Hyaline-vascular Castleman's Disease Research Articles

Multicentric Hyaline-Vascular Castleman Disease: The Missing Link?

Introduction: Castleman disease (CD) is a rare lymphoproliferative disorder that encompasses distinct clinicopathological entities. Unicentric form of the disease (UCD) usually involves a single lymph node station, exhibits hyaline vascular (HV) pathological changes with follicular dendritic cell expansion, and may associate with specific and life-threatening complications including paraneoplastic pemphigus (PNP) and follicular dendritic cell sarcoma (FDCS). Plasma cell interfollicular infiltration is absent in the HV subtype as opposed to the Plasma-Cell (PC) or Mixed subtypes. PC subtype has been associated with inflammatory symptoms thought to be secondary to plasma cell infiltration and represents the majority of HHV8-negative “idiopathic” multicentric forms of the disease. Interestingly, patients with PC-UCD behave as PC-MCD, suggesting a stronger role of histology rather than Unicentric vs Multicentric phenotype in disease expression. We here studied the phenotype of HV-MCD, a rare subtype of CD characterized by hyaline-vascular pathological changes involving 2 or more lymph node stations. Methods: Patients were screened through the French national reference center for CD between January 1994 and June 2023. Cases were followed from first symptoms to last follow-up visit. Comparators consisted of patients with HV-UCD and patients with PC/Mixed iMCD (P/M-iMCD). All cases and biopsies were reviewed by three expert clinicians and one expert pathologist. Results: Sixteen patients with a diagnosis of HHV8-negative HV-MCD were identified. All patients had supra- and infradiaphragmatic involvement. Four were excluded because of a subsequent diagnosis of POEMS, systemic lupus erythematosus, tuberculosis, or inborn error of immunity. The 12 remaining patients were considered as having HV-iMCD and compared to 101 patients with P/M-iMCD and 139 patients with HV-UCD. Characteristics and comparison between the 3 groups are depicted in Table 1. When compared to HV-UCD, HV-iMCD displayed frequent systemic involvement with significantly higher ECOG, higher frequency of fever and splenomegaly, and higher levels of C-reactive protein, gammaglobulins, leukocytes and ferritinemia ( Figure 1). Age at diagnosis was not different between HV-UCD and HV-iMCD. No cases of TAFRO syndrome were observed in HV-UCD whereas 4 cases were observed in HV-iMCD. All cases of PNP (n=12) and FDCS (n=3) were observed in the HV-UCD group. We observed a younger age at diagnosis in HV-iMCD compared to P/M-iMCD (median 24 [range 13-70] vs median 50 [range 11-88] years respectively, p = 0.01). We also noted a trend towards lower levels of C reactive protein and gammaglobulins as well a higher albumin level in HV-iMCD vs P/M-iMCD but these differences were not significant ( Figure 1 and Table 1). Treatments were similar between HV-iMCD and P/M-iMCD with prescription of steroids or anti-IL6R as first line therapy in 8/12 (67%) and 61/101 (60%) respectively. As expected, surgery was the first line therapy in 98/139 (72%) of HV-UCD patients. One patient with HV-iMCD and two with HV-UCD died (logrank p = 0.10), whereas 13 died in the P/M-iMCD group (logrank p = 0.8). Conclusions: These data altogether indicate that HV-iMCD appears to be a distinct entity in CD landscape, sharing pathological aspects of HV-UCD and clinical/biological features of P/M iMCD. In line with this view, there seems to be no overlap between HV-UCD and HV-iMCD-related complications. The findings also suggest that nodal PC infiltration is not sufficient to explain the inflammatory pattern of iMCD.

Analysis of characteristics of four patients with adrenal unicentric Castleman disease.

Castleman Disease (CD) is a group of diseases with characteristic lymph node histopathology, characterized by marked enlargement of deep or superficial lymph nodes. Adrenal CD is rarely reported, and an accurate preoperative diagnosis of adrenal CD is difficult. We report four cases of CD in the adrenal gland confirmed by pathology and review the characteristics of this rare disease, highlighting the necessity of diagnostic evaluation and follow-up of the patients. All of the patients sought medical advice because of adrenal incidentalomas. No significant abnormalities were presented in the biochemistry or endocrine systems. The imaging suggested a moderate-to-large mass with uneven moderate contrast enhancement of the adrenal region, similar to a pheochromocytoma. All cases were misdiagnosed as pheochromocytomas before operation and finally confirmed by histopathology. Three cases were pathologically diagnosed as hyaline vascular CD, and one case was diagnosed as plasma cell CD. All the patients are alive without recurrence after a median follow-up of 8 years. The adrenal CD should be considered after excluding pheochromocytoma and malignancy in the adrenal region. The long-term prognosis of patients with complete resection of the mass is excellent.

