scholarly journals Rare giant dermatofibrosarcoma protuberans with uncommon fibrosarcomatous transformation - case report and review of literature

2021 ◽  
Vol 8 (10) ◽  
pp. 3171
Author(s):  
Manisha Aggarwal ◽  
Dinesh Manchikanti ◽  
Sunayana Misra ◽  
Shaji Thomas ◽  
Ashish Arsia ◽  
...  
Keyword(s):  
Clinical Course ◽  
Dermatofibrosarcoma Protuberans ◽  
Distant Metastases ◽  
Metastatic Potential ◽  
Recurrence Risk ◽  
Skin Tumor ◽  
Rapid Progression ◽  
Split Skin Grafting ◽  
Aggressive Clinical Course ◽  
Slow Growing

Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue sarcoma with aggressive local behavior but with a low metastatic potential. Although slow growing and indolent, they rarely reach huge sizes. Very uncommonly, these locally invasive tumors undergo fibrosarcomatous transformation with a more aggressive clinical course, with higher rate of recurrence risk and distant metastases. A 32-years-old lady, presented with a gradually progressive lump in the upper central back for the past 6 years, with rapid progression in size during the last 6 months. On examination, she had a single lump of size 18×18 cm in the midline of the upper back, with prominent veins over its surface. Magnetic resonance imaging (MRI) showed no connection with the spinal canal and appeared flush with the paraspinal muscles. Core needle biopsy showed DFSP. The patient underwent a wide local excision with split skin grafting. The histopathology now showed a fibrosarcomatous transformation of DFSP. The patient again underwent a wide re-excision with a 3 cm margin. Histology reported no evidence of tumor cells in the specimen. The patient’s postoperative period was uneventful and she was referred for adjuvant radiotherapy. DFSP is a rare, slow-growing malignant fibroblastic mesenchymal skin tumor with low metastatic potential. However, in any patient with long standing DFSP with a recent increase in size, this fibrosarcomatous transformation must be kept in mind as it represents an uncommon form of DFSP that tends to follow a more aggressive clinical course, with higher rate of recurrence risk and distant metastases.

scholarly journals Spontaneous Remission in an Older Patient with RelapsedFLT3ITD Mutant AML

2016 ◽  
Vol 2016 ◽  
pp. 1-7 ◽  
Author(s):  
Pankit Vachhani ◽  
Jason H. Mendler ◽  
Andrew Evans ◽  
George Deeb ◽  
Petr Starostik ◽  
...  
Keyword(s):  
Myeloid Leukemia ◽  
Tandem Duplication ◽  
Clinical Course ◽  
Spontaneous Remission ◽  
Rapid Progression ◽  
Older Patient ◽  
Internal Tandem Duplication ◽  
First Case ◽  
Short Overall Survival ◽  
Aggressive Clinical Course

Spontaneous remission (SR) of acute myeloid leukemia (AML) is a very rare phenomenon. AML characterized byFLT3internal tandem duplication (FLT3ITD) is typically associated with an aggressive clinical course with rapid progression, relapse, and short overall survival in the absence of transplantation. We report here the first case of SR ofFLT3ITD mutant AML in the literature. Our patient was an elderly woman with relapsedNPM1andFLT3ITD mutant AML whose disease underwent SR for a brief duration without precipitating cause. We review the potential immune mechanisms underlying SR in AML and discuss the implications for novel immunotherapeutic approaches forFLT3mutant AML.

scholarly journals Pancreatic metastasis of dermatofibrosarcoma protuberans: a rare case

2020 ◽  
Vol 13 (1) ◽  
pp. e232614 ◽  
Author(s):  
Durga Sowgandhi Chilukuri ◽  
Prithviraj Premkumar ◽  
Balasubramanian Venkitaraman ◽  
Jagadesh Chandra Bose Soundararajan
Keyword(s):  
Case Report ◽  
High Risk ◽  
Soft Tissue ◽  
Local Recurrence ◽  
Rare Case ◽  
Dermatofibrosarcoma Protuberans ◽  
Metastatic Potential ◽  
Pancreatic Metastasis ◽  
Solitary Metastasis ◽  
Slow Growing

Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue malignancy which is locally aggressive, slow growing. It has a very low metastatic potential and has high risk of local recurrence. We report a 65-year-old man with recurrent DFSP of thigh with pancreatic metastasis. Apart from our patient, only four other cases of pancreatic metastasis of DFSP have been reported. Our patient had a solitary metastasis to pancreas and was treated with distal pancreaticosplenectomy. Outcome of the patient was good. We present this case report to emphasise that resection may be considered for solitary metastasis of DFSP and can be managed successfully.

DIRECT RESULTS OF SURGICAL TREATMENT OF THREE-NEGATIVE BREAST CANCER

2020 ◽  
pp. 59-62
Author(s):  
Anna Vladimirovna Baranova
Keyword(s):  
Breast Cancer ◽  
Surgical Treatment ◽  
Lymph Nodes ◽  
Clinical Course ◽  
Radical Mastectomy ◽  
Distant Metastases ◽  
Wound Complications ◽  
Post Surgery ◽  
Aggressive Clinical Course ◽  
Direct Results

Three−negative breast cancer is characterized by aggressive clinical course, early metastasis and poor prognosis, leading to more active oncosurgical tactics and systemic treatment. Due to the aggressive clinical course of this oncopathology with increasing risk of locoregional and distant metastases, the most common surgical treatment tactics are modified variants of radical mastectomy with wide dissection of lymph nodes in areas of potential metastasis. Radicalism of the operation is the cause of the increase in the incidence of wound and lymphoenosis, the development of which depends on many factors, the determination of which is important for the development of methods of prevention of complications. In recent years, in the early stages of the disease, a radical breast resection after a course of adjuvant radiotherapy has been considered as an alternative. To study the immediate post−surgery results of three−negative breast cancer, depending on the initial clinical and pathological parameters and features of therapeutic tactics, a study was conducted in which 66 patients participated. Patients underwent radical mastectomy and breast resection, as well as lymph node dissection. Nine patients underwent aloplastic reconstruction. The results of the analysis showed that most often after radical surgeries, irrespective of their volume, there are lymphovenous complications, the development of which is affected by an increase in body mass index and lesions of lymph nodes. Aloplastic breast reconstruction contributes to a reduced incidence of lymphoid complications, and neoadjuvant chemotherapy is associated with an increased incidence of wound complications. These factors should be considered when planning the surgery protocols of patients with three negative breast cancer. Key words: three−negative breast cancer, surgical treatment, postoperative complications, risk factors.

Sclerosing Epithelioid Fibrosarcoma of the Kidney: First Reported Case in a Young Child

2021 ◽  
pp. 109352662097773
Author(s):  
Justin L Kurtz ◽  
Serena Y Tan ◽  
Florette K Hazard
Keyword(s):  
Clinical Course ◽  
Distant Metastases ◽  
Renal Tumors ◽  
Low Grade ◽  
Visceral Organ ◽  
Deep Soft Tissue ◽  
Sclerosing Epithelioid Fibrosarcoma ◽  
Patient Reported ◽  
Aggressive Clinical Course ◽  
Middle Aged Adults

Sclerosing epithelioid fibrosarcoma (SEF) is a rare variant of fibrosarcoma primarily arising in the deep soft tissue of the extremities and trunk. Despite having the morphologic appearance of a low-grade sarcoma, it generally has an aggressive clinical course with frequent local recurrences and distant metastases. It typically occurs in middle aged adults and is characterized by immunoexpression of MUC4 and recurrent gene fusions, most commonly EWSR1-CREB3L1. We report a primary renal SEF in a 4-year-old male. To our knowledge, this is the youngest patient reported with SEF and the second case of SEF in a pre-adolescent child. It is the eleventh reported case of primary renal SEF in the literature. While SEF arising in visceral organs is rare, the kidney is the most common primary site of any visceral organ. This case demonstrates SEF can occur in pre-adolescents, is an important consideration when evaluating sarcomas in young children, and should be considered in the differential diagnosis for primary renal tumors.

scholarly journals Current experimental disease-modifying therapeutics for multiple system atrophy

2021 ◽  
Author(s):  
Miguel Lemos ◽  
Gregor K. Wenning ◽  
Nadia Stefanova
Keyword(s):  
Multiple System Atrophy ◽  
Clinical Presentation ◽  
Clinical Course ◽  
Neurodegenerative Disorder ◽  
Rapid Progression ◽  
Cytoplasmic Inclusions ◽  
Disease Modifying ◽  
Aggressive Clinical Course ◽  
Preclinical And Clinical Studies ◽  
Variable Clinical Presentation

