scholarly journals Rapidly fatal SMARCA4-deficient undifferentiated sarcoma originating from hybrid hemosiderotic fibrolipomatous tumor/pleomorphic hyalinizing angiectatic tumor of the foot

2021 ◽  
Author(s):  
Abbas Agaimy ◽  
Norbert Meidenbauer ◽  
William R. Sukov ◽  
Robert Stoehr ◽  
Michael Vieth ◽  
...  
Keyword(s):  
Systemic Chemotherapy ◽  
Molecular Testing ◽  
Low Grade ◽  
Close Link ◽  
Undifferentiated Sarcoma ◽  
Multiple Metastases ◽  
Pleomorphic Hyalinizing Angiectatic Tumor ◽  
Rhabdoid Features ◽  
Myxoinflammatory Fibroblastic Sarcoma ◽  
Rapid Regrowth

AbstractPleomorphic hyalinizing angiectatic tumor (PHAT) of soft parts and hemosiderotic fibrolipomatous tumor (HFLT) are two rare low-grade locally recurring neoplasms with predilection for the foot/ankle. Recent studies support a close link between the two entities, and origin of PHAT from HFLT and occurrence of hybrid HFLT/PHAT have been documented. Both lesions often harbor TGFBR3 or MGEA5 rearrangements. Rare sarcomas originating from HFLT/PHAT have been reported, typically resembling myxofibrosarcoma or myxoinflammatory fibroblastic sarcoma. We describe a novel SMARCA4-deficient undifferentiated sarcoma with rhabdoid features originating from hybrid HFLT/PHAT in the foot of a 54-year-old male. The tumor pursued a highly aggressive course with rapid regrowth after resection and multiple metastases resulting in patient’s death within 5 months, despite systemic chemotherapy. Immunohistochemistry revealed SMARCA4 loss in the undifferentiated sarcoma, but not in the HFLT/PHAT. Molecular testing confirmed TGFBR3/MGEA5 rearrangements. This report expands the phenotypes of sarcomas developing from pre-existing PHAT/HFLT.

Cytologic and Histologic Features of Pleomorphic Undifferentiated Sarcoma Arising in a Hybrid Hemosiderotic Fibrolipomatous Tumor and Pleomorphic Hyalinizing Angiectatic Tumor: Report of an Unusual Case with a Literature Review

2015 ◽  
Vol 59 (6) ◽  
pp. 493-497 ◽  
Author(s):  
Elizabeth Morency ◽  
William Laskin ◽  
Xiaoqi Lin
Keyword(s):  
Unusual Case ◽  
Surgical Excision ◽  
Excisional Biopsy ◽  
Metastatic Potential ◽  
Low Grade ◽  
Undifferentiated Sarcoma ◽  
High Grade Sarcoma ◽  
Pleomorphic Hyalinizing Angiectatic Tumor ◽  
Mitotic Figures ◽  
Vacuolated Cytoplasm

Background: Pleomorphic hyalinizing angiectatic tumor (PHAT) and hemosiderotic fibrolipomatous tumor (HFLT) are low-grade neoplasms that share clinicopathologic features and recurring translocation t(1;10)(p22;q24) involving the TGFBR3 and MGEA5 genes. Coexistence of these tumors with a high-grade sarcoma is exceedingly rare and the cytologic features have not been widely described in the literature. Case: A 55-year-old female presented with a soft tissue tumor on the dorsum of the foot. Cytologic smears and corresponding core biopsies were composed of a population of markedly pleomorphic spindle cells seen singly and in loose clusters within a myxofibrous matrix and infiltrating fat, with coarse chromatin, prominent nucleoli, irregular nuclear contours and delicate to vacuolated cytoplasm. Intracytoplasmic hemosiderin granules and rare intranuclear cytoplasmic pseudoinclusions were identified. The histologic features of the excisional biopsy mirrored those of the cytologic preparations, but also demonstrated cellular foci of higher-grade sarcoma composed of markedly pleomorphic tumor cells with large vesicular nuclei and prominent nucleoli, exhibiting a mitotic index of 12 mitotic figures per 10 high-powered fields. Conclusion: While HFLT/PHAT generally can be managed by wide local excision, it is important to be aware of their capacity to harbor higher-grade lesions with metastatic potential which may require more radical surgical excision.

