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Superficial CD34-Positive Fibroblastic Tumor
Superficial CD34-positive fibroblastic tumor (SCD34FT) is a relatively uncommon soft tissue neoplasm, first identified in 2014 and included in the latest, fifth edition of the World Health Organization Soft Tissue and Bone Tumors Classification. A subset of SCD34FTs that exhibit PRDM10 rearrangement may overlap with other PRDM10-rearranged soft tissue tumors, suggesting a shared pathogenic pathway. We report a case of a 16-year-old patient, one of the youngest diagnosed, with an SCD34FT on the forearm, featuring both PRDM10 rearrangement and a unique, diffuse expression of cytokeratin (CK AE1/AE3). This case contributes to the expanding literature on SCD34FT, highlighting its clinical and pathological diversity.
Superficial CD34‐positive fibroblastic tumor (SPFT) is an extremely rare neoplasm of borderline (intermediate) category. To the best of our knowledge, less than 40 cases have been reported in the English literature. It is imperative to understand and emphasize its cytological features as fine needle aspiration cytology (FNAC) is still considered a first line of investigation in such cases in many countries including India. We present a case of a young male aged 27 years who presented to the General Surgery OPD with a history of slow‐growing mass over right thigh for 7 years. FNAC and subsequent histopathological examination revealed a diagnosis of SPFT.
Aims
Superficial CD34‐positive fibroblastic tumour (SCD34FT) is an uncommon but distinctive low‐grade neoplasm of the skin and subcutis that shows frequent CADM3 expression by immunohistochemistry (IHC). In this study, prompted by an index case resembling ‘atypical fibrous histiocytoma (FH)’ that was positive for CADM3 IHC, we systematically examined a cohort of tumours previously diagnosed as ‘atypical FH’ by applying CADM3 and fluorescence in situ hybridization (FISH) for PRDM10 rearrangement, to investigate the overlap between these tumour types.
Methods and Results
Forty cases of atypical FH were retrieved, including CD34‐positive tumours ( n = 20) and CD34‐negative tumours ( n = 20). All tumours were stained for CADM3. All CADM3‐positive tumours were evaluated by FISH to assess for PRDM10 rearrangement. Eleven CD34‐positive tumours (11/20, 55%) coexpressed CADM3 and were reclassified as SCD34FT. None (0/20) of the CD34‐negative atypical FH were CADM3‐positive. Reclassified SCD34FT (10/11) arose on the lower extremity, with frequent involvement of the thigh ( n = 8). Features suggestive of atypical FH were observed in many reclassified cases including variable cellularity, spindled morphology, infiltrative tumour margins, collagen entrapment, epidermal hyperpigmentation, and acanthosis. Variably prominent multinucleate giant cells, including Touton‐like forms, were also present. An informative FISH result was obtained in 10/11 reclassified tumours, with 60% (6/10) demonstrating PRDM10 rearrangement.
Conclusion
A significant subset of tumours that histologically resemble atypical FH, and are positive for CD34, coexpress CADM3 and harbour PRDM10 rearrangement, supporting their reclassification as SCD34FT. Awareness of this morphologic overlap and the application of CADM3 IHC can aid the distinction between SCD34FT and atypical FH.
Superficial CD34-positive fibroblastic tumor (SCPFT) is a recently described disease entity characterized by marked nuclear pleomorphism, low mitotic count, and diffuse CD34 positivity. It is a rare, distinctive, low-grade fibroblastic neoplasm. To date, only 44 cases have been reported in the English-language literature. Herein, we report two cases of SCPFT involving a 48-year-old male and a 22-year-old male with superficial tumors on the right and left thighs, respectively. Excision was performed in both cases. Histologically, both tumors showed spindle-to-epithelioid cells arranged in fascicular or sheet-like patterns. Most cells displayed granular or eosinophilic glassy cytoplasm, marked nuclear pleomorphism, and a low mitotic rate. On immunohistochemical staining, tumor cells were diffusely positive for CD34 and negative for S100 protein, smooth muscle actin, and desmin. After wide excision, neither patient experienced recurrence or metastasis after 16 months and 11 months of clinical follow-up, respectively. To the best of our knowledge, these are the first two cases of SCPFT reported in Korea. We believe these case reports would contribute to the clinicopathological understanding of SCPFT and assist clinicians in differentiating this tumor from other superficial soft tissue neoplasms.
Background:
Superficial CD34-positive fibroblast tumors (SCPFTs) are newly recognized fibroblast and myofibroblast tumors representing intermediate tumors. To the best of our knowledge, fewer than 50 cases have been reported. Perianal SCPFT has not been previously reported.