AbstractMultiple system atrophy (MSA) is a challenging neurodegenerative disorder with a difficult and often inaccurate early diagnosis, still lacking effective treatment. It is characterized by a highly variable clinical presentation with parkinsonism, cerebellar ataxia, autonomic dysfunction, and pyramidal signs, with a rapid progression and an aggressive clinical course. The definite MSA diagnosis is only possible post-mortem, when the presence of distinctive oligodendroglial cytoplasmic inclusions (GCIs), mainly composed of misfolded and aggregated α-Synuclein (α-Syn) is demonstrated. The process of α-Syn accumulation and aggregation within oligodendrocytes is accepted one of the main pathological events underlying MSA. However, MSA is considered a multifactorial disorder with multiple pathogenic events acting together including neuroinflammation, oxidative stress, and disrupted neurotrophic support, among others. The discussed here treatment approaches are based on our current understanding of the pathogenesis of MSA and the results of preclinical and clinical therapeutic studies conducted over the last 2 decades. We summarize leading disease-modifying approaches for MSA including targeting α-Syn pathology, modulation of neuroinflammation, and enhancement of neuroprotection. In conclusion, we outline some challenges related to the need to overcome the gap in translation between preclinical and clinical studies towards a successful disease modification in MSA.

Insular carcinoma of thyroid presenting as cervical cord compression

2000 ◽  
Vol 114 (10) ◽  
pp. 808-810 ◽  
Author(s):  
I. Ganly ◽  
J. Crowther
Keyword(s):  
Thyroid Carcinoma ◽  
Clinical Course ◽  
Spinal Metastases ◽  
Metastatic Potential ◽  
Cord Compression ◽  
Cervical Cord ◽  
Advanced Stage ◽  
Cervical Cord Compression ◽  
Aggressive Clinical Course ◽  
Insular Carcinoma

Insular carcinoma of thyroid is a very rare subtype of thyroid carcinoma which is characterized by its advanced stage at presentation and aggressive clinical course. We describe a case of insular carcinoma of the thyroid presenting in an unusual fashion with cervical cord compression at initial presentation. Cord compression is shown to be due to spinal metastases from vascular spread of the tumour and this typifies the high metastatic potential of this tumour subtype.

scholarly journals Dermatofibrosarcoma Protuberans of the Scalp with Fibrosarcomatous Degeneration and Pulmonary Metastasis

2011 ◽  
Vol 1 ◽  
pp. 55 ◽  
Author(s):  
Joseph L Gatlin ◽  
Richard Hosch ◽  
Majid Khan
Keyword(s):  
Head And Neck ◽  
Lung Metastasis ◽  
Pulmonary Metastasis ◽  
Dermatofibrosarcoma Protuberans ◽  
Distant Metastases ◽  
Cutaneous Tumor ◽  
Slow Growing

Dermatofibrosarcoma protuberans is a rare locally aggressive cutaneous tumor of intermediate malignancy. It is a slow-growing neoplasm with a marked propensity to recur after resection. Head and neck involvement is unusual and distant metastases are quite rare but tend to be more frequent in tumors that undergo fibrosarcomatous degeneration. We present the imaging and corresponding histopathology in a case of dermatofibrosarcoma protuberans of the scalp demonstrating fibrosarcomatous degeneration and lung metastasis.

scholarly journals Giant Darier–Ferrand dermatofibrosarcoma protuberans of the abdomen and pelvis: a case report

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
A. Triki ◽  
M. Aloui ◽  
M. Ghalleb ◽  
I. Jbir ◽  
Ben Naceur ◽  
...  
Keyword(s):  
Case Report ◽  
High Risk ◽  
Skin Flap ◽  
Dermatofibrosarcoma Protuberans ◽  
Metastatic Potential ◽  
Wide Excision ◽  
Skin Tumor ◽  
Local Skin ◽  
Case Presentation ◽  
Local Skin Flap

Abstract Background Darier–Ferrand dermatofibrosarcoma (DFS) is a rare malignant cutaneous neoplasm characterized by local aggressiveness, high risk of recurrence, and low metastatic potential. Case presentation A 60-year-old Tunisian man with recurrent abdominopelvic DFS for which he had undergone multiple excisions presented with an extensive DFS that invaded the external genitals. He underwent a large excision with emasculation and thin cutaneous graft of the abdominal wall and local skin flap in the pelvis. Conclusion DFS is a rare yet recurrent skin tumor. Wide excision with free margins remains the cornerstone of treatment. We report a case of a giant DFS treated with wide excision and reconstructive surgery to cover the defect.

Sign in / Sign up

Export Citation Format

Share Document