scholarly journals The t(1;10)(p22;q24) TGFBR3/MGEA5 Translocation in Pleomorphic Hyalinizing Angiectatic Tumor, Myxoinflammatory Fibroblastic Sarcoma, and Hemosiderotic Fibrolipomatous Tumor

2018 ◽  
Vol 143 (2) ◽  
pp. 212-221 ◽  
Author(s):  
Huifei Liu ◽  
William R. Sukov ◽  
Jae Y. Ro
Keyword(s):  
Transforming Growth Factor ◽  
Transforming Growth Factor Β ◽  
Low Grade ◽  
Pathologic Features ◽  
Mesenchymal Neoplasm ◽  
Recurrent Translocation ◽  
Pleomorphic Hyalinizing Angiectatic Tumor ◽  
Controversial Topic ◽  
Β Receptor ◽  
Myxoinflammatory Fibroblastic Sarcoma

Context.— Pleomorphic hyalinizing angiectatic tumor (PHAT) of soft parts, hemosiderotic fibrolipomatous tumor (HFLT), and myxoinflammatory fibroblastic sarcoma (MIFS) are 3 distinct entities of low-grade spindle cell mesenchymal neoplasm. These tumors have similar clinical presentations and partially overlapping but distinctive pathologic features. A recurrent translocation, t(1;10)(p22;q24), has been detected in a subset of PHAT, HFLT, MIFS, and HFLT/MIFS hybrid cases. Translocation t(1;10)(p22;q24) involves transforming growth factor β-receptor 3 (TGFBR3) and meningioma-expressed antigen 5 (MGEA5) genes on chromosomes 1p22 and 10q24, respectively. However, the percentage of translocation in PHAT, HFLT, and MIFS varies significantly among different studies. The relationship among these tumors has been a controversial topic among experts. Objective.— To discuss the diagnostic and functional significance of translocation t(1;10)(p22;q24) TGFBR3/MGEA5 rearrangement in HFLT, PHAT, and MIFS. Data Sources.— PubMed was used for this study. Conclusions.— Diagnosis of HFLT, PHAT, and MIFS is challenging because of a lack of unique morphologic, immunophenotypic, molecular, and cytogenetic markers. The recurrent t(1;10)(p22;q24) translocation and/or TGFBR3/MGEA5 rearrangement was reported in 55 patients, with a relatively even distribution among HFLT, PHAT, and MIFS (17 HFLT, 15 MIFS, 13 MIFS/HFLT, and 10 PHAT). This indicates that current morphology-based diagnostic criteria do not identify reliably the subset of soft tissue tumor with t(1;10) translocation. Genetic heterogeneity of these tumors is supported by the recent detection of a mutually exclusive, second recurrent genetic change, t(7;17) TOM1L2-BRAF translocation or BRAF amplification, in a subset of MIFS.

scholarly journals Clinical Utility of Real-Time Targeted Molecular Profiling in the Clinical Management of Ovarian Cancer: The ALLOCATE Study

2019 ◽  
pp. 1-18
Author(s):  
Olga Kondrashova ◽  
Gwo-Yaw Ho ◽  
George Au-Yeung ◽  
Leakhena Leas ◽  
Tiffany Boughtwood ◽  
...  
Keyword(s):  
Ovarian Cancer ◽  
Real Time ◽  
Targeted Therapies ◽  
Clinical Management ◽  
Clinical Utility ◽  
Advanced Ovarian Cancer ◽  
Molecular Profiling ◽  
Molecular Testing ◽  
Low Grade ◽  
Ovarian Carcinomas

PURPOSE The ALLOCATE study was designed as a pilot to demonstrate the feasibility and clinical utility of real-time targeted molecular profiling of patients with recurrent or advanced ovarian cancer for identification of potential targeted therapies. PATIENTS AND METHODS A total of 113 patients with ovarian cancer of varying histologies were recruited from two tertiary hospitals, with 99 patient cases suitable for prospective analysis. Targeted molecular and methylation profiling of fresh biopsy and archived tumor samples were performed by screening for mutations or copy-number variations in 44 genes and for promoter methylation of BRCA1 and RAD51C. RESULTS Somatic genomic or methylation events were identified in 85% of all patient cases, with potentially actionable events with defined targeted therapies (including four resistance events) detected in 60% of all patient cases. On the basis of these findings, six patients received molecularly guided therapy, three patients had unsuspected germline cancer–associated BRCA1/ 2 mutations and were referred for genetic counseling, and two intermediate differentiated (grade 2) serous ovarian carcinomas were reclassified as low grade, leading to changes in clinical management. Additionally, secondary reversion mutations in BRCA1/ 2 were identified in fresh biopsy samples of two patients, consistent with clinical platinum/poly (ADP-ribose) polymerase inhibitor resistance. Timely reporting of results if molecular testing is done at disease recurrence, as well as early referral for patients with platinum-resistant cancers, were identified as factors that could improve the clinical utility of molecular profiling. CONCLUSION ALLOCATE molecular profiling identified known genomic and methylation alterations of the different ovarian cancer subtypes and was deemed feasible and useful in routine clinical practice. Better patient selection and access to a wider range of targeted therapies or clinical trials will further enhance the clinical utility of molecular profiling.

scholarly journals P1.06-019 The Possibility of the Additional Local Therapy to Systemic Chemotherapy in Advanced Lung Cancer Cases with Multiple Metastases

2017 ◽  
Vol 12 (1) ◽  
pp. S676-S677
Author(s):  
Takeshi Honda ◽  
Hirofumi Uehara ◽  
Maika Natsume ◽  
Yoko Fukasawa ◽  
Takahiko Sakamoto ◽  
...  
Keyword(s):  
Lung Cancer ◽  
Systemic Chemotherapy ◽  
Local Therapy ◽  
Advanced Lung Cancer ◽  
Multiple Metastases

Retrospective study of non-mucinous appendiceal adenocarcinomas: Role of systemic chemotherapy and cytoreductive surgery.

2016 ◽  
Vol 34 (4_suppl) ◽  
pp. 263-263
Author(s):  
Marc Isamu Uemura ◽  
Wei Qiao ◽  
Keith F. Fournier ◽  
Jeffrey Morris ◽  
Paul F. Mansfield ◽  
...  
Keyword(s):  
Cytoreductive Surgery ◽  
Systemic Chemotherapy ◽  
Stage Iv ◽  
Stage Ii ◽  
Stage Iii ◽  
Rank Test ◽  
Low Grade ◽  
Histologic Subtype ◽  
Well Differentiated ◽  
Quantitative Response

263 Background: The majority of studies evaluating appendiceal epithelial neoplasms have focused on those with mucinous histology. Few studies have reported on non-mucinous appendiceal adenocarcinomas. We performed the largest single-center study to investigate this histologic subtype, in order to describe the natural history and impact of both cytoreductive surgery (CRS) and systemic chemotherapy. Methods: We retrospectively reviewed 172 pts with non-mucinous appendiceal adenocarcinoma evaluated at the UT-MD Anderson Cancer Center between 1990 and 2015. Patient demographics, tumor characteristics, therapy received, and outcomes were recorded. Response assessment was semi-quantitative (response vs. no response) according to the treating physician. Overall survival (OS) and time to progression (TTP) were calculated using Kaplan Meier product-limit method and survival rates compared using the log rank test. Results: Median age at diagnosis was 52.9 yrs (M:F 1:1). Most pts presented with advanced stage: stage I (1.7%), stage II (32.5%), stage III (14.5%), and stage IV (51.2%). No patient had well-differentiated histology. 56% had moderate and 44% poor histology. Median OS by stage was 90.9m [95% CI: 70.8 to 172.9] for stage II, 52.1m [95% CI: 28.9 to NA] for stage III and 28.3m [95% CI: 22.9 to 31.9] for stage IV, (p < 0.0001). In pts with metastatic disease (n = 128) CRS was attempted in 20 (15.6%) and was complete (CCS 0/1) in 12. The median OS for pts achieving complete CRS was 48.6m. Systemic chemotherapy was administered to 92% (118/128) of metastatic pts. The median TTP was 9.4m [95% CI: 8.0 to 11.5] and semi-quantitative response rate was 54%. The majority of pts received either oxaliplatin-based, 57%, or irinotecan-based, 23%, first-line chemotherapy regimens. No statistical difference in TTP (p = 0.9) or OS (p = 0.07) between different chemotherapies was seen. Conclusions: In contrast to mucinous appendiceal neoplasms, non-mucinous appendiceal adenocarcinomas rarely present with low-grade (well-differentiated) histology. Treatment approaches appear more akin to colorectal cancer with most metastatic pts undergoing systemic chemotherapy and a minority undergoing CRS.

Are patients with oligodendroglioma at higher risk for radiation neurotoxicity?

2019 ◽  
Vol 37 (15_suppl) ◽  
pp. 2061-2061
Author(s):  
Haroon Ahmad ◽  
Sohil H. Patel ◽  
Joseph Donahue ◽  
M. Beatriz Lopes ◽  
Benjamin Purow ◽  
...  
Keyword(s):  
Tumor Progression ◽  
Standard Of Care ◽  
Fold Increase ◽  
Patient Characteristics ◽  
High Dose ◽  
Molecular Testing ◽  
Low Grade ◽  
Who Grade ◽  
Signal Abnormality ◽  
Anaplastic Gliomas

2061 Background: Symptomatic radiation neurotoxicity (RN), manifesting on MRI as focal necrosis and/or T2 signal abnormality, is a dreaded complication of radiation therapy (RT). While RT is standard of care for anaplastic gliomas, the long-term benefit vs risk profile in low-grade gliomas is not well defined. Patients with oligodendroglioma carry a better overall survival than those with astrocytoma. Anecdotally, they are more prone to experience RN than astrocytomas, as suggested by Acharya et al in 2017. We hypothesized that, independent of grade, oligodendrogliomas have a higher incidence of RN as compared to astrocytomas. Methods: We reviewed the records of 628 patients with WHO grade II and III gliomas from our institution. Study population comprised 326 patients with: standard fractionated RT, pathology confirmation by a neuropathologist, and follow up of at least 2 years after diagnosis. RN was defined as either histologically confirmed by pathology or requiring intervention for clinically presumed RN (bevacizumab or high-dose steroids.) A separate category included patients with dramatic cognitive decline with increased T2 signal abnormality, in the absence or tumor progression. Results: There were 131 patients with oligodendroglioma, based upon 1p/19q co-deletion (105 cases) or histology in the absence of molecular testing (26 cases). The remaining 195 patients had astrocytoma with intact 1p/19q, isocitrate dehydrogenase (IDH) wild-type, or diagnosed histologically absent molecular testing. The incidence of RN were 18.3% and 8.2% for oligodendroglioma and astrocytoma, respectively (p = 0.0063). An additional four patients with oligodendroglioma and two with astrocytoma had significant cognitive deterioration with increased T2 signal abnormality, without tumor progression. Conclusions: The greater than two-fold increase in RN incidence for oligodendrogliomas is significant and suggests patients with oligodendrogliomas may be more at risk to develop RN. Therefore, in patients with oligodendroglioma, the consideration of fractionated RT needs to be weighed against the increased potential for RN. Analysis of baseline imaging and patient characteristics variables that correlate with development of RN are ongoing and will be presented at the meeting.

scholarly journals Uterine sarcoma: a clinical case and a literature review

2019 ◽  
Vol 25 (4) ◽  
pp. 206-218
Author(s):  
Diana Bužinskienė ◽  
Saulius Mikėnas ◽  
Gražina Drąsutienė ◽  
Matas Mongirdas
Keyword(s):  
Clinical Case ◽  
Uterine Sarcoma ◽  
Imaging Modalities ◽  
Histological Evaluation ◽  
Low Grade ◽  
Undifferentiated Sarcoma ◽  
Uterine Sarcomas ◽  
Uterine Tumour ◽  
Presenting Symptoms ◽  
Power Morcellation

Background. Uterine sarcomas are rare gynaecologic tumours representing 3–7% of all uterine malignancies. The aetiology of sarcomas is still unclear: it is thought, that chromosomal translocations have influence on wide histological variety of sarcomas. Presenting symptoms are vague and nonspecific. Usually sarcoma causes abnormal vaginal bleeding, can cause abdominal or pelvic pain, or manifests as a rapidly growing uterine tumour. The diagnosis of sarcoma is often made retrospectively after surgical removal of a presumed benign uterine neoplasm, because imaging modalities such as ultrasound, computed tomography, or magnetic resonance imaging cannot yet accurately and reliably distinguish between benign leiomyoma and malignant pathology. If there are certain clinical features that raise a suspicion of malignancy in the uterus, it is recommended to avoid the use of power morcellation through laparoscopic surgery in order to prevent disease dissemination. Materials and methods. We present a clinical case of a 64-year-old patient, who was referred to hospital due to abdominal pain and tenesmus that lasted for two days. From a past medical history it was known that previously the patient had been diagnosed with uterine myoma. Transvaginal ultrasonography showed a 10.4 cm × 9.8 cm uterine tumour of nonhomogeneous structure with signs of necrosis and good vascularization. The patient refused urgent hysterectomy, that was advised to her. The patient was operated on one month later and total hysterectomy with bilateral salpingooforectomy was performed. Postoperative histological evaluation showed undifferentiated sarcoma uterus pT1b L/V0. Imaging modalities were made to evaluate possible dissemination of the disease. In the absence of signs of disease progression, the patient received radiotherapy and brachytherapy and was followed-up by doctors. Results and conclusions. Uterine sarcomas are highly malignant tumours that originate from smooth muscles and connective tissue elements of the uterus and make up 1% of all malignant gynaecological tumours and about 3–7% of all malignant uterine tumours. Imaging modalities cannot yet reliably distinguish benign myomas from malignant sarcomas. It is important not to damage the wholeness of uterus during operation in order to prevent dissemination of the disease in the abdominal cavity. The low-grade endometrial stromal sarcoma has the best survival prognosis, while carcinosarcoma and undifferentiated uterine sarcoma have the lowest survival rates.

scholarly journals Immunophenotype of Pediatric NTRK fusion mesenchymal tumors

2020 ◽  
Author(s):  
Jeongwan Kang ◽  
Jin Woo Park ◽  
Jae-Kyung Won ◽  
Jeong Mo Bae ◽  
Jaemoon Koh ◽  
...  
Keyword(s):  
S100 Protein ◽  
Protein A ◽  
Mitotic Rate ◽  
University Hospital ◽  
Low Grade ◽  
Fusion Partner ◽  
Pediatric Tumors ◽  
Mesenchymal Tumors ◽  
Undifferentiated Sarcoma ◽  
Infantile Fibrosarcoma

