scholarly journals Low grade fibromyxoid sarcoma of the perineum: a surgical challenge

2016 ◽  
Vol 4 (1) ◽  
pp. 429
Author(s):  
Afzal Anees ◽  
Kaushal D. Singh ◽  
Shehtaj Khan
Keyword(s):  
Local Recurrence ◽  
Surgical Management ◽  
Surgical Excision ◽  
Low Grade ◽  
Rare Presentation ◽  
Desmoid Fibromatosis ◽  
Fibromyxoid Sarcoma ◽  
Slow Growing ◽  
Late Metastasis

Low-grade fibromyxoid sarcoma is a rare, slow growing and deceptively benign-appearing neoplasm. It is presently diagnosed on the basis of histopathology (typical fibro-myxoid appearance), immunohistochemistry (vimentin staining) and cytogenetics [chimeric FUS/CREB3L2 gene produced by t (7;16), (q33;p11)]. This tumour should be differentiated from other resembling tumours like myxofibrosarcoma, sclerosing epitheloid fibrosarcoma, desmoid fibromatosis and others. It has the potential for local recurrence and late metastasis, if not treated adequately. Although surgical excision is the only hope for treatment, reporting of details of surgical management have often been neglected for this primarily pathological entity. We report a case of a 30-year female who had a perineal mass which was managed by wide surgical excision. It was diagnosed as low-grade fibromyxoid sarcoma on the basis of histopathology and immunohistochemistry. This extremely rare presentation is, thus, discussed in context of challenging nature of its surgical excision. Patient is in follow-up and has no recurrence even after 5 years.

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.

scholarly journals Biphenotypic Sinonasal Sarcoma: A Case Report and Review of Literature

2021 ◽  
pp. 014556132199919
Author(s):  
Evan Kominsky ◽  
Andre E. Boyke ◽  
Daniel Madani ◽  
Ameet Kamat ◽  
Bradley A. Schiff ◽  
...  
Keyword(s):  
Surgical Excision ◽  
Adjuvant Radiation ◽  
Low Grade ◽  
Recurrence Rates ◽  
Radiologic Evaluation ◽  
Standardized Treatment ◽  
Slow Growing ◽  
Biphenotypic Sinonasal Sarcoma

Objectives: Biphenotypic sinonasal sarcoma (BSNS), previously low-grade sinonasal sarcoma with neural and myogenic features, is a rare tumor of the sinonasal tract first described in 2012. Due to its rarity, limited literature is available in providing clinicians with a standardized treatment regimen, particularly in cases of positive surgical margins. This article aims to provide a clinical review of the currently available reported cases of BSNS, as well as presenting clinical, radiologic, and pathologic details of 2 novel cases. Methods: Online electronic databases include PubMed and Embase where queried for reports of biphenotypic sinonasal sarcoma or low-grade sinonasal sarcoma with neural and myogenic features. Two previously unpublished cases were included in the results. Data including clinical presentation, epidemiologic data, radiologic evaluation, intraoperative details, histopathology, treatment modality, and postoperative follow-up information were included. Results: A total of 100 previously published cases were identified in 12 prior articles. Mean age at presentation was 52.9 years. Extrasinonasal extension was observed in 27.4% of cases with most common site of extension being cribriform plate. Forty-seven cases included treatment details with surgical excision being the most common modality. Recurrence rates were identical for both surgical excision alone and surgical excision with adjuvant radiotherapy (33.3%). Conclusions: Biphenotypic sinonasal sarcoma is a slow-growing tumor that is amenable to surgical resection. Recurrence rates are similar between surgical excision and surgical excision with adjuvant radiation therapy, but limited data in reported cases preclude a determination of treatment superiority.

Management and outcomes of phyllodes tumours – 10 year experience

2021 ◽  
pp. 1-6
Author(s):  
P.A. Boland ◽  
A. Ali Beegan ◽  
M. Stokes ◽  
M.R. Kell ◽  
J.M. Barry ◽  
...  
Keyword(s):  
Local Recurrence ◽  
Surgical Management ◽  
Surgical Excision ◽  
Breast Conserving Surgery ◽  
Tertiary Referral Centre ◽  
Benign Pathology ◽  
Phyllodes Tumour ◽  
Phyllodes Tumours ◽  
Tumour Characteristics

INTRODUCTION: Phyllodes tumours represent 0.3–1% of breast tumours, typically presenting in women aged 35–55 years. They are classified into benign, borderline and malignant grades and exhibit a spectrum of features. There is significant debate surrounding the optimal management of phyllodes tumour, particularly regarding appropriate margins. METHODS: This is a retrospective review of a prospectively maintained database of patients who underwent surgical management for phyllodes tumours in a single tertiary referral centre from 2007–2017. Patient demographics, tumour characteristics, surgical treatment and follow-up data were analysed. Tumour margins were classified as positive (0 mm), close (≤2 mm) and clear (>2 mm). RESULTS: A total of 57 patients underwent surgical excision of a phyllodes tumour. The Mean age was 37.7 years (range: ages 14–91) with mean follow-up of 38.5 months (range: 0.5–133 months). There were 44 (77%) benign, 4 (7%) borderline and 9 (16%) malignant phyllodes cases. 54 patients had breast conserving surgery (BCS) and 3 underwent mastectomy. 30 (53%) patients underwent re-excision of margins. The final margin status was clear in 32 (56%), close in 13 (23%) and positive in 12 (21%). During follow-up, 4 patients were diagnosed with local recurrence (2 malignant, 1 borderline and 1 benign pathology on recurrence samples). CONCLUSION: There are no clear guidelines for the surgical management and follow-up of phyllodes tumours. This study suggests that patients with malignant phyllodes and positive margins are more likely to develop local recurrence. There is a need for large prospective studies to guide the development of future guidelines.