Case summary:
A 55-year-old man was hospitalized upon discovering a painless perianal lump 10 d prior. Physical examination showed a lump of approximately 3 cm × 4 cm in the 7 to 8 o'clock direction in the perianal area. Perianal abscess was considered the primary diagnosis. Lump removal surgery was performed under epidural anesthesia. Postoperative pathology showed a well-circumscribed, soft tissue-derived, spindle-cell tumor with strong CD34 positivity by immunohistochemistry. The final diagnosis was perianal SCPFT. There were no complications, and the patient was followed for more than 8 mo without recurrence or metastasis.
Conclusion:
We report a case of perianal superficial CD34-positive fibroblast tumor. This rare mesenchymal neoplasm has distinctive histomorphology, which is important for diagnosis. Comprehensive consideration of clinical information, imaging, histology, and immunohistochemistry is important for diagnosis.
This study reports a case of superficial CD34-positive fibroblastic tumor (SCPFT) in a child and analyze the major known clinicopathological features of SCPFT and other skin mesenchymal tumors, contributing to an accurate diagnosis of this rare disease. We summarize the clinicopathologic features of an 8-year-old girl who was diagnosed with SCPFT and 46 previously reported SCPFT cases. Post-operative histopathologic examination of the current case showed the tumor lesion was well-circumscribed; tumor cells were spindled-to-polygonal with a fascicular pattern; most nuclei displayed hyperchromasia and low mitotic rate; intranuclear pseudoinclusions could be found; and abundant eosinophilic cytoplasm and partial myxoid stroma were observed. Immunohistochemistry revealed strong and diffuse CD34-positivity, vimentin staining positively but no S-100, SMA, NSE, CD31, desmin, cytokeratin, STAT6, β-catenin, MDM2, or ERG expression. The Ki-67 and CD68 labeling indexes were approximately 1%. There were no rearrangements of PDGFB or PRDM10 tested by FISH. After surgical resection, the patient had no signs of recurrence or metastasis at a 6-month follow-up. The present case is the first that describes SCPFT in children and has significant clinical implications. SCPFT should be differentiated from other skin mesenchymal tumors. The presented compilation of all so far published SCPFT cases will help in diagnosing successfully SCPFT and increasing awareness of this tumor to guide clinical practice.
2 0 1 7 I n d I a n J o u r n a l o f P a t h o l o g y a n d M I c r o b I o l o g y | P u b l I s h e d b y W o l t e r s K l u W e r ‑ M e d K n o W 377 Brief Communication ABSTRACT Earlier, categorized under low grade malignant fibrous histiocytoma and low grade sarcomas, 'superficial CD34 positive fibroblastic tumor', a newly proposed entity, is a low grade mesenchymal tumor of intermediate malignant potential. Morphological features include monomorphic neoplastic spindle cells arranged in a fascicular to storiform pattern along with sheets of pleomorphic epithelioid cells, and multinucleated giant cells with glassy cytoplasm. Diffuse vimentin and characteristic diffuse CD34 positivity is seen in all cases with few showing focal cytokeratin expression. Rest of the melanocytic and mesenchymal markers are negative. Herein, we present a case of superficial CD34‑positive fibroblastic tumor in arm swelling of a 16‑year‑old female. Differential diagnosis of the same on the basis of morphology as well as immunohistochemistry has also been discussed. This is the first case reported from India to the best of our knowledge.
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 is a recently described soft-tissue tumor entity. A 48 year-old-male presented with a gradually increasing soft-tissue mass in his right forearm of 2 years' duration, along with multiple subcutaneous soft-tissue nodular lesions, and reminiscent of lipomas over his body. He underwent a wide excision of his forearm mass. Microscopic sections showed a circumscribed tumor in the dermis and subcutaneous fat, composed of spindle cells, inflammatory cells, including lymphocytes, plasma cells, and eosinophils, along with interspersed markedly pleomorphic giant cells containing moderate-to-abundant “glassy” cytoplasm, vesicular nuclei, exhibiting prominent nucleoli, and intranuclear pseudoinclusions. There were no significant mitotic figures, areas of hemorrhage, necrosis, or pigment histiocytes. By immunohistochemistry, the tumor cells were diffusely positive for CD34 while negative for cytokeratin (CK), pan CK (AE1/AE3), S100 protein, CD30, and CD31. MIB1/Ki-67 was low and highlighted 4%–5% tumor nuclei. Diagnosis of superficial CD34-positive fibroblastic tumor was offered. Sections from the various resection margins were free of tumor. Postresection, the patient is alive with no evidence of disease for the past 8 months. This constitutes as one of the first case reports of this rare tumor entity from our country. Its diagnostic and treatment implications are discussed herewith.
