scholarly journals A Rare Malignant Disease, Dermatofibrosarcoma Protuberans of the Breast: A Retrospective Analysis and Review of Literature

2020 ◽  
Vol 2020 ◽  
pp. 1-10
Author(s):  
Yihua Wang ◽  
Yu Wang ◽  
Rui Chen ◽  
Zhenrong Tang ◽  
Shengchun Liu
Keyword(s):  
Retrospective Analysis ◽  
Smooth Muscle Actin ◽  
Clinical Information ◽  
Dermatofibrosarcoma Protuberans ◽  
Surgical Excision ◽  
Low Grade ◽  
Pubmed Database ◽  
Storiform Pattern ◽  
Rare Malignancy

Dermatofibrosarcoma protuberans (DFSP) is a rare low-grade fibroblastic mesenchymal tumor derived from the dermis. The aim of this retrospective analysis was to summarize the clinicopathological data from our cases and published cases to offer more evidence for the recognition of dermatofibrosarcoma protuberans (DFSP). A total of 6 breast DFSP patients who had received treatment in our hospital were retrospectively enrolled, and detailed clinicopathological data were gathered for analysis. The median age was 29.5 years (ranging from 17 to 42 years). Most cases presented a red or brown-red, mobile, well-circumscribed, protruding, breast mass (ranging from 1 to 3 cm). For histopathology, all cases (6/6) showed a storiform pattern of spindle cells that were positive for CD34 (6/6) and Vimentin (5/6) and negative for smooth muscle actin (0/6) and S-100 protein (0/6). The majority of patients (5/6) underwent wide local excision, with 2 cases treated with radiotherapy. With a median follow-up of 36 months, all 6 patients survived without recurrence or metastasis. The PubMed database was used to search for similar cases. Eventually, 36 cases were included in this review, while cases without detailed clinical information or not reported in English were excluded from the analysis. To summarize, DFSP of the breast is an extremely rare malignancy characterized by spindle tumor cells arranged in a storiform pattern and positivity for CD34. The core needle biopsy is one of the crucial methods for its preoperative diagnosis. Management of DFSP is mainly based on surgical excision. It is prone to local recurrence, so long-term follow-up is required.

Piloleiomyosarcoma in Seven Ferrets

2001 ◽  
Vol 38 (6) ◽  
pp. 710-711 ◽  
Author(s):  
B. H. Rickman ◽  
L. E. Craig ◽  
M. H. Goldschmidt
Keyword(s):  
Smooth Muscle ◽  
Smooth Muscle Cells ◽  
Immunohistochemical Staining ◽  
Smooth Muscle Actin ◽  
Surgical Excision ◽  
Muscle Actin ◽  
Mustela Putorius ◽  
Nuclear Pleomorphism ◽  
Complete Surgical Excision

In each of seven ferrets ( Mustela putorius furo) with leiomyosarcoma, a single dermal mass was identified and biopsied. Each mass consisted of a well-demarcated but nonencapsulated proliferation of large spindle- to strap-shaped cells arranged in interwoven bundles. The cells resembled the smooth muscle cells of the adjacent arrector pili muscles, but with marked nuclear pleomorphism. Immunohistochemical staining for smooth muscle actin, desmin, and vimentin was positive and staining for myoglobin and cytokeratin was negative. Follow-up on three of the ferrets indicates that the prognosis is good following complete surgical excision.

A Novel Surgical Stapling Technique for Rectal Mass Removal: A Retrospective Analysis

2009 ◽  
Vol 45 (2) ◽  
pp. 67-71 ◽  
Author(s):  
Jen Swiderski ◽  
Stephen Withrow
Keyword(s):  
Adjuvant Therapy ◽  
Retrospective Analysis ◽  
Surgical Excision ◽  
Histopathological Diagnosis ◽  
Surgical Stapling ◽  
Stapling Device ◽  
Novel Technique ◽  
Rectal Mass ◽  
Mean Time

