Dermatofibrosarcoma Research Articles

Incidence and Survival Outcomes of Dermatofibrosarcoma Protuberans From 2000 to 2020: A Population-Based Retrospective Cohort Analysis.

Recent changes in the incidence and survival of dermatofibrosarcoma protuberans (DFSP) have not been described. To characterize the incidence and survival of DFSP. A retrospective cohort study of patients with DFSP from 2000 to 2020 in the Surveillance, Epidemiology, and End Results database was performed. Cox and Fine-Gray regression models were used to assess overall and DFSP-specific survival. The incidence of DFSP has not changed from 2000 to 2020 with 4.6 cases/million person-years, with higher rates in dark-skinned and middle-age individuals. Factors associated with overall mortality in DFSP patients include advanced age ( p < .0001), male sex (hazard ratio [HR] 1.8, p < .0001), larger tumors (HR 1.002 per millimeter, p < .001), lower household income (HR 1.8, p = .0002), and lower extremity location (HR 1.7, p = .008). Mohs surgery is associated with improved overall survival (HR 0.4, p = .02). Large tumor size (6.0+ cm, HR 6.7, p = .01) and advanced age (age 80+ years, HR 21.3, p = .003) were associated with worse DFSP-specific mortality. Dermatofibrosarcoma protuberans incidence has remained constant from 2000 to 2020. Increasing age and tumor size, decreased income, male sex, and lower extremity location are associated with worsened survival. Mohs surgery is associated with improved overall survival. Increased age and tumor size are associated with worsened DFSP-specific mortality.

Unraveling intratumoral complexity in metastatic dermatofibrosarcoma protuberans through single-cell RNA sequencing analysis.

Dermatofibrosarcoma protuberans (DFSP) stands as a rare and locally aggressive soft tissue tumor, characterized by intricated molecular alterations. The imperative to unravel the complexities of intratumor heterogeneity underscores effective clinical management. Herein, we harnessed single-cell RNA sequencing (scRNA-seq) to conduct a comprehensive analysis encompassing samples from primary sites, satellite foci, and lymph node metastases. Rigorous preprocessing of raw scRNA-seq data ensued, and employing t-distributed stochastic neighbor embedding (tSNE) analysis, we unveiled seven major cell populations and fifteen distinct subpopulations. Malignant cell subpopulations were delineated using infercnv for copy number variation calculations. Functional and metabolic variations of diverse malignant cell populations across samples were deciphered utilizing GSVA and the scMetabolism R packages. Additionally, the exploration of differentiation trajectories within diverse fibroblast subpopulations was orchestrated through pseudotime trajectory analyses employing CytoTRACE and Monocle2, and further bolstered by GO analyses to elucidate the functional disparities across distinct differentiation states. In parallel, we segmented the cellular components of the immune microenvironment and verified the presence of SPP1+ macrophage, which constituted the major constituent in lymph node metastases. Remarkably, the CellChat facilitated a comprehensive intercellular communication analysis. This study culminates in an all-encompassing single-cell transcriptome atlas, propounding novel insights into the multifaceted nature of intratumor heterogeneity and fundamental molecular mechanisms propelling metastatic DFSP.

Outcome of Surgical Management of Dermatofibrosarcoma Protuberance: A Single-Institution Multidisciplinary Approach.

Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing, and locally aggressive soft tissue tumor with a high recurrence rate and metastatic potential, even with the proper treatment. This was a retrospective (case series) study that took place at King Abdulaziz Medical City, Riyadh, Kingdom of Saudi Arabia, to determine the outcomes of and appropriate margin excision for DFSP. All patients who were diagnosed with DFSP from 2016 to 2021 were included. The following variables were assessed: demographics, tumor characteristics, management options, and most importantly, whether patients were managed with an oncology-oriented approach or a non-oncology-oriented approach. There were a total of seventeen patients with DFSP, four of whom had fibrosarcomatous differentiation (FS-DFSP). The majority (N = 13, 76.5%) of the patients were females. The lower extremities and back were the most common locations for DFSP, accounting for 47.1% and 23.5%, respectively. Only two (11.76%) patients had metastatic disease, one of whom had FS-DFSP. The minimum resection margin was 3 cm, and the maximum was 5 cm. Thirteen (76.47%) patients were managed with an oncology-oriented approach (Group I), 23% of whom had post-excision positive margins. However, all patients who were managed with a non-oncology-oriented approach (Group II) had positive margins post-excision. More than three-quarters (76.9%) of group I underwent wide resection. Split-thickness skin grafting and primary closure were the most commonly used reconstruction methods in groups I and II, respectively. The mean planned margins in groups I and II were 3.9 cm and 1.7 cm, respectively. The findings of this study suggest that a planned wide-margin excision with a minimum safe margin of 3-5 cm should be implemented to reduce the recurrence, metastasis, and need for further surgeries in patients with DFSP.