Castleman Disease and Literature Review from Mankweng Hospital, Limpopo, South Africa

Castleman disease is a lymphoproliferative disorder and is easily confused with lymphoma or other lymphadenopathy. Castleman’s disease is divided into unicentric Castleman disease, which involved a single enlarged lymph node or region of lymph nodes, and multicentric Castleman disease, which involved multiple lymph node stations. In this paper we highlight the presentation of Castleman’s disease on a female patient to share our experience with literature review. Case Presentation: A 29 years old female patient was referred to our surgical outpatient clinic from the peripheral hospital with right & left painless neck cervical mass slowly growing which started 3 years ago along with a right breast lump and patient was RVD non-reactive. On physical examination she was severely wasted. She had a 4cm by 3cm mass of the right supraclavicular region & another mass of 3cm x 1 cm mass on the left supraclavicular area. Her abdominal examination revealed hepatomegaly which is extended 8 cm below the subcostal margin. Histology report of cervical mass concluded Reactive lymph nodes with features of hyaline vascular Castleman disease and Breast mass revealed fibroadenoma. Immunohistochemistry of cervical node biopsy for HHV8 is negative. Blood result of inflammatory markers: C - reactive protein was 459 mg/L. Conclusions: Owing to the rarity of this condition, Castleman Disease Collaborative Network (CDCN) guideline is important for assessment & treatment plan.

Imaging Features and Misdiagnosis Analysis of Hyaline Vascular Castleman Disease in the Parotid Region: A Case Report

Imaging Features and Misdiagnosis Analysis of Hyaline Vascular Castleman Disease in the Parotid Region: A Case Report

Hyaline-vascular Castleman disease with dysplastic follicular dendritic cells in liver: report of a case

本文报道1例发生于55岁男性的肝脏原发性透明血管型Castleman病伴滤泡树突细胞异型增生的病例。组织学表现为透明血管型Castleman病背景中滤泡间区和滤泡生发中心出现有异型的梭形肿瘤细胞，肿瘤细胞表达CD21、CD23、CD35。该病罕见，提示肝脏Castleman病应广泛取材避免遗漏滤泡树突细胞病变区域，造成疾病漏诊。.

Immunoglobulin G4-Related Disease (IgG4-RD) Mimicking Hyaline Vascular Castleman Disease

Abstract Introduction/Objective Immunoglobulin G4-related disease (IgG4-RD) is a newly emerging immune-mediated fibroinflammatory disease characterized by increased IgG-4+ plasma cells. In soft tissue and visceral organs, the IgG4+ plasma cells often accompany a storiform pattern of fibrosis with obliterative phlebitis. In lymph nodes, rare cases have been described in the literature mimicking Multicentric Castleman Disease. Methods/Case Report Herein, we present a case of a 59-year-old male with history of lung adenocarcinoma status post resection one year prior, who presented with several months of unintentional weight loss and worsening of cervical lymphadenopathy. CT scan of the neck demonstrated extensive cervical lymphadenopathy. Incidentally, patient had traveled to a foreign country and had a positive QuantiFERON result. Clinical suspicion was either nodal metastatic disease or TB infection. An excisional biopsy of the left cervical lymph node was performed. Microscopic examination revealed numerous hyperplastic follicles with significant hyaline deposition, concentrically thickened mantle zones, radially penetrating vessels, increased interfollicular vascularity and plasmacytosis; morphologic features commonly associated with Hyaline-Vascular Castleman Disease (HVCD). Additionally, plasmacytosis within germinal center was identified. Immunohistochemical stains demonstrated significant IgG4+ plasma cells (approaching 108/HPF) within the germinal centers. All plasma cells were polytypic. Results (if a Case Study enter NA) N/A. Conclusion Commonly described patterns for IgG4-RD in the literature are multicentric Castleman disease (MCD)-like, whereas, in our case, we present intrafollicular plasma cell proliferation pattern of IgG4-RD with classic background features of HVCD. Accurate diagnosis and distinction between these two entities is imperative for prognostication, treatment and clinical management of IgG4-RD.