Both benign and malignant rectal masses occur in dogs. The mainstay of treatment is surgical excision with adjuvant therapy based on histopathological diagnosis and completeness of removal. Location of the mass within the rectum helps dictate the approach used. This paper describes the use of a novel technique for removal of rectal masses involving the distal third of the rectum in seven dogs. To perform this technique, the rectum is prolapsed and stay sutures are placed to maintain prolapse. A thoracoabdominal stapling device is placed at the base of the mass with a minimum of 0.5- to 1-cm margins, and the mass is amputated. Mean time to veterinarian follow-up was 564 days, and no dog had recurrence of disease during this time.

scholarly journals Dermatofibrosarcoma Protuberans: A Rare Cause of Neck Swelling

2020 ◽  
Vol 22 (2) ◽  
pp. 110-113
Author(s):  
Md Abdur Rahman ◽  
Mahbubul Alam ◽  
ABM Luthful Kabir ◽  
Mohammod Harun Or Rarhid ◽  
Abul Kalam Prodhan
Keyword(s):  
Adjuvant Radiotherapy ◽  
Cutaneous Lesion ◽  
Dermatofibrosarcoma Protuberans ◽  
Complete Removal ◽  
Low Grade ◽  
Incisional Biopsy ◽  
Neck Swelling ◽  
Rare Tumour ◽  
History Of

Dermatofibrosarcomaprotuberans (DFSP) is a low-grade soft tissue sarcoma (fibrosarcoma) originated from dermal and subdermal layer of the skin. The tumor infiltrates into the deeps in the form of villous or finger. For this reason, it is quite difficult to get clear surgical margins during tumor excision and the recurrence is a problem encountered frequently. We are reporting this rare tumour in a 40-year-old man presented with a 5-years history of slowly growing cutaneous lesion of the neck. Incisional biopsy confirmed the diagnosis of DFSP. Subsequently, the patient underwent wide local surgical resection, followed by reconstruction. Histopathology report revealed dermatofibrosarcomaprotuberans (DFSP). Although DFSP behave as non-aggressive malignancy, surgery with complete removal of the affected area is the treatment of choice. Moreover, adjuvant radiotherapy and follow-up of the patient is essential in order to prevent recurrence. Bangladesh J Otorhinolaryngol; October 2016; 22(2): 110-113

scholarly journals Low-grade myofibroblastic sarcoma of retroperitoneum: a rare case

2019 ◽  
Vol 6 (2) ◽  
pp. 633
Author(s):  
Suresh K. Choudhary ◽  
Shalu Gupta ◽  
Somendra Bansal ◽  
Narender Kumar
Keyword(s):  
Splenic Flexure ◽  
Soft Tissues ◽  
Imaging Modality ◽  
Smooth Muscle Actin ◽  
Abdominal Cavity ◽  
Neck Region ◽  
Low Grade ◽  
Tissue Mass ◽  
Myofibroblastic Sarcoma

Low-grade myofibroblastic sarcoma (LGMFS) is an uncommon tumor which develops mainly in the bone or soft tissues of the head and neck region, trunk, or extremities and extremely rarely found in the abdominal cavity. The rarity of the disease and its low-grade features make an accurate diagnosis difficult in most cases. We recently encountered a giant LGMFS which had developed in retroperitoneum and surgically resected with gratifying results. An 18 years old female presented with complaint of left sided abdominal pain since 8 months and left sided abdominal lump since 5 months. Imaging examinations revealed retroperitoneal soft tissue mass, and surgical treatment was scheduled. During operation, a tumor about 20x18x15cm in diameter with its anterior aspect covered with the pancreas, mesocolon and splenic flexure of colon with densely adhered to splenic vessels and left renal vessels was found. The tumor had firm adhesions to the surrounding tissues, and it was excised with concomitant distal pancreato-splenectomy, left nephrectomy and resection of splenic flexure of colon with colo-colic anastomosis. Histopathologically, fusiform cells were arranged in a complicated or storiform pattern, and immunohistochemical staining revealed that the tumor was positive for vimentin and focally positive for α-smooth muscle actin (SMA), negative for desmin, CD34, CD117, EMA, DOG-1 and S-100. Diagnosis of LGMFS was made. During 6 months follow up patient is asymptomatic and ultrasound abdomen is normal. In case of LGMFS, favorable prognosis can be attained by complete resection of the primary lesion and regular follow up of patient by physical examination and imaging modality.