Reconstruction of an Abdominal Hernia after Wide Tumor Resection with Multilayer Acellular Dermal Matrix and Skin Graft: A Case Report

An abdominal hernia is a relatively common complication after abdominal surgery. However, no reports have previously described the reconstruction of a soft tissue defect with a hernia after wide excision of a tumor in the abdominal wall. A 41-year-old man presented with a painless, rapidly growing mass in his abdominal wall. Enhanced magnetic resonance imaging of the abdomen revealed a 7.8×6.4×4.2 cm mass, consistent with a soft tissue sarcoma suspected to be angiosarcoma in the subcutaneous layer of the left anterior upper abdominal wall. After wide excision of the mass, a hernia measuring 6.0×5.8 cm was observed. The abdominal hernia was repaired using multilayered acellular dermal matrix (ADM), and the soft tissue defect was covered with a split-thickness skin graft. A histopathological examination confirmed that the mass was fibrosarcomatous dermatofibrosarcoma protuberans (DFSP-FS). No complications occurred after surgery, and the patient was followed up for 10 months without any issues. Herein, we present a case of hernia after the removal of DFSP-FS in the abdominal wall, which was repaired using multilayer ADM and a split-thickness skin graft.

A challenging fibrosarcomatous variant of dermatofibrosarcoma protuberans of head and neck: a case report

Dermatofibrosarcoma protuberans (DFSP) is an unusual tumour of soft tissue with local invasive property with high rate of recurrence after surgical treatment. DFSP is frequently seen on the trunk and proximal extremities, although a 10-15% cases accounts in head and neck region. Mainstay for success of surgery in DFSP remains complete removal of tumour with adequate surgical margins of approximately 3cms. In head neck region challenges associated with surgery may be inadequate surgical margins, poor functional and cosmetic outcomes. We present a case of a 35 years old male reported to our hospital with complaint of rapidly growing mass over right cheek and retroauricular region causing facial deformity. Incisional biopsy diagnosis was made DFSP. The patient underwent wide local excision of tumour followed by adjuvant radiotherapy. Final histopatholgy showed fibrosarcomatous transformation of DFSP. After 24 months of post-op follow up, patient recovery was satisfactory without any signs of recurrence. This case is presented for its rarity and it highlights the need for proper diagnosis and treatment plan.

Factors associated with time to surgical treatment for dermatofibrosarcoma protuberans

Factors associated with time to surgical treatment for dermatofibrosarcoma protuberans

Basal Cell Carcinoma Mimicking Dermatofibrosarcoma Protuberans: Case Report

Introduction: Basal cell carcinoma (BCC) is the most common type of carcinoma worldwide. BCC development is the result of a complex interaction between environmental, phenotypic and genetic factors. Presentation is highly heterogeneous, presenting from superficial or nodular lesions with a good prognosis to very extensive lesions in atypical locations clinically and radiologically mimicking other tumors. Case presentation: We present the case of a 34-year-old man with no past medical history, with an asymptomatic nodule that was increasing in size on the right thigh. High-frequency ultrasonography (HFUS) evaluation revealed an oval-shaped lesion with mixed echogenicity and posterior enhancement, with Doppler showing increased vascularization. A biopsy of the lesion was performed with suspicion of dermatofibrosarcoma protuberans, reporting unexpectedly an infiltrative basal cell carcinoma. The tumor was excised with free margins in the final pathology report. Conclusions: HFUS is an increasingly utilized tool that can accompany the study of a malignant lesion. Further studies are required to define more specific criteria for different skin tumors, always in conjunction with histology.

Analysis of H3k27me3 Expression in Malignant Peripheral Nerve Sheath Tumor (MPNST) and Other Spindle Cell Sarcoma Mimicking MPNST

Malignant Peripheral Nerve Sheath Tumor (MPNST) is a type of spindle cell sarcoma with approximately 5% of all sarcomas. Its diagnosis is challenging due to the absence of specific immunohistochemical markers. Recently, H3K27me3 was discovered as a potential specific immunohistochemical marker to differentiate MPNST from other sarcomas and distinguish between low and high-grade MPNST. Therefore, this research aims to investigate the use of the H3K27me3 as a potential specific marker for Malignant Peripheral Nerve Sheath Tumor (MPNST). A cross-sectional analysis was conducted on 50 cases of sarcomas, including 13 MPNST, 14 synovial sarcomas, 13 dermatofibrosarcoma protuberans (DFSP), and 10 leiomyosarcomas originating from the Department of Anatomical Pathology, Faculty of Medicine, Universitas Indonesia, Cipto Mangunkusumo Hospital (FMUI-CMH) from January 2013 to December 2021. H3K27me3 images were obtained and categorized as complete loss when more than 95% of the tumor cells showed loss of nuclear staining. The results found in MPNST showed a loss of H3K27me3 expression, which is statistically significant compared to other sarcomas mimicking MPNST (p=0.021), indicating its potential as a diagnostic marker. There is a difference in the expression of H3K27me3 between the high and low-grade MPNST but it is not statistically significant (p=0.105). This showed that H3K27me3 loss of expression can be used to diagnose MPNST, especially high-grade MPNST, and differentiate it from other sarcomas mimicking MPNST.

Connexin 43 in Dermatofibroma and Dermatofibrosarcoma Protuberans: Diagnostic, Pathogenic, and Therapeutic Implications.

Connexins play a crucial role in the formation of gap junctions that connect cells to each other, as well as cells to the surrounding environment. In recent years, connexin 43 has been extensively studied in various human tumors. In this study, we conducted an immunohistochemical analysis to evaluate the expression of connexin in 16 dermatofibromas (DFs) and 13 dermatofibrosarcoma protuberans (DFSP). Connexin was diffusely expressed in the cytoplasm of all DFs with moderate or strong intensity, whereas all DFSPs showed negative staining. In addition to its diagnostic implications, the loss of Cx43 may elucidate the invasive capacity of DFSP and offer a potential avenue for future therapeutic interventions.

DARIER-FERRAND DERMATOFIBROSARCOMA OF THE FACE: TWO CASES REPORT

Darier-Ferrand dermatofibrosarcoma or dermatofibrosarcoma protuberans (DFSP) is a cutaneous mesenchymal tumor of intermediate malignancy. It is a rare but not exceptional tumor, accounting for 0.1% of malignant skin tumors. Histological examination is essential for diagnosis. Wide surgical excision is the standard treatment. We report two cases of Darier-Ferrand dermatofibrosarcoma of the face. A wide local excision is the gold-standard treatment, with negative margins of 3–5 cm from the tumor edge including the skin, the subcutaneous tissue, and the underlying fascia. However, this is a mutilating surgery, especially for cervicofacial lesions which is the case for our two patients For cervico-facial localizations,some authors advise the Mohs micrographic surgery for its low recurrence rates described. Target adjuvant therapy with imatinib has been shown to reduce tumor size and improve surgical resectability and can be proposed as an option for our patients. However, its unavailability and high-cost limit its use in our context. A combination of conservative excision and adjuvant radiotherapy was reported to reduce the rate of local recurrence and should be also considered by practicians. To improve prognosis, early, codified, multidisciplinary management is essential for this pathology.

Dawn of Staging for Head and Neck Soft Tissue Sarcoma: Validation of the Novel 8th Edition AJCC T Classification and Proposed Stage Groupings

After decades of stagnation, the 8th edition TNM (TNM8) introduced a new T classification for head and neck (HN) soft tissue sarcomas (STS). New size cutoffs of 2 and 4 cm define T1-3, and a novel T4 category is defined by local invasion of adjoining structures. These size cutoffs had been chosen arbitrarily to advance data collection in this unique disease site since literature showed approximately 70% of HN STS did not reach the previous size threshold (5 cm) for the existing T1 category. The definition of the TNM8 T categories also align with mucosal HN cancers. No stage grouping for HN STS was defined since this new classification required more data collection to derive stage groups. This study aims to validate the TNM8 T classification and to propose stage groupings. Clinical data of all adult (>16 years) HN STS patients treated from 1988 - 2019 with curative intent in our tertiary cancer center were retrieved from a prospective database, and supplemented with chart review. As per TNM8, cutaneous angiosarcoma, embryonal and alveolar rhabdomyosarcoma, Kaposi sarcoma, and dermatofibrosarcoma protuberans were excluded due to their different behavior. Multivariate analysis (MVA) identified prognostic factors for overall survival (OS). Adjusted hazard ratios (AHR) and recursive partitioning analysis (RPA) were used to derive stage groupings. Stage grouping performance for OS was assessed and also compared against the existing TNM8 groups for non-HN STS. A total of 221 patients (N1: 2; M1: 2) were included. Of the 219 M0 patients, 63% were males; median tumor size was 3.0 cm (range: 0.3-14.0); the proportion of TNM8 T1-T4 were 35%, 34%, 26%, and 5%, respectively. Median follow up was 5.9 years. Five-year OS was 79%. MVA confirmed the prognostic value of T category (T4 HR 7.73, 95% CI 3.62-16.5) and grade (G2/3 vs G1 HR 3.7, 95% CI 1.82-7.53), in addition to age (HR 1.03, 95% CI 1.01-1.04) (all p<0.001) for OS. AHR model derived T1-3_Grade 1 as stage 1; T1-3_Grade 2/3 as stage II; and T4_any Grade or any T_N1 as stage III (Table 1); the corresponding 5-year OS was 93%, 73%, and 38%, respectively. Both patients with M1 died within 1.5 years after diagnosis and M1 disease was designated stage IV. The AHR-grouping outperformed the RPA and non-HN TNM8 stage grouping for hazard consistency, hazard discrimination, percent variance explained, hazard difference, and sample size balance. The novel T4 category introduced in TNM8 is associated with a >7 fold increased risk of death. Grade continues to be a critical prognostic factor in HN STS. The TNM8 HN STS T classifications have been validated, and the proposed new stage groupings with TNM8 incorporating grade have excellent performance for OS.

Survival outcomes of rare cutaneous malignancies within an insured cohort of patients, 1988-2018

There are limited survival data on cutaneous angiosarcoma (CAS), dermatofibrosarcoma protuberans (DFSP), Merkel cell carcinoma (MCC), and sebaceous carcinoma (SC). To analyze survival trends in CAS, DFSP, MCC, and SC among a racially diverse, insured cohort of patients. Using data from the Kaiser Permanente Southern California Cancer Registry, we identified adults diagnosed with CAS, DFSP, MCC, or SC between January 1, 1988 and December 31 2018, followed through December 31, 2021. Our cohort consisted of 83 diagnoses of CAS, 490 diagnoses of DFSP, 411 diagnoses of MCC, and 249 diagnoses of SC. Our analysis revealed no significant differences in overall or disease-specific 1000 person-years mortality rates among our populations of non-Hispanic Whites, Hispanics, African American/Blacks, and Asian American/Pacific Islanders diagnosed with CAS, DFSP, MCC, or SC. On multivariate analysis, controlling for patient and tumor characteristics, there was similarly no increased risk of overall mortality for minorities diagnosed with CAS, DFSP, MCC, or SC. Retrospective nature of the analysis and small sample size. Contrary to existing literature, our results show a notable lack of racially driven survival disparities among insured individuals with CAS, DFSP, MCC, and SC, emphasizing the importance of health care coverage.

