Superficial CD34-positive Fibroblastic Tumor Research Articles

Diagnostic conundrum of Superficial CD34-positive fibroblastic tumor (SCPFT): a series of five cases

Diagnostic conundrum of Superficial CD34-positive fibroblastic tumor (SCPFT): a series of five cases

Superficial CD34-Positive Fibroblastic Tumor: A Case Series and a Review of Literature

Superficial CD34-Positive Fibroblastic Tumor: A Case Series and a Review of Literature

Usefulness of SynCAM3 and cyclin D1 immunohistochemistry in distinguishing superficial CD34-positive fibroblastic tumor from its histological mimics.

Superficial CD34-positive fibroblastic tumor (SCPFT) is a fibroblastic/myofibroblastic soft tissue tumor of rarely metastasizing intermediate malignancy. Some recent studies have described a relationship between SCPFT and PRDM10-rearranged soft tissue tumor (PRT) based on SynCAM3 and PRDM10 expression on immunohistochemistry. We performed CD34, cytokeratin AE1/AE3, SynCAM3, and PRDM10 immunohistochemistry in SCPFT and its histological mimics, including myxoinflammatory fibroblastic sarcoma (MIFS), superficially localized myxofibrosarcoma (MFS), and undifferentiated pleomorphic sarcoma. We also examined cyclin D1 expression because it is expressed in MIFS and MFS. We conducted fluorescence in situ hybridization (FISH) of PRDM10 rearrangement in SCPFT cases. On immunohistochemistry, only SCPFT showed strong and diffuse SynCAM3 expression. SCPFT also exhibited strong nuclear and weak cytoplasmic cyclin D1 expression, which was similar to that observed in MIFS. Two of five SCPFT cases exhibited nuclear PRDM10 expression. FISH revealed PRDM10 split signals in 44% and 24% of tumor cells in two SCPFT cases showing nuclear PRDM10 expression on immunohistochemistry, respectively. A minority of non-SCPFT cases showed focal SynCAM3 expression, but a combination of SynCAM3 and cyclin D1 in addition to CD34 and cytokeratin AE1/AE3 may be useful for the differential diagnosis of SCPFT and its histological mimics.

Superficial CD34 Positive Fibroblastic Tumour with Myxoid Stroma.

Superficial CD34 positive fibroblastic tumor is a rare low-grade neoplasm of the skin and subcutis with indolent behavior. This entity has been included in the current World Health Organisation (WHO) classification of soft tissue tumors. Pathological diagnosis can be challenging due to significant morphological overlap with other entities and the large spectrum of CD34 positive tumors. We report a case in a twenty-five male which showed characteristic diagnostic features, but in addition showed myxoid stroma. The presence of myxoid stroma has not been previously emphasized in this entity and broadens the histologic differential diagnosis significantly to include myxoid soft tissue tumors. A subset of these tumors harbor PRDM10-rearrangements, but a defining molecular feature has not yet been described, highlighting the need for further molecular characterization of this potentially genetically heterogenous tumor. Awareness of this entity among surgeons and pathologists is important to prevent misclassification as an aggressive sarcoma and avoid over-treatment.

Superficial CD34-Positive Fibroblastic Tumor: A Clinicopathologic, Immunohistochemical, and Molecular Study of 59 Cases.