IDIOPATHIC MULTICENTRIC CASTLEMAN DISEASE (IMCD) AS A MANIFESTATION OF POST-COVID-19 INFLAMMATORY SYNDROME

IDIOPATHIC MULTICENTRIC CASTLEMAN DISEASE (IMCD) AS A MANIFESTATION OF POST-COVID-19 INFLAMMATORY SYNDROME

A novel diagnostic tool for hyaline vascular Castleman disease versus lymphoma based on contrast-enhanced computed tomography in neck mass.

Castleman disease and lymphoma each have a distinct treatment plan; however, they share the same features on contrast-enhanced computed tomography. To assess the quantitative outcomes of Castleman disease versus lymphoma using contrast-enhanced computed tomography based on Hounsfield units (HU). We retrospectively reviewed eight patients with unicentric Castleman disease and 30 patients with lymphoma based on pathological diagnosis at China Medical University Hospital between 2015 and 2020. Preoperative computed tomography with contrast scans was reviewed, and the HU of each tumor were measured. This study included eight patients with unicentric Castleman disease (four men and four women; mean age, 33 years) and 25 patients with lymphoma (11 men and 14 women; mean age, 53 years). There was no significant difference in heterogeneity between the two diseases (0.161 ± 0.052 vs 0.239 ± 0.063, p = 0.22); however, enhancement in Castleman disease was higher than that in lymphoma (126.40 ± 31.90 vs 74.19 ± 7.11, p < 0.001), providing a very good diagnostic tool (cutoff point at 88.5-91.3, sensitivity 0.86/specificity 0.88). Furthermore, we found a highly linear relationship in Castleman disease, which was not noted in lymphoma. The value of HU provides a good diagnostic tool for the differential diagnosis of Castleman disease versus lymphoma in the neck lymph nodes. Considering the linear relationship in Castleman disease, an increasingly accurate differential diagnosis can be made.

Ixazomib-Based Regimen Followed by Autologous Hematopoietic Stem Cell Transplantation in the Treatment of POEMS Syndrome

To investigate the safety and efficacy of a new proteasome inhibitor Ixazomib followed by autologous hematopoietic stem cell transplantation (AHSCT) in the treatment of POEMS syndrome. The clinical manifestations, diagnosis and treatment process and follow-up results of 4 patients with POEMS syndrome who were treated with Ixazomib-based regimen combined with AHSCT in Wuhan No.1 Hospital from February 2018 to July 2020 were analyzed retrospectively. All patients were male, aged from 37-54 years old, with varying degrees of peripheral neuropathy, organ enlargement (liver, spleen or lymph nodes), circulatory overload (peripheral edema and/or pleural effusion), osteosclerosis, endocrine diseases (thyroid, gonads, etc.), skin changes (pigmentation, hemangioma, white nails, etc.), M protein, papilledema and other clinical manifestations and characteristics at the time of initial treatment. Two patients were pathologically diagnosed as hyaline vascular Castleman disease by lymph node biopsy. Three patients underwent lumbar puncture examinations and all showed elevated cerebrospinal fluid protein. All patients received at least 2 cycles of sequential AHSCT after induction chemotherapy based on ixazomib. The follow-up time was 10-28 months, and the median follow-up time was 16 months. All cases survived. The complications were controllable during the treatment. Moreover, the clinical symptoms related to the disease were improved to a certain extent after the treatment. The levels of vascular endothelial growth factor (VEGF) showed a gradual decline. Ixazomib combined with AHSCT is safe and effective in the treatment of POEMS syndrome.

ALK+ hyaline vascular Castleman disease: a new kid on the block.

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Follicular dendritic cell sarcoma.