The Burden of Skin Cancers in Saudi Arabia Through 2011-2022.

Introduction Skin cancers are classified into melanoma and non-melanoma or keratinocyte cancers. No recent data are found about the epidemiology of skin cancers in Saudi Arabia. The current study aims to determine the burden of skin cancer in the last 11 years from 2011 to 2022. Methods Patients who were diagnosed with any type of skin cancer were enrolled in the study. The diagnosis was conducted based on histopathology and immunohistochemistry. Different variables like age, type of cancer, type of lesions, and treatment approach used were measured. Results A total of 91 patients were diagnosed with skin cancers during the study period. The head and neck were the most common sites for skin cancers. Only 4/91 cases reported invasive melanoma. Both squamous cell carcinoma (SCC) (34/91) and basal cell carcinoma (BCC) (28/91) were found to be the most reported skin cancers. Other cancers including mycosis fungoides (MF) (10/91), Kaposi's sarcoma (6/91), and dermatofibrosarcomaprotuberans (DFSP) (5/91) were also detected. The rest of the detected tumors were rarely detected. Aggressive CD4+/CD4+/CD8+ MF was more prevalent than CD3+/CD4+/CD8- MF cancer cases. CD34+ /S100- DFSP cancers were evident in most of the DFSP cases. Human herpes virus 8 was detected in all Kaposi's sarcoma cases and all of them were HIV-confirmed cases. Surgical treatment was the most frequently used approach to treat skin cancers, followed by phototherapy (9.9%), surgical/radiotherapy (5.5%), surgical/chemotherapy (4.4%), chemotherapy (3.3%), and then chemoradiotherapy immunotherapy (1.1%). Conclusion The incidences of SCC and BCC are relatively high in comparison to other types of skin cancers with the surgical intervention being most frequently used.

"Unusual Presentation of Epitheloid Dermatofibroma- Rare Case Report".

Dermatofibroma is a commonly occurring cutaneous entity usually centered within the skin's dermis. Dermatofibromas are referred to as benign fibrous histiocytomas of the skin, superficial/cutaneous benign fibrous histiocytomas, or common fibrous histiocytoma. These mesenchymal cell lesions of the dermis clinically are firm subcutaneous nodules that occur on the extremities in the vast majority of cases and may or may not be associated with overlying skin changes.A 20years old male presented to ENT OPD, at a private hospital, with complaints of a huge mass over right side of face since 15years, which was slowly growing and not associated with pain. On clinical examination, hard, non tender, lobulated cauliflower like mass located over right side of face extending from right side temporal region to upper border of mandible from superior to inferior. From anterior to posterior it was extending from lateral 1/3rd of forehead and covering lateral canthus of right eye upto right side tragus. We have taken incisional biopsy which was suggestive of dermatofibroma. Then surgery was performed with patient's consent. Excision with 1cm free margin was done. Raw area was covered with full thickness skin grafting and advancement flap. We found no recurrence till date. Dermatofibroma in the head and neck region is less common and often present a difficult differential diagnosis like Dermatofibrosarcoma protuberans, Kaposi Sarcoma, Basal cell carcinomas. The aim of case report is to represent case of dermatofibroma of epitheloid variety which is unusual in size.

Dermatofibroma Versus Dermatofibrosarcoma Protuberans: A Nuclear Morphology Study.

The locally invasive soft-tissue sarcoma, dermatofibrosarcoma protuberans (DFSPs), shares certain histologic features of the much more common and benign dermatofibroma (DF). While immunohistochemical stains, specifically cluster of differentiation 34 and Factor XIIIa, can be used to distinguish the 2 entities using microscopy, these markers are not entirely sensitive nor specific. Three-dimensionally, DFSP nuclei resemble a "puck" or "coin"-like shape. As hematoxylin/eosin-stained slides are prepared, these "puck" nuclei are fixed in an infinite number of orientations depending on their current position in rotation about their axes within the tumor cells. Under histological examination, this random nuclear positioning produces the appearance of 2 predominate morphologies: an ovoid "disk" shape (en face) and a narrow spindled shape (side view), which distribute in a roughly 50:50 ratio throughout the tumor sample slide. Nuclear morphology was analyzed in 324 DFSP and DF samples at high magnification (×400) to determine the presence or absence of a predominant morphology in which nuclei appear to alternate between an ovoid (en face) and spindled (side view) throughout most of the tumor sample. An alternating ovoid-spindled nuclear morphology was the predominant cytology in 98% of DFSP and was not predominant in 100% of DF samples (P < 0.001). This morphology was found to be highly specific (Sp = 1) and sensitive (Sn = 0.98) for DFSP. This unique nuclear morphology may be a more sensitive and specific diagnostic tool in identifying DFSP from DF in comparison with costly immunohistochemical stains.