Abstract Background: While ETV6-NTRK3 fusion is common in infantile fibrosarcoma, NTRK1/3 fusion in pediatric tumors is scarce and, consequently, not well known. Herein, we evaluated NTRK1/3 fusion pediatric mesenchymal tumors clinicopathologically and immunophenotypically. Methods: We reviewed nine NTRK -fusion pediatric sarcomas confirmed by fluorescence in situ hybridization and/or next-generation sequencing from Seoul National University Hospital between 2002 and 2020. Results: One case of TPR - NTRK1 fusion-positive intracranial extra-axial high-grade undifferentiated sarcoma (12-year-old boy), one case of LMNA-NTRK1 fusion-positive low-grade infantile fibrosarcoma of the forehead (3-year-old boy), one case of ETV6-NTRK3 fusion-positive inflammatory myofibroblastic tumor (IMT) (3-months-old girl), and six cases of ETV6-NTRK3 fusion-positive infantile fibrosarcoma (median age: 2.6 months, range: 1.6–5.6 months, M: F = 5:1) were reviewed. The Trk immunopositive pattern was distinctive according to the fusion genes. We notified nuclear positivity in TPR-NTRK1 fusion sarcoma, nuclear membrane positivity in LMNA-NTRK1 fusion sarcoma, and both cytoplasmic and nuclear positivity in ETV6-NTRK3 fusion-positive IMT and infantile fibrosarcomas. Also, the TPR-NTRK1 fusion sarcoma showed robust positivity for CD34/nestin and high mitoses. The LMNA-NTRK1 fusion sarcoma revealed CD34/S100 protein/nestin/CD10 coexpression, and a low mitotic rate. The IMT with ETV6-NTRK3 fusion expressed SMA. Six infantile fibrosarcomas with ETV6-NTRK3 fusion showed variable coexpression of nestin (6/6)/CD10 (4/5)/ S100 protein (3/6). Conclusions: All cases of NTRK1 and NTRK3 fusion-positive pediatric tumors robustly expressed the Trk protein. A Trk immunopositive pattern and CD34/S100/nestin/CD10/SMA immunohistochemical phenotype may suggest NTRK fusion partner genes and diagnoses. LMNA-NTRK1 fusion sarcoma might be a low-grade subtype of infantile fibrosarcoma. Interestingly, more than half of the infantile fibrosarcoma cases were positive for S100 protein and CD10. The follow-up period of TPR-NTRK1 and LMNA-NTRK1 fusion tumors are not enough to predict prognosis. However, ETV6-NTRK3 fusion infantile fibrosarcomas showed an excellent prognosis with no evidence of disease for an average of 11.7 years after gross total resection of the tumor.

scholarly journals Clinicopathological findings of pediatric NTRK fusion mesenchymal tumors

2020 ◽  
Vol 15 (1) ◽  
Author(s):  
Jeongwan Kang ◽  
Jin Woo Park ◽  
Jae-Kyung Won ◽  
Jeong Mo Bae ◽  
Jaemoon Koh ◽  
...  
Keyword(s):  
S100 Protein ◽  
Mitotic Rate ◽  
University Hospital ◽  
Low Grade ◽  
Fusion Partner ◽  
Pediatric Tumors ◽  
Mesenchymal Tumors ◽  
Undifferentiated Sarcoma ◽  
Infantile Fibrosarcoma ◽  
Clinicopathological Findings

Abstract Background While ETV6- NTRK3 fusion is common in infantile fibrosarcoma, NTRK1/3 fusion in pediatric tumors is scarce and, consequently, not well known. Herein, we evaluated for the presence of NTRK1/3 fusion in pediatric mesenchymal tumors, clinicopathologically and immunophenotypically. Methods We reviewed nine NTRK fusion-positive pediatric sarcomas confirmed by fluorescence in situ hybridization and/or next-generation sequencing from Seoul National University Hospital between 2002 and 2020. Results One case of TPR-NTRK1 fusion-positive intracranial, extra-axial, high-grade undifferentiated sarcoma (12-year-old boy), one case of LMNA-NTRK1 fusion-positive low-grade infantile fibrosarcoma of the forehead (3-year-old boy), one case of ETV6-NTRK3 fusion-positive inflammatory myofibroblastic tumor (IMT) (3-months-old girl), and six cases of ETV6-NTRK3 fusion-positive infantile fibrosarcoma (median age: 2.6 months, range: 1.6–5.6 months, M: F = 5:1) were reviewed. The Trk immunopositivity patterns were distinct, depending on what fusion genes were present. We observed nuclear positivity in TPR-NTRK1 fusion-positive sarcoma, nuclear membrane positivity in LMNA-NTRK1 fusion-positive sarcoma, and both cytoplasmic and nuclear positivity in ETV6-NTRK3 fusion-positive IMT and infantile fibrosarcomas. Also, the TPR-NTRK1 fusion-positive sarcoma showed robust positivity for CD34/nestin, and also showed high mitotic rate. The LMNA-NTRK1 fusion-positive sarcoma revealed CD34/S100 protein/nestin/CD10 coexpression, and a low mitotic rate. The IMT with ETV6-NTRK3 fusion expressed SMA. Six infantile fibrosarcomas with ETV6-NTRK3 fusion showed variable coexpression of nestin (6/6)/CD10 (4/5)/ S100 protein (3/6). Conclusions All cases of NTRK1 and NTRK3 fusion-positive pediatric tumors robustly expressed the Trk protein. A Trk immunopositive pattern and CD34/S100/nestin/CD10/SMA immunohistochemical expression may suggest the presence of NTRK fusion partner genes. LMNA-NTRK1 fusion sarcoma might be a low-grade subtype of infantile fibrosarcoma. Interestingly, more than half of the infantile fibrosarcoma cases were positive for S100 protein and CD10. The follow-up period of TPR-NTRK1 and LMNA-NTRK1 fusion-positive tumors are not enough to predict prognosis. However, ETV6-NTRK3 fusion-positive infantile fibrosarcomas showed an excellent prognosis with no evidence of disease for an average of 11.7 years, after gross total resection of the tumor.