Superficial CD34-positive fibroblastic tumor (SCD34FT) is a rare soft tissue neoplasm that shows overlapping features with PRDM10 -rearranged soft tissue tumor ( PRDM10 -STT). This study characterizes the clinicopathologic, immunohistochemical, and molecular features of SCD34FT in a series of 59 cases. Fluorescence in situ hybridization to assess for PRDM10 rearrangement was performed in 12 tumors. Immunohistochemistry for CADM3 and WT1 was performed; CADM3 was also assessed in histologic mimics. Our cohort of 33 male and 26 female had a median age of 42 (range: 14 to 85) years. Tumors were most commonly located in the lower limb (73%), upper limb (8%), back (7%), and supraclavicular region (3%). The median tumor size was 3.0cm (range: 1.0 to 9.0cm). Clinical follow-up in 32 patients (median duration: 26mo) revealed 2 local recurrences (6%). One patient developed regional lymph node metastases which were completely excised. Microscopically, SCD34FT comprised spindled and pleomorphic cells with glassy cytoplasm and occasional granular cell change. Fluorescence in situ hybridization confirmed PRDM10 rearrangement in 3/8 cases (38%). SCD34FT frequently expressed CADM3 (95%) and WT1 (75%). CADM3 was less diffusely positive in pleomorphic hyalinizing angiectatic tumor (40%), pleomorphic liposarcoma (20%), and undifferentiated pleomorphic sarcoma (10%). We corroborate that SCD34FT is indolent but may rarely metastasize to lymph nodes without adverse outcomes. CADM3 and WT1 may be useful in the distinction from histologic mimics. Since cases of SCD34FT with and without demonstrable PRDM10 rearrangement were clinicopathologically indistinguishable, our study further supports that SCD34FT and PRDM10 -STT likely constitute a single entity.

Overlapping morphological, immunohistochemical and genetic features of superficial CD34-positive fibroblastic tumor and PRDM10-rearranged soft tissue tumor

AbstractSuperficial CD34-positive fibroblastic tumor (SCD34FT) is a recently recognized soft tissue tumor that is considered to be of borderline malignancy. The pathogenesis of this tumor remains incompletely understood, but it has been suggested that SCD34FT overlaps with tumors showing fusions involving the PRDM10 gene. Previous analyses of PRDM10-rearranged tumors have demonstrated that they have a distinct gene expression profile, resulting in high expression of CADM3 (also known as SynCam3), which can be detected immunohistochemically. Here, we investigated a series (n = 43) of SCD34FT or PRDM10-rearranged tumors and potential mimics (n = 226) with regard to morphological, genetic, and immunohistochemical features. The results show that SCD34FT and PRDM10-rearranged tumor are morphologically indistinguishable; 41 of 43 tumors of both entities are CADM3-positive. Hence, we suggest that they constitute a single entity, preferably referred to as SCD34FT. Expression of CADM3 was only rarely seen in other soft tissue tumors, except in tumors with Schwann cell differentiation. Thus, IHC for CADM3, in combination with the characteristic morphological features, is a valuable adjunct in the diagnosis of SCD34FT.

Superficial CD34-positive fibroblastic tumor: a clinicopathological analysis of 25 cases

Objective: To investigate the clinicopathological features and immunophenotype of superficial CD34-positive fibroblastic tumor (SCDFT) with an emphasis on differential diagnosis. Methods: The clinicopathological data and immunohistochemical profiles of 25 cases of SCPFT diagnosed from March 2015 to June 2020 in Department of Pathology, Fudan University Shanghai Cancer Center, China were analyzed. The literature was reviewed. Results: There were 14 males and 11 females, with the age at presentation ranging from 16 to 60 years (mean 38 years; median 40 years). Tumor occurred in the thigh (n=9), buttock (n=4), upper arm (n=3), shoulder (n=2), waist (n=2), lower leg (n=2), chest wall (n=1), abdominal wall (n=1) and vulva (n=1). Most of the patients presented with a slowly growing cutaneous nodule, with a mean diameter of 2.6 cm (range 1-5 cm). The duration of symptoms ranged from 1 week to 30 years. Microscopically, most of the tumors were located in the deep dermis to superficial subcutis. They were well circumscribed but frequently showed focal infiltration into adjacent adipose tissue. Tumor cells were composed of fascicles and sheets of spindled to polygonal cells with abundant eosinophilic cytoplasm possessing frequent granular or glassy appearance. Lipidized cells were also present. The striking feature was the presence of hyperchromatic pleomorphic cells, containing conspicuous nucleoli and intranuclear pseudoinclusion. Mitotic activity was very low. Besides, there were inflammatory infiltrates in the stroma. All tumors showed strong and diffuse immunohistochemical staining of CD34, with frequent focal expression of CKpan and occasional immunoreactivity of desmin. Follow-up data, which were available in 18 patients (range 7 to 69 months), showed a local recurrence in one patient and disease-free in all others. Conclusions: SCPFT is a newly-recognized low-grade fibroblastic tumor. Due to the marked nuclear pleomorphism, it is not uncommon to misdiagnose SCPFT as other pleomorphic mesenchymal tumors. Familiarity with its clinicopathological characteristics is helpful in avoiding overdiagnosis and overtreatment.