SummaryFollicular dendritic cells (FDC) are mesenchymal-derived dendritic cells located in B-follicles where they play a pivotal role in triggering and maintaining B-cell adaptive immune response. In 1986 Dr. Juan Rosai first reported a series of neoplasms showing features of FDC and defined it as Follicular Dendritic Cell Tumor, subsequently renamed as “sarcoma” (FDCS). In its seminal and subsequent articles Rosai and colleagues highlighted the heterogeneous microscopic appearance of FDCS and its immunohistochemical and ultrastructural features.FDCS mostly occurs in extranodal sites (79.4% of cases) and lymph nodes (15.1%); in about 7%-10% of cases it is associated with hyaline-vascular Castleman disease. Given its significant growth pattern and cytological variability, FDCS can be confused with various neoplasms and even inflammatory processes. The diagnosis requires the use of a broad spectrum of FDC markers (e.g. CD21, CD23, CD35, clusterin, CXCL13, podoplanin), particularly considering that tumor antigen-loss is frequent. The inflammatory-pseudotumor-like (IPT-like) variant of FDCS, in addition to its peculiar histopathological and clinical features, is characterized by positivity of tumor cells for Epstein-Barr virus, representing a diagnostic requisite.No distinctive genetic and molecular anomalies have been identified in FDCS. It often carries an aberrant clonal karyotype and chromosomal structural alterations, frequently involving onco-suppressor genes. Direct or next generation sequencing showed alterations on genes belonging to the NF-κB regulatory pathway and cell-cycle regulators. In contrast to hematopoietic-derived histiocytic and dendritic cells tumors, FDCS typically lacks mutations in genes related to the MAPK pathway.FDCS recurs locally in 28% and metastasizes in 27% of cases. Extent of the disease, surgical resectability and histopathological features are significantly associated with the outcome. IPT-like FDCS behaves as an indolent tumor, even if it often recurs locally over years.Complete surgical excision is the gold standard of treatment. Data on targeted therapies (e.g.: tyrosine kinase inhibitors) or immune checkpoint inhibitors are very limited and responses are variable. A better understanding of the molecular drivers of this tumor may lead to potential new therapeutic strategies.

Mediastinal Lymphoproliferative Disorders.

Lymphoproliferative disorders comprise 50% to 60% of all mediastinal malignancies in both children and adults. Primary mediastinal involvement is rare (∼5%), whereas secondary mediastinal involvement by systemic disease is more common (10% to 25%). Primary mediastinal disease is defined as involvement by a lymphoproliferative disorder of mediastinal lymph nodes, the thymus, and/or extranodal mediastinal organs without evidence of systemic disease at presentation. In this review, the clinical, radiologic, histopathologic, immunohistochemical, and genetic features of some of the most characteristic mediastinal lymphoproliferative disorders are presented. The entities discussed here include: classic Hodgkin lymphoma with emphasis on nodular sclerosis and mixed cellularity types, and non-Hodgkin lymphomas, including primary mediastinal (thymic) large B-cell lymphoma, mediastinal gray zone lymphoma, mediastinal diffuse large B-cell lymphoma, thymic marginal zone lymphoma, mediastinal plasmacytoma, T-lymphoblastic lymphoma, and anaplastic large cell lymphoma. Although not a malignant process, hyaline vascular Castleman disease is also discussed here as this disorder commonly involves the mediastinum. Despite multiple advances in hematopathology in recent decades, the day-to-day diagnosis of these lesions still requires a morphologic approach and a proper selection of immunohistochemical markers. For this reason, it is crucial for general pathologists to be familiar with these entities and their particular clinicoradiologic presentation.

T-lymphoblastic leukemia/lymphoma with interfollicular growth pattern and Castleman-like morphologic features

Indolent T-lymphoblastic proliferation (iT-LBP) is a recently recognized benign pathologic condition commonly associated with Castleman disease. The lymph node of this condition shows preserved lymphoid architecture with proliferation of nonclonal T-lymphoblasts in the interfollicular area. iT-LBP is a rare condition and a diagnosis of exclusion. As a rule, it requires sophisticated techniques to exclude T-lymphoblastic leukemia/lymphoma (T-LBL/L). We present an unusual T-LBL/L in a lymph node mimicking iT-LBP. The case showed preserved nodal architecture, morphologic features of hyaline vascular Castleman disease, proliferation of T-lymphoblasts in the interfollicular area, and no clonal T-cell receptor gene rearrangement. The follow-up lymph node specimen excised 6 months later showed full-blown lymphoblastic lymphoma with an abnormal karyotype, near-early-T-precursor phenotype, partial CD19 expression, and SETD2 mutation.

Morphologic variant of follicular lymphoma reminiscent of hyaline-vascular Castleman disease.