Diagnostic value of high-frequency ultrasound (HFUS) in evaluation of subcutaneous lesions.

It is unknown whether high-frequency ultrasound (HFUS) can evaluate invisible subcutaneous lesions. We aimed to investigate the diagnostic value of HFUS in invisible subcutaneous lesions. Patients with invisible subcutaneous lesions were prospectively recruited from two centres. Before undergoing biopsy or surgery, each lesion was independently evaluated by two clinicians. One provides a clinical diagnosis by only clinical examination and the other provides an integrated diagnosis by combining clinical examination and HFUS information. Diagnoses were classified as correct, wrong, and indeterminate. A total of 391 lesions from 355 patients were enrolled, including 225 epidermoid cysts, 77 lipomas, 25 pilomatrixomas, 21 haemangiomas, 19 dermatofibromas, 11 dermatofibrosarcoma protuberans (DFSP), 7 neurofibromas, and 6 leiomyomas. Using pathological results as the gold standard, diagnostic performance was compared. The number of correct diagnoses increased from 185 (47.3%) by clinical examination alone to 316 (80.8%) after the addition of HFUS (P<0.05). Meanwhile, the indeterminate diagnosis rate decreased from 143 (36.6%) to 10 (2.6%). Using HFUS, the accuracy improved significantly for epidermoid cysts (59.6%vs. 86.7%), lipomas (50.6%vs. 94.8%), pilomatrixomas (0%vs. 48.0%), haemangiomas (23.8%vs. 57.1%), and DFSPs (0%vs. 81.8%) (all p<0.05). However, HFUS did not significantly improve the diagnostic accuracy of dermatofibromas (15.8%vs. 21.1%, p>0.999), neurofibromas (42.9%vs. 71.4%, p=0.625), or leiomyomas (16.7%vs. 100%, p=0.063). Combining HFUS and clinical examination can generally improve the diagnostic accuracy and decrease the indeterminacy of invisible subcutaneous lesions, especially epidermoid cysts, lipomas, pilomatrixomas, haemangiomas, and DFSPs. However, for some rare lesions, HFUS cannot provide useful information.

Cross-species evaluation of fibroblast activation protein alpha as potential imaging target for soft tissue sarcoma: a comparative immunohistochemical study in humans, dogs, and cats.

Complete surgical tumor resection is paramount in the management of soft tissue sarcoma (STS) in humans, dogs, and cats alike. Near-infrared targeted tracers for fluorescence-guided surgery (FGS) could facilitate intraoperative visualization of the tumor and improve resection accuracy. Target identification is complicated in STS due to the rarity and heterogeneity of the disease. This study aims to validate the expression of fibroblast activation protein alpha (FAP) in selected human, canine, and feline STS subtypes to assess the value of FAP as a target for FGS and to validate companion animals as a translational model. Formalin-fixed and paraffin-embedded tissue samples from 53 canine STSs (perivascular wall tumor (PWT), canine fibrosarcoma (cFS), and STS not further specified (NOS)), 24 feline fibrosarcomas, and 39 human STSs (myxofibrosarcoma, undifferentiated pleomorphic sarcoma, dermatofibrosarcoma protuberans, and malignant peripheral nerve sheath tumor) as well as six canine and seven feline healthy controls and 10 inflamed tissue samples were immunohistochemically stained for their FAP expression. FAP labeling in tumor, peritumoral, healthy skin, and inflamed tissue samples was quantified using a visually assessed semiquantitative expression score and digital image analysis. Target selection criteria (TASC) scoring was subsequently performed as previously described. Eighty-five percent (85%) of human (33/39), 76% of canine (40/53), and 92% of feline (22/24) STSs showed FAP positivity in over 10% of the tumor cells. A high expression was determined in 53% canine (28/53), 67% feline (16/24), and 44% human STSs (17/39). The average FAP-labeled area of canine, feline, and human STSs was 31%, 33%, and 42%, respectively (p > 0.8990). The FAP-positive tumor area was larger in STS compared to healthy and peritumoral tissue samples (p < 0.0001). TASC scores were above 18 for all feline and human STS subtypes and canine PWTs but not for canine STS NOS and cFS. This study represents the first cross-species target evaluation of FAP for STS. Our results demonstrate that FAP expression is increased in various STS subtypes compared to non-cancerous tissues across species, thereby validating dogs and cats as suitable animal models. Based on a TASC score, FAP could be considered a target for FGS.

