplasms/diagnosis , Sebaceous Gland Neoplasms/therapy , Sebaceous Gland Neoplasms/pathology , Prognosis , Sentinel Lymph Node Biopsy/adverse effects , Mohs Surgery/adverse effectsABSTRACT
El carcinoma sebáceo de mama (CSM) es una neoplasia muy poco frecuente. De acuerdo con la clasificación actual de la OMS, el CSM se define como un carcinoma de la mama en el que sus células presentan diferenciación sebácea en al menos el 50% de las mismas y no existe ninguna relación con las glándulas sebáceas de los anejos de la piel. Acorde con esta definición tan solo encontramos 21 casos descritos en la literatura. Presentamos un nuevo caso de CSM junto a una revisión de características clínicas, anatomopatológicas y terapéuticas de esta rara estirpe tumoral de mama
Sebaceous gland carcinoma (SGC) is an exceedingly rare neoplasm. According to the WHO, SGC is defined as a breast carcinoma in which at least 50% of cells show sebaceous differentiation and there is no relationship with the sebaceous glands of the skin annexes. Only 21 reported cases in the literature fit this definition. We present a new case of this rare breast tumour, as well as a review of its clinical, histological and therapeutic features
Subject(s)
Humans , Female , Aged , Adenocarcinoma, Sebaceous/pathology , Breast Neoplasms/pathology , Carcinoma, Ductal/pathology , Immunohistochemistry/methods , Mammography/methods , Ultrasonography, Mammary/methods , Mastectomy/methods , Diagnosis, DifferentialABSTRACT
RESUMEN El carcinoma sebáceo es una neoplasia anexial poco común que puede originarse en cualquier lugar del cuerpo donde existan glándulas sebáceas, siendo la principal ubicación la cabeza y el cuello, existiendo la presentación ocular y extraocular. Alcanza entre 0,2% a 4,6% de todas las neoplasias cutáneas malignas, con mayor incidencia en los adultos mayores caucásicos. La presentación clínica habitual corresponde a un nódulo duro o quístico subcutáneo eritematoso o amarillento, indoloro, de crecimiento acelerado. Sin embargo, puede manifestarse como una amplia gama de lesiones cutáneas, lo que, agregado a su baja incidencia, sea altamente subdiagnosticado. Se presenta el caso de un paciente de 64 años con tumor exofítico malar izquierdo con ulceraciones y áreas de necrosis en la superficie, sin compromiso de párpado inferior, de agudeza visual ni motilidad ocular. Se realiza resección del tumor, cuya biopsia definitiva confirma diagnóstico de carcinoma sebáceo extraocular.
ABSTRACT Sebaceous carcinoma is a rare anexal neoplasm that can develop on any part of the body containing sebaceous glands, but nearly the 70% of sebaceous carcinoma occur on the head and neck, being two ways of presentation, ocular and extraocular. It represents 0.2-4.6 % of all malignant neoplastic cutaneous lesions, whit a peak incidence in the eighth decades of life, in caucasian people. Typical clinical presentation of sebaceous carcinoma is a painless, firm or cystic subcutaneous nodule described as pink to red-yellow, however clinical features can be quite varied, which added to its low incidence leads to the diagnosis is often delayed for months to years. We present the case of a 64 years old patient whit an exofitic malar tumor ulcerated and necrotic in the surface, without compromising the lower eyelid, vision or ocular mobility. The biopsy of the tumor resection shows an extraocular sebaceous carcinoma.
Subject(s)
Humans , Male , Middle Aged , Sebaceous Gland Neoplasms/surgery , Sebaceous Gland Neoplasms/pathology , Skin Neoplasms/surgery , Skin Neoplasms/pathology , Adenocarcinoma, Sebaceous/surgery , Adenocarcinoma, Sebaceous/pathology , Biopsy , Tomography, X-Ray Computed , NecrosisABSTRACT
The finding of a sebaceous carcinoma in a mature teratoma is rare in human pathology, with fewer than 10 cases currently reported in the literature. In this article, we report a case of sebaceous carcinoma in a mature teratoma of the ovary in a 59-year-old patient and discuss its histological findings.
Subject(s)
Humans , Female , Middle Aged , Ovarian Neoplasms/pathology , Sebaceous Gland Neoplasms/pathology , Adenocarcinoma, Sebaceous/pathology , Teratoma/pathologyABSTRACT
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