Follicular lymphoma (FL) with hyaline-vascular Castleman disease (FL-HVCD)-like features is a rare morphologic variant, with fewer than 20 cases in the literature. Herein, we report a case of FL-HVCD in a 37-year-old female who presented with isolated neck lymph node enlargement. The excised lymph node showed features reminiscent of HVCD, including regressed germinal centers (GCs) surrounded by onion skin-like mantle zones, lollipop lesions composed of hyalinized blood vessels penetrating into regressed GCs, and hyalinized interfollicular stroma. In addition, focal areas of abnormally conglomerated GCs composed of homogeneous, small centrocytes with strong BCL2, CD10, and BCL6 expression were observed, indicating partial involvement of the FL. Several other lymphoid follicles showed features of in situ follicular neoplasia. Based on the observations, a diagnosis of FL-HVCD was made. Although FLHVCD is very rare, the possibility of this variant should be considered in cases resembling CD. Identification of abnormal, neoplastic follicles and ancillary immunostaining are helpful for proper diagnosis.

Primary hyaline vascular Castleman disease of the kidney: case report and literature review

BackgroundCastleman’s disease (CD) is an uncommon type of benign proliferation of the lymphoid tissue, characterized by local or systemic lymphadenopathy that most frequently appears in the mediastinum; involvement of the kidney is uncommon, and proliferation originating from the kidney is extremely rare. Herein, we report a rare case of hyaline vascular Castleman’s disease (HV-CD) in a 56-year-old male patient and discuss its morphological characteristics and differential diagnoses including mantle cell lymphoma (MCL), follicular lymphoma (FL), and nodal marginal zone lymphoma (NMZL).Case presentationA right upper-middle renal mass was detected after physical examination in a 56-year-old man without any clinical symptoms and a previous partial resection of the right kidney. Microscopically, the lymphoid follicles were increased in number and had expanded mantle zones and atrophic germinal centers. Vascular proliferation and hyalinization in the interfollicular zones were observed. Immunohistochemical staining showed CD20-positive cells in the mantle zones; CD21 and CD35 were expressed in the dendritic cells, CD3 was positive in a small number of T cells, and CD38 and CD138 were positive in the plasma cells. Additionally, Ki-67 expression was positive in the follicle centers. In contrast, staining for Bcl-2 in the germinal centers and cyclin D1 were negative. The immunohistochemical analysis combined with the morphological results supported the diagnosis of HV-CD. The patient recovered well after surgery.ConclusionsPrimarily renal HV-CD without lymph node hyperplasia or clinical symptoms is extraordinarily rare and different from the multicentric-type CD (MCD) with kidney involvement. Therefore, it is extremely important to improve the awareness of this diagnosis. Attention should be paid to the difference between HV-CD and common lymph node reactive hyperplasia, MCL, FL, NMZL, and so on. To avoid misdiagnosis as a renal malignant tumor requiring radical resection, distinguishing these diseases is crucial.

Hyaline vascular variant of unicentric Castleman disease of the tonsil: a case report

BackgroundCastleman disease (CD) is a lymphoproliferative disorder with an unknown etiology. The disease may be unicentric (UCD) or multicentric (MCD), and three histopathologic variants have been described: hyaline vascular (HV), plasma cell (PC), and mixed type. Extranodal CD is rare. Herein, we report a case of CD presenting as a tonsillar mass, which has not been documented in the literature.Case presentationThe patient was a 32-year-old man. Laryngoscopy revealed tonsillar hypertrophy, and the patient underwent a low-temperature plasma tonsillectomy. Microscopic examination of permanent sections showed lymphoid follicular hyperplasia, a portion of which appeared to be a fusion of nodular hyperplasia (composed of lymphoid follicles of variable size and shape). These distinctive follicles with atrophic hyalinized germinal centers and a broad mantle zone of small lymphocytes formed concentric rings (so-called onion-skin arrangement). Medium-sized vessels and a plethora of capillaries were present in the center of the lymphatic follicles, mantle zones, and interfollicular areas. A characteristic lollipop appearance was also observed due to the onion-skin arrangement of the expanded mantle zone lymphocytes with a vessel penetrating the germinal center. No aberrant lymphoid population was present based on CD3, CD5, CD20, CD79α, CD21, CD23, bcl-2, cyclin D1, and ki-67 immunostaining. Tests for human herpesvirus (HHV)-8 and Epstein Barr virus (EBV)-encoded small RNA (EBER) were negative. Therefore, a diagnosis of an HV variant UCD was rendered. The patient was treated by local excision without any other therapy based on the diagnosis. At the 7-month follow up, the patient had no recurrent symptoms or masses.ConclusionWe present an unusual case of a tonsil presenting hyaline vascular Castleman disease (HVCD). This study aims to highlight CD as a differential diagnosis that should be considered by otolaryngologists and pathologists for lymphoproliferative disorders of the tonsil.