44538 A rare, under-recognized case of subcutaneous Dermatofibrosarcoma Protuberans

44538 A rare, under-recognized case of subcutaneous Dermatofibrosarcoma Protuberans

Novel role of fluorescent in situ hybridization technique (FISH) in recommended surgical margins of dermatofibrosarcoma protuberans: A preliminary study

Dermatofibrosarcoma protuberans (DFSP) is a rare skin tumor. There is no standard recommendation for its surgical management. The currently used histological analysis are HES (hematoxylin eosin saffron) staining and immunohistochemistry for CD34 expression in particular cases. Fluorescent in situ hybridization (FISH) technique is only used to qualify the DFSP as translocated or non-translocated and is not used as a diagnostic method. The aim of our study was to determine by FISH (as a diagnostic method) whether cancerous cells that could not be identified through HES staining±immunohistochemistry were present at the two-centimeter margins that were found to be tumor-free. Samples from patients who underwent surgery between 2010 and 2018 were collected. Intralesional and peripheral (at 2cm margins) paraffin slides were included. An average of 7.4 slides per specimen was analyzed. Firstly, the preselected slides were reread by a senior pathologist to confirm the absence of microscopic findings of DFSP at 2cm margins. Secondly a FISH analysis was used as a quantitative diagnostic approach, in order to find the t(17;22) translocation. Among the seven specimens that included 2cm margins, two samples presented one or more translocations, which were not visible in standard morphology assessments at two centimeters tumor-free margins. FISH analysis can have a new role in defining tumor-free margins. This would reduce the incidence of disease recurrence after resection and improve the post-operative complementary care.

Head and Neck Sarcomas Characteristics at Dr. Hasan Sadikin General Hospital for 5 Years (2017-2021)

Background: Sarcoma is a malignant neoplasm originating from mesodermal tissue with an incidence rate of 1% of neoplasms throughout the body. Head and neck sarcoma requires more complex management and until now, reviews of this disease are still very rare. This study aimed to report the incidence and characteristics of head and neck sarcoma during 2017-2021 at Dr. Hasan Sadikin General Hospital, Universitas Padjadjaran, Bandung.&#x0D; Methods: Research data was taken from patient medical records through SIRS at Dr. Hasan Sadikin General Hospital during the 2017-2021 period according to the ICD10 of head and neck malignancies and histopathological data.&#x0D; Results: During 2017-2021, 15 cases of head and neck sarcoma were found, consisting of 9 cases (60%) rhabdomyosarcoma, 2 cases (13.3%) fibrosarcoma, and 1 case each (6.7%) synovial sarcoma, undifferentiated pleomorphic sarcoma, angiosarcoma and dermatofibrosarcoma protuberans. The neck and shoulder area are the most frequently affected site (40%), followed by the scalp (26.7%), maxilla (20%), and oral cavity (13.3%). The highest incidence rates were in female (60%) and children aged &lt;17 years (46.7%).&#x0D; Conclusion: The incidence of head and neck sarcoma at Dr. Hasan Sadikin General Hospital Bandung is rare. The most frequent cases are rhabdomyosarcoma the most commonly affected areas are the neck and shoulders.