Sarcoma Fibroblástico Mixoinflamatório: Relato de Caso/Myxoinflammatory Fibroblastic Sarcoma: Case Report

1970 ◽  
Vol 5 (2) ◽  
pp. 139-147
Author(s):  
Paula Boson Trotta ◽  
Karen Guimarães Rosa ◽  
Luciano Martins Alves da Rosa Martins Alves da Rosa
Keyword(s):  
Case Report ◽  
Subcutaneous Tissue ◽  
Tumor Resection ◽  
Distal Region ◽  
Distal Phalanx ◽  
Low Grade ◽  
Wound Area ◽  
The Right ◽  
Specific Symptoms ◽  
Myxoinflammatory Fibroblastic Sarcoma

RESUMOIntrodução: O Sarcoma Fibroblástico Mixoinflamatório (SFMI) é um tumor de partes moles de baixo grau, raro, que apresenta predileção equivalente em ambos os gêneros, sem sofrer influência de fatores genéticos ou ambientais. Apresenta sintomas inespecíficos, o que dificulta o diagnóstico. A dor está presente no diagnóstico em cerca de 17% dos pacientes. Outro fator que atrapalha o diagnóstico está na raridade do tumor e inabilidade técnica de detectá-lo. Casuística: Apresenta-se um caso de um paciente do sexo masculino, de 47 anos encaminhado ao ortopedista com tumoração em região distal do segundo quirodáctilo da mão direita. Após cirurgia de ressecção tumoral foi detectado um tecido que se originava na falange distal e tinha coloração amarelada e consistência densa, mesclado com o subcutâneo. Observou-se isquemia transitória na polpa digital da falange distal ainda durante a cirurgia. Posterior exame imuno-histoquímico revelou tratar-se de um Sarcoma Fibroblástico Mixoinflamatório. Após a cirurgia, o paciente retorna com necrose da polpa digital submetida a cirurgia. Foi realizada limpeza e debridamento do tecido necrótico. O paciente evoluiu bem com total epitelização da área cruenta. Conclusão: Foi concluído tratar-se de um sarcoma de difícil diagnóstico, porém de baixa metastatização e bom prognostico.Palavras-chave: Sarcoma, Isquemia, Necrose. ABSTRACTIntroduction: Myxoinflammatory Fibroblastic Sarcoma is a low-grade, rare tumor of soft parts that presents equivalent predilection for both genders  suffering no influence of genetic or enviormental factors. It has non-specific symptoms, making it difficult to diagnose. Pain appears in the diagnosis only in 17% of patients. Another factor that hinders the diagnosis is in the fewness numbers of patients that have it, and the technical inability to detect it. Case Report: This case report describes the case of a male patient, 47 years old, that referred to the orthopedist a tumor in the distal region of the second finger of the right hand. After tumor resection it was detected a tissue that originated in the distal phalanx and had a yellowish color and dense consistency, mixed with the subcutaneous tissue. It was observed transient ischemia in the digital pulp of the distal phalanx during surgery. Subsequent immunohistochemical examination revealed that it was Myxoinflammatory Fibroblastic Sarcoma. After the surgery, the patient returned with necrosis of the fingertip. Cleaning and debridement of necrotic tissue was performed. The patient developed a complete epithelialization of the wound area. Conclusion: The conclusion is that this is a sarcoma which is difficult to diagnose, but with low metastasis and good prognosis.Keywords: Sarcoma, Ischemia, Necrosis.

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