scholarly journals Clinical and molecular characterization of a multi-institutional cohort of pediatric spinal cord low-grade gliomas

2020 ◽  
Vol 2 (1) ◽  
Author(s):  
Sydney T Grob ◽  
Liana Nobre ◽  
Kristen R Campbell ◽  
Kurtis D Davies ◽  
Scott Ryall ◽  
...  
Keyword(s):  
Polymerase Chain Reaction ◽  
Clinical Information ◽  
Complete Fusion ◽  
Low Grade ◽  
Chain Reaction ◽  
Braf Mutations ◽  
Genetic Aberrations ◽  
Low Grade Gliomas ◽  
Polymerase Chain

Abstract Background The mitogen-activated protein kinases/extracelluar signal-regulated kinases pathway is involved in cell growth and proliferation, and mutations in BRAF have made it an oncogene of interest in pediatric cancer. Previous studies found that BRAF mutations as well as KIAA1549–BRAF fusions are common in intracranial low-grade gliomas (LGGs). Fewer studies have tested for the presence of these genetic changes in spinal LGGs. The aim of this study was to better understand the prevalence of BRAF and other genetic aberrations in spinal LGG. Methods We retrospectively analyzed 46 spinal gliomas from patients aged 1–25 years from Children’s Hospital Colorado (CHCO) and The Hospital for Sick Children (SickKids). CHCO utilized a 67-gene panel that assessed BRAF and additionally screened for other possible genetic abnormalities of interest. At SickKids, BRAFV600E was assessed by droplet digital polymerase chain reaction and immunohistochemistry. BRAF fusions were detected by fluorescence in situ hybridization, reverse transcription polymerase chain reaction, or NanoString platform. Data were correlated with clinical information. Results Of 31 samples with complete fusion analysis, 13 (42%) harbored KIAA1549–BRAF. All 13 (100%) patients with confirmed KIAA1549–BRAF survived the entirety of the study period (median [interquartile range] follow-up time: 47 months [27–85 months]) and 15 (83.3%) fusion-negative patients survived (follow-up time: 37.5 months [19.8–69.5 months]). Other mutations of interest were also identified in this patient cohort including BRAFV600E, PTPN11, H3F3A, TP53, FGFR1, and CDKN2A deletion. Conclusion KIAA1549–BRAF was seen in higher frequency than BRAFV600E or other genetic aberrations in pediatric spinal LGGs and experienced lower death rates compared to KIAA1549–BRAF negative patients, although this was not statistically significant.

scholarly journals Left convex thoracic scoliosis: retrospective analysis of 25 patients after surgical treatment

2011 ◽  
Vol 10 (3) ◽  
pp. 205-210
Author(s):  
Birgit Deetjen ◽  
Ulf Liljenqvist ◽  
Tobias L. Schulte ◽  
Carolin Schmidt ◽  
Tobias Lange ◽  
...  
Keyword(s):  
Cobb Angle ◽  
Retrospective Analysis ◽  
Perioperative Complications ◽  
Congenital Scoliosis ◽  
Significant Loss ◽  
Low Grade ◽  
Scoliosis Surgery ◽  
Prolonged Intubation ◽  
Major Complications

OBJECTIVE: A retrospective analysis of clinical and radiological data was conducted, with an emphasis on perioperative complications and risk factors and a minimum follow-up period of two years. The postoperative quality of life was assessed using the SRS-22 questionnaire. METHODS: Between 1999 and 2009, 25 patients (nine male, 16 female) with LCTS, with a mean age of 13.7 years (2.3-29.8 years), were treated with correction and instrumented fusion at a single institution. Seven patients had congenital scoliosis and 18 patients had noncongenital scoliosis (idiopathic, n = 5; neuropathic, n = 4; neoplasm-associated/iatrogenic, n = 3; secondary to other conditions, n = 6). The average preoperative Cobb angle was 74° (49-102°). RESULTS: A mean correction of 51% was achieved postoperatively. The mean Cobb angle at the final follow-up examination was 45° (19-85°), with a significant loss of correction of 8.8° on average. Major complications affected five patients (20%): respiratory insufficiency requiring prolonged intubation, intraoperative cardiac arrest with resuscitation being necessary twice in one patient, persistent clonus, low-grade infection, implant-based complications requiring revision surgery, and adding-on. Minor complications were observed in 22 patients (88%), mainly gastrointestinal and pulmonary. No cases of paraplegia or death occurred. A noncongenital etiology had been diagnosed before the age of 10 years in all of the patients who had major complications. The best score on the SRS-22 questionnaire was achieved in the domain of pain (87%), while the poorest was in the domain of self-image (68%). CONCLUSIONS: The results of this study emphasize an increased complication rate in patients with LCTS scheduled for scoliosis surgery. Additional preoperative examinations (MRI, paediatric consultation, cardiologic consultation, pulmonary function test) are mandatory in patients with LCTS. Preoperatively, patients should be informed about the increased cardiopulmonary and neurological risk which may be associated with scoliosis surgery.

Concordance of Intradepartmental Consultations of Barrett’s Dysplasia

2019 ◽  
Vol 152 (Supplement_1) ◽  
pp. S54-S55
Author(s):  
Fengming Chen ◽  
Yongjun Liu ◽  
Francesca Ruggiero
Keyword(s):  
Patient Management ◽  
Clinical Information ◽  
Final Diagnosis ◽  
Progression Rate ◽  
Low Grade ◽  
Rate Of Progression ◽  
Major Discrepancy ◽  
A Minor ◽  
Optimal Patient Management

Abstract Introduction Barrett’s esophagus (BE) is a well-known precursor to esophageal adenocarcinoma (EAC). Simple BE has an annual rate of progression to EAC of only up to 0.5%, while BE with low-grade dysplasia (LGD) or with high-grade dysplasia (HGD) has a higher progression rate of ~10% and ~40%, respectively. Therefore, accurate diagnosis and grading of dysplasia in BE are critical for optimal patient management. However, grading dysplasia is not well defined in practice, which often results in poor interobserver and/or intraobserver reproducibility. In this study, we aim to (1) investigate the concordance of intradepartmental consultations of BE dysplasia and (2) compare consultant diagnosis with final diagnosis and follow-up diagnosis. Methods We retrospectively reviewed 856 intradepartmental consultation records obtained from May 2017 to March 2018. For cases of Barrett’s dysplasia in biopsy specimens, H&E-stained slides were re-reviewed and the corresponding clinical information was retrieved from the electronic medical record. Results Twenty intradepartmental consultation cases of Barrett’s dysplasia were identified (involving 2 females and 18 males, mean age 67.8 ± 8.6 years, ranging 50-81 years). The most frequent reasons for consultation were indefinite dysplasia (IND) vs LGD and LGD vs HGD. Half of the cases showed concordance between referring pathologist and consultant pathologist(s), while 10% of the case showed a major discrepancy (resulting in significant changes in patient management and/or prognosis) and 40% showed a minor discrepancy (resulting in no significant impact on patient management and/or prognosis). The final diagnoses were changed after consultation for cases with major discrepancy, while 60% of cases with minor discrepancy remained the original diagnoses. Conclusions Intradepartmental consultations are strongly recommended for the challenging cases of BE dysplasia, which can effectively prevent over- or underdiagnosis. For challenging cases such as IND vs LGD, two or more consultants are usually needed to reach an agreement.

scholarly journals Primary Columellar Angiosarcoma: A Case Report

2005 ◽  
Vol 84 (1) ◽  
pp. 45-51 ◽  
Author(s):  
Pedro Oliveira ◽  
Ricardo Correia ◽  
Eugénia Castro ◽  
Rosete Almeida ◽  
Agostinho Silva
Keyword(s):  
Head And Neck ◽  
Surgical Excision ◽  
Long Term Follow Up ◽  
Rare Malignancy ◽  
High Level ◽  
Immunohistochemical Studies ◽  
High Degree ◽  
Extensive Surgery

Angiosarcoma of the head and neck, a rare malignancy, is associated with a high degree of invasiveness and poor survival. A high level of suspicion followed by histopathologic and immunohistochemical studies is warranted in order to arrive at a well-timed and accurate diagnosis. We report the case of a 56-year-old man who developed an unusually small neoplasm in the nasal columella. Rapid diagnosis allowed for simple treatment with surgical excision, a rare circumstance because most of these tumors require extensive surgery. Close long-term follow-up of patients with angiosarcoma of the head and neck is vitally important.

scholarly journals Multiple Histological Subtypes of Dermatofibrosarcoma Protuberans Occurring in the Same Tumor

2015 ◽  
Vol 53 (1) ◽  
pp. 81-90
Author(s):  
C. Socoliuc ◽  
Sabina Zurac ◽  
R. Andrei ◽  
Florica Stăniceanu
Keyword(s):  
Tumor Cells ◽  
Histopathological Examination ◽  
Smooth Muscle Actin ◽  
Dermatofibrosarcoma Protuberans ◽  
Giant Cells ◽  
Negative Staining ◽  
Low Grade ◽  
Body Type ◽  
Morphological Aspects ◽  
Resection Specimen

AbstractDermatofibrosarcoma protuberans (DFSP) represents a low-grade cutaneous sarcoma which may have different histological aspects, presenting as a fibrosarcomatous, pigmented, juvenile, myxoid, atrophic, sclerosing or myoid lesion. Some of these subtypes may occur isolated or in association with one of the others creating hybrid lesions. We present the case of a 66 years old woman having a 4 cm diameter tumor located on the abdominal wall. Histopathological examination of the resection specimen revealed areas of typical DFSP associated with fibrosarcomatous transformation, myoid and myxoid areas. Also, focally, pleomorphic tumor cells and foreign-body type multinucleated giant cells were observed. Immunostains revealed CD34 positivity in typical DFSP and myxoid areas with negative staining of some of the tumor cells in fibrosarcomatous areas and negative staining of myoid areas. Smooth muscle actin was positive in myoid areas. The nature of myoid fascicles in DFSP is a matter of debate, being uncertain whether these represent a type of tumor differentiation or a reactive myoid proliferation. In this particular case, finding the association of myoid cells with blood vessel walls sustains their reactive nature. We present the morphological aspects of the different areas of the tumor with emphasis on differential diagnostic problems and clinical implications.

scholarly journals Giant Low-Grade Fibromyxoid Sarcoma in the Neck

2020 ◽  
Vol 63 (9) ◽  
pp. 432-435
Author(s):  
Jong Min Park ◽  
Hye Rin Lim ◽  
Jo Heon Kim ◽  
Dong Hoon Lee
Keyword(s):  
Neck Region ◽  
Surgical Excision ◽  
Late Recurrence ◽  
Low Grade ◽  
Head And Neck Region ◽  
Long Term Follow Up ◽  
Fibromyxoid Sarcoma ◽  
Middle Aged Adults

Low-grade fibromyxoid sarcoma (LGFS) is a soft tissue tumor that rarely occurs in the head and neck region. It occurs mainly in the proximal extremities and the trunk and is prevalent in the young and middle-aged adults. In the present case, LGFS was present at an atypical location and at an unusual age. The treatment of choice for LGFS is radical wide surgical excision with a clear margin. Long-term follow-up is essential for all patients with LGFS, as it has the potential for late recurrence or metastasis.

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