Superficial CD34-positive fibroblastic tumor and PRDM10-rearranged soft tissue tumor are overlapping entities: a comprehensive study of 20 cases.

Superficial CD34-positive fibroblastic tumor (SCD34FT) and PRDM10-rearranged soft tissue tumor (PRDM10-STT) are rare mesenchymal tumors. These lesions have clinicopathological similarities, but their relationship remains controversial. This study aimed to characterise a series of cases of SCD34FT and PRDM10-STT. Ten lesions each of SCD34FT and PRDM10-STT were studied using immunohistochemistry, array-comparative genomic hybridisation (aCGH), RNA sequencing and exome sequencing. Tumors mainly occurred in young adults, were generally small (<5cm) and arose predominantly in the superficial soft tissues of the lower extremities. Follow-up data were available in 15 cases (SCD34FT, n=7, median 16months; PRDM10-STT, n=8, median 14months), local recurrences occurred in four cases (SCD34FT, two of 10; PRDM10-STT, two of 10), while no distant spread was documented. Morphologically, tumors were relatively well-circumscribed and composed of sheets and fascicles of spindle and pleomorphic cells showing low mitotic activity (<1/mm²) without necrosis. Other findings included: granular cell change, lipoblast-like cells, ectatic blood vessels with fibrinous material, myxoid stromal changes, metaplastic bone and increased mitotic activity (>1/mm²). All tumors diffusely expressed CD34, while pan-keratin and desmin were commonly seen focally. SynCAM3 was diffusely expressed in 12 cases (SCD34FT, n=5; PRDM10-STT, n=7), independently of fusion status. aCGH profiles were 'flat' (PRDM10-STT, n=4; SCD34FT, n=2) and exome sequencing showed no recurrent pathogenic mutations (PRDM10-STT, n=2; SCD34FT, n=4). Overall, the only morphological features seen exclusively in PRDM10-STT were myxoid stromal changes (three of 10) and metaplastic bone (two of 10). We expand the current knowledge on PRDM10-STT and SCD34FT and provide additional evidence for considering them as overlapping entities.

Clinicopathological features of superficial CD34-positive fibroblastic tumor.

BACKGROUNDSuperficial CD34-positive fibroblastoma (SCPFT) is a newly discovered mesenchymal tumor characterized by high polymorphism, low mitotic rate, and diffuse CD34-positive reactions.AIMTo further determine the clinicopathological features of SCPFT.METHODSWe retrospectively analyzed the clinicopathological data, immunohistochemistry results, and differential diagnoses of four patients with SCPFT and performed a literature review. Relevant fusion genes were also detected.RESULTSThe tumors were all located in the lower extremities and presented as slow-growing painless masses located in the dermis and subcutaneous tissue. Microscopically, the tumors were composed of spindle-shaped to epithelioid cells with scattered abnormal and pleomorphic nuclei on a fibrous or fibromyxoid background. Necrosis was not found in the tumor tissues, and mitotic figures were rare. Immunohistochemically, the tumor cells were strongly positive for vimentin and CD34, and CKpan showed focal positivity in two tumors. All four patients were followed (13-57 mo, mean 35 mo), and one patient experienced local recurrence. CONCLUSIONSCPFT is a newly discovered borderline mesenchymal tumor that can locally recur or even metastasize. Familiarity with its clinicopathological features will help avoid confusion with skin mesenchymal tumors with similar features.

浅表性CD34阳性成纤维细胞肿瘤一例

浅表性CD34阳性成纤维细胞肿瘤一例

PRDM10-rearranged Soft Tissue Tumor: A Clinicopathologic Study of 9 Cases.

Gene fusion transcripts containing PRDM10 were recently identified in low-grade undifferentiated pleomorphic sarcomas (UPS). Here, we describe the morphologic and clinical features of 9 such tumors from 5 men and 4 women (age: 20 to 61 y). Three cases had previously been diagnosed as UPS, 3 as superficial CD34-positive fibroblastic tumor (SCD34FT), 2 as pleomorphic liposarcoma, and 1 as pleomorphic hyalinizing angiectatic tumor. The tumors were located in the superficial and deep soft tissues of the thigh/knee region (4 cases), shoulder (2 cases), foot, trunk, and perineum (1 case each) ranging in size from 1 to 6 cm. All showed poorly defined cellular fascicles of pleomorphic cells within a fibrous stroma with frequent myxoid change and a prominent inflammatory infiltrate. All displayed highly pleomorphic nuclear features, but a low mitotic count. Most tumors were well circumscribed. One of 9 tumors recurred locally, but none metastasized. Immunohistochemically, all were CD34 and showed nuclear positivity for PRDM10; focal positivity for cytokeratins was seen in 5/6 cases. PRDM10 immunoreactivity was evaluated in 50 soft tissue tumors that could mimic PRDM10-rearranged tumors, including 4 cases exhibiting histologic features within the spectrum of SCD34FT. Except for 2/6 pleomorphic liposarcomas and 1/4 myxofibrosarcomas, other tumors did not show nuclear positivity but displayed weak to moderate cytoplasmic immunoreactivity. In conclusion, PRDM10-rearranged soft tissue tumor is characterized by pleomorphic morphology and a low mitotic count. Its morphologic spectrum overlaps with SCD34FT. Clinical features of this small series suggest an indolent behavior, justifying its distinction from UPS and other sarcomas.

Superficial CD34-Positive Fibroblastic Tumor Successfully Treated With Mohs Micrographic Surgery: ERRATUM.

Superficial CD34-Positive Fibroblastic Tumor Successfully Treated With Mohs Micrographic Surgery: ERRATUM.

Cytological investigation of superficial CD34-positive fibroblastic tumor

Cytological investigation of superficial CD34-positive fibroblastic tumor

Superficial CD34-Positive Fibroblastic Tumor Treated With Mohs Micrographic Surgery

Superficial CD34-Positive Fibroblastic Tumor Treated With Mohs Micrographic Surgery

Detailed analysis of a superficial CD34-positive fibroblastic tumor: A case report and review of the literature.

Superficial cluster of differentiation (CD)34-positive fibroblastic tumor (SCPFT) is a rare mesenchymal neoplasm of borderline malignancy. It is characterized by a superficial location, marked cellular pleomorphism, an extremely low incidence of mitotic figures, and strong CD34 immunohistochemical positivity. As SCPFT is a recently described neoplasm, its characteristics are yet to be fully elucidated. To the best of our knowledge, no detailed studies regarding the imaging findings and cytogenetic analyses of SCPFTs exist. The present study describes a typical case of an 18-year-old man who developed an SCPFT measuring 87×70×80 mm in the subcutaneous adipose tissue of his right thigh. Computed tomography (CT) revealed a well-marginated tumor without calcification, and the enhancement on CT was weak. The tumor demonstrated abnormal uptake on 2-(18F) fluoro-2-deoxy-D-glucose positron emission tomography (PET), with a maximum standardized uptake value of 2.57. Magnetic resonance imaging (MRI) revealed a clearly defined tumor that exhibited homogeneous low signal intensity on T1-weighted imaging and high signal intensity on T2-weighted imaging, with small lobulated structures. Histopathologically, the tumor was composed of irregular spindle-to-oval-shaped cells with eosinophilic glassy cytoplasm and hyperchromatic, bizarre and pleomorphic nuclei that frequently exhibited intranuclear pseudoinclusions. Immunohistochemically, the tumor cells were diffusely and strongly positive for CD34. The Mindbomb E3 ubiquitin protein ligase 1 labeling index was 8.6%. Ultrastructurally, the tumor cells exhibited irregular or convoluted nuclei with abundant euchromatin-prominent nucleoli. The cytoplasmic organelles consisted of scattered, abundant rough endoplasmic reticulum, mitochondria, lysosomes, ribosomal rosettes and aggregated lipid globules. Of 18 metaphase cells identified, 2 demonstrated translocation between chromosomes 2 and 5 in cytogenetic studies. To the best of our knowledge, this is the first study describing imaging data (CT, MRI and PET-CT) and chromosomal aberrations for SCPFT.