High-Grade CD5-Positive Follicular Lymphoma With Hyaline-Vascular Castleman Disease–Like Follicles

High-Grade CD5-Positive Follicular Lymphoma With Hyaline-Vascular Castleman Disease–Like Follicles

Castleman Disease and Rosai-Dorfman Disease.

This chapter describes the main features of two different diseases, Castleman Disease (CD) and Rosai-Dorfman Disease (RDD). Castleman disease (CD) is a clinical and histopathologically heterogeneous lymphoproliferative disorder that encompasses at least three distinct entities with some common overlapping morphological features: Hyaline Vascular CD (HVCD), Unicentric Plasma Cell CD and Multicentric CD. The most important feature of HVCD is the presence of abnormal germinal centers with hyaline-vascular transformation, sometimes showing multiple germinal centers within a single reactive lymphoid follicle, this outlining HVCD as a disorder of follicular dendritic cells. Unicentric and multicentric CD are, in contrast, lymphoproliferative lesions. Proinflammatory hypercytokinemia is an essential feature of multicentric CD, distinguished by a florid clinical presentation. Rosai-Dorfmann Disease is a histiocytic proliferative disorder diagnosed by the presence of tissue infiltration by S100-positive CD1a-negative histiocytes and plasma cell aggregates, often with Russell bodies. A typical, though not specific, characteristic of the disease is emperipolesis. Initially considered to be an inflammatory/reactive condition, molecular studies suggest that at least some cases of RDD could be considered as a low-grade histiocytic neoplastic process.

Follicular lymphoma with hyaline-vascular Castleman-like features: analysis of 6 cases and review of the literature

Follicular lymphoma (FL) with features reminiscent of hyaline-vascular Castleman disease (CD) is an unusual morphologic variant that may create diagnostic difficulties. To our knowledge, only 5 cases of this variant have been reported. We describe the clinicopathologic features of 6 cases including 2 men and 4 women with a median age of 63 years (range, 41-77). Morphologically, all lymph node biopsy specimens showed at least a focal area of conventional FL; 4 cases showed neoplastic follicles with hyalinized blood vessels penetrating into germinal centers (lollipop-like lesions); 4 cases had interfollicular areas with increased vascular stroma; 2 cases showed small neoplastic follicles with prominent, onionskin-like mantle zones; and 1 case showed 2 or more germinal centers within follicles (twinning). The small neoplastic follicles were more cellular than lymphocyte-depleted follicles of true hyaline-vascular CD, and the interface between germinal centers and mantle zones was ill defined. No cases showed dysplastic follicular dendritic cells. Immunohistochemistry for BCL-2 was positive in all 6 cases. Flow cytometry immunophenotypic analysis showed a monotypic B-cell population in 2 of 3 cases assessed. Conventional cytogenetic or fluorescence in situ hybridization studies performed in 3 cases showed t(14;18)(q32;q21) or IGH-BCL2, supporting the diagnosis of FL. The cases presented here add clinicopathologic data to the few cases of FL with hyaline-vascular CD-like features reported previously in the literature. Distinguishing this variant of FL from hyaline-vascular CD is important given the differences in treatment and prognosis of patients with each disease.

Rare Location of Castleman's Disease in the Temporal Region: A Case Report Involving a Young Korean Woman and Review of the Literature.

Castleman's disease (CD) is an uncommon benign lymphoproliferative disorder of unknown etiology. Histopathologically, it is divided into three types: hyaline-vascular, plasma cellular, and multicentric CD. The mass usually presents asymptomatically; however, it can cause non-specific symptoms such as fever and fatigue. Although CD can be found wherever lymph nodes are present, 75% of cases are reported in the mediastinum, and occurrence in the head and neck is rare. Herein, we report a rare case of CD presenting as a superficial mass in the temporal region. To the best of our knowledge, this is the first report of temporal CD in Korea involving a young patient.