Aims:
We describe an additional series of superficial CD34-positive fibroblastic tumor, a newly described neoplasm, to enhance the recognition of an emerging novel entity.
Methods and results:
The clinicopathological features and immunophenotypes of 11 cases of superficial CD34-positive fibroblastic tumor were studied. There were 8 males and 3 females with a median age of 36 years. Tumor occurred in the thigh (n=4), buttock (n=3), shoulder (n=2), upper arm (n=1) and waist (n=1). Histologically, all tumors were characterized by relative circumscription, pleomorphic spindled to polygonal cells with variable enlarged bizarre-appearing cells, intranuclear cytoplasmic pseudoinclusion, and extremely low mitotic activity. By immunohistochemistry, neoplastic cells demonstrated diffuse and strong expression for CD34 and focal staining of cytokeratin. Follow-up thus far revealed an indolent clinical behavior.
Conclusions:
Superficial CD34-positive fibroblastic tumor represents a new member in the family of cutaneous CD34-positive spindle cell tumors. Familiarity with its clinicopathological characteristics is helpful in avoiding confusion with a variety of cutaneous mesenchymal tumors with overlapping features. This article is protected by copyright. All rights reserved.
Superficial CD34-positive fibroblastic tumor is a low-grade mesenchymal neoplasm of superficial soft tissues characterized by fascicles of spindle to epithelioid cells displaying nuclear pleomorphism and strong diffuse CD34 immunoreactivity. The intraoperative imprint cytology preparations (ICP) of a superficial CD34-positive fibroblastic tumor from a 50-year-old female are described. To the best of our knowledge, there is no report of the cytologic findings of superficial CD34-positive fibroblastic tumor in the English medical literature. The ICP, differential diagnosis, tissue correlation, and ancillary studies of this fascinating entity are discussed. Diagn. Cytopathol. 2016. © 2016 Wiley Periodicals, Inc.
A 48-year-old Japanese woman presented with a painless, slow-growing, brown nodule of 15 mm in diameter on the left thigh. She noticed the nodule a few years before the first presentation to our hospital. She underwent total resection of the nodule. On histopathological examination, a relatively well-defined tumor with infiltrative growth was located in the dermis and extended into the subcutis. The tumor was composed of spindle to polygonal cells with pleomorphic nuclei arranged in a sheet-like or fascicular pattern. Tumor cells with granular cytoplasm were also scattered. Immunohistochemical examination revealed that the tumor cells were strongly positive for CD34. The fusion transcripts of the collagen type 1 alpha 1 platelet-derived growth factor beta chain were not detected. After excluding other fibroblastic tumors through histopathological and immunohistochemical examinations, a diagnosis of superficial CD34-positive fibroblastic tumor (SCPFT) was made. SCPFT is a recently proposed fibroblastic tumor that is characterized by striking pleomorphism, granular cytoplasm, low mitotic rate and diffuse CD34 expression. Only two reports with 20 cases have been reported so far. The present case is the first that corresponds to SCPFT in Japan.
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.Modern Pathology advance online publication, 26 July 2013; doi:10.1038/modpathol.2013.139.
Myxoinflammatory fibroblastic sarcoma (MIFS) is a low-grade malignant neoplasm for which limited genetic information, including a t(1;10)(p22;q24) and amplification of chromosome 3 material, is available. To further characterize these aberrations, we have investigated eight soft tissue sarcomas diagnosed as MIFS, haemosiderotic fibrolipomatous tumour (HFT), myxoid spindle cell/pleomorphic sarcoma with MIFS features, and inflammatory malignant fibrous histiocytoma/undifferentiated pleomorphic sarcoma with prominent inflammation (IMFH) harbouring a t(1;10) or variants thereof and/or ring chromosomes with possible involvement of chromosome 3. Using chromosome banding, fluorescence in situ hybridization, array-based comparative genomic hybridization, global gene expression, and real-time quantitative PCR analyses, we identified the breakpoint regions on chromosomes 1 and 10, demonstrated and delineated the commonly amplified region on chromosome 3, and assessed the consequences of these alterations for gene expression. The breakpoints in the t(1;10) mapped to TGFBR3 in 1p22 and in or near MGEA5 in 10q24, resulting in transcriptional up-regulation of NPM3 and particularly FGF8, two consecutive genes located close to MGEA5. The ring chromosomes contained a commonly amplified 1.44 Mb region in 3p11-12, which was associated with increased expression of VGLL3 and CHMP2B. The identified genetic aberrations were not confined to MIFS; an identical t(1;10) was also found in a case of HFT and the amplicon in 3p was seen in an IMFH.