Superficial CD34-positive fibroblastic tumor: report of a case

Superficial CD34-positive fibroblastic tumor: report of a case

Superficial CD34－positive fibroblastic tumorの1例

39歳，女性。初診の約1年前からある左大腿屈側の硬結を近医にて切除された。病理組織像では真皮深層から皮下脂肪組織浅層にかけて周囲との境界がやや不明瞭な限局性の結節があり，束状となって交差する単調な紡錘形細胞や，緩やかな束状やシート状に分布する硝子様や顆粒状，黄色腫様の胞体をもつ腫瘍細胞で構成されていた。腫瘍細胞の核は大型で多形性があり，核内偽封入体や大型の核小体，核分裂像も散見された（15個/50高倍率視野）。免疫組織化学的染色では，腫瘍細胞は，CD34がびまん性に強陽性，AE1/AE3，S－100 protein，α－SMA，Desmin，CD68，Factor XIIIaは陰性であった。Ki－67の陽性率は＜5％であった。中年の浅い軟部組織に限局する小型の病変で，CD34にびまん性に強陽性を示す多形性の目立つ腫瘍細胞で構成されており，核分裂像が目立たず，Ki67の陽性率が低い特徴などからsuperficial CD34－positive fibroblastic tumorと診断した。本症は稀な低悪性度の軟部腫瘍である。他疾患との鑑別点について解説した。

Superficial CD34-positive fibroblastic tumor: report of 18 cases of a distinctive low-grade mesenchymal neoplasm of intermediate (borderline) malignancy

Fibroblastic mesenchymal tumors show a spectrum of biological behavior, from benign to fully malignant. We report our experience of two decades with a distinctive, previously undescribed low-grade fibroblastic tumor of the superficial soft tissues. Eighteen cases were identified within our consultation files, previously coded as ‘low-grade sarcoma, not further classified' and ‘malignant fibrous histiocytoma, low grade'. The tumors occurred in adults (median age 38 years, range 20–76 years) of either sex (10 males and 8 females), ranged in size from 1.5 to 10 cm (mean 4.1 cm), and were confined to the superficial soft tissues of the thigh (N=5), knee (N=2), and other sites. Histological features included a fascicular growth pattern of the neoplastic spindled cells with striking, often bizarre cellular pleomorphism and variably prominent nucleoli. Necrosis was seen in one case. All cases showed strong, diffuse CD34 positivity and 68% of tested cases demonstrated focal cytokeratin expression. Desmin, ERG, FLI-1, smooth muscle actin, and S100 protein were negative. TP53 overexpression was absent. Fluorescence in-situ hybridization studies for TGFBR3 and/or MGEA5 rearrangements were negative in all tested cases. Clinical follow-up was available in 13 patients (median duration of 24 months; range 1–104 months). Twelve of 13 patients had no disease recurrence. One patient had regional lymph node metastases, 7 years after incomplete excision of the primary tumor. All patients are currently alive and disease free. The unique clinicopathological features of superficial CD34-positive fibroblastic tumor define them as a novel subset of low-grade fibroblastic neoplasms, best considered to be of borderline malignancy.