scholarly journals Multiple Koenen Tumors, a Rare Entity: Combination Treatment with 1% Topical Sirolimus Electrofulguration and Excision

2020 ◽  
Vol 7 (1) ◽  
pp. 66-70
Author(s):  
Vishalakshi Viswanath ◽  
Jay D. Gupte ◽  
Niharika Prabhu ◽  
Nilima L. Gour
Keyword(s):  
Tuberous Sclerosis ◽  
Combination Treatment ◽  
Tumor Regression ◽  
Surgical Excision ◽  
Tumor Formation ◽  
Rare Entity ◽  
Rare Presentation ◽  
Cutaneous Manifestations ◽  
Elderly Female

<b><i>Introduction:</i></b> Koenen tumors are benign, cutaneous manifestations of tuberous sclerosis. These are disfiguring, painful, and challenging to treat as they frequently recur. We report a case of long-standing, multiple Koenen tumors affecting all twenty nails in an elderly female who was successfully treated with a combination of topical sirolimus 1%, surgical excision, and electrofulguration. <b><i>Case Report:</i></b> A 57-year-old lady presented with multiple, asymptomatic periungual, and subungual tumors affecting all twenty nails since 27 years. Cutaneous examination revealed confetti macules, ash-leaf macule, and shagreen patch over trunk. Nail biopsy was compatible with Koenen’s tumor. Computerized tomography of brain showed diffuse patchy sclerosis. The tumors were treated with topical sirolimus 1% ointment for 10 months with excellent regression. Electro­fulguration for both great toenails and surgical excision of right thumbnail periungual fibroma was done. 1% sirolimus was advised after the surgical treatment. There were no adverse effects or recurrence of tumors over a 2-year follow-up. <b><i>Discussion:</i></b> Topical sirolimus 1% was effective in tumor regression and preventing new tumor formation. Larger tumors that interfered in daily chores were treated with excision and electrofulguration. Thus, a combination treatment for this rare presentation of tuberous sclerosis provided optimum results.

scholarly journals Giant Parotid Pleomorphic Adenoma with Atypical Histological Presentation and Long-Term Recurrence-Free Follow-Up after Surgery: A Case Report and Review of the Literature

2020 ◽  
Vol 2020 ◽  
pp. 1-18
Author(s):  
Mohammed AlKindi ◽  
Sundar Ramalingam ◽  
Lujain Abdulmajeed Hakeem ◽  
Manal A. AlSheddi
Keyword(s):  
Pleomorphic Adenoma ◽  
Surgical Management ◽  
Early Stage ◽  
English Literature ◽  
Benign Tumors ◽  
Adjuvant Radiation ◽  
Extracapsular Dissection ◽  
Slow Growing

Salivary gland tumors (SGT) comprise 3% of all head and neck tumors, are mostly benign, and arise frequently in the parotid gland. Pleomorphic adenoma (PA) is the commonest SGT, representing 60-70% of all benign parotid tumors. Clinically, parotid PA presents as irregular, lobulated, asymptomatic, slow-growing preauricular mass, involving both superficial and deep lobes, and could grow to gigantic proportions. Histologically, PA has epithelial and mesenchymal elements in chondromyxoid matrix and is managed surgically. Based on a review of 43 cases reported in English literature since 1995, giant parotid PA is reported as large as 35 cm (diameter) and 7.3 kg (resected weight). Although rare, 10 cases of malignant transformation were reported in the review. Surgical management included extracapsular dissection (ECD), superficial parotidectomy, and total parotidectomy for benign tumors, and adjuvant radiation or chemotherapy for malignant tumors. We further present the case of a 36-year-old healthy male with slow-growing and asymptomatic giant parotid PA, of 4-year duration. The patient presented with firm, lobulated preauricular swelling, provisionally diagnosed as PA based on radiographic and cytological findings. The tumor was resected through ECD, and the patient had uneventful postoperative recovery and a 7-year recurrence-free follow-up period. Histological examination revealed epimyoepithelial proliferation punctuated by chondromyxoid areas, with extensive squamous metaplasia and keratin cysts. To the best of knowledge from indexed literature, giant parotid PA is rarely reported in Saudi Arabia. In addition to its rarity, this case is reported for its benign nature despite atypical histological presentation, successful surgical management without complications, and long-term recurrence-free follow-up. Based on this report, clinicians must be aware of atypical histological presentations associated with PA and plan suitable surgical management and follow-up to avoid morbidity. Nevertheless, attempts must be made to diagnose and manage these lesions at an early stage and before they reach gigantic proportions.

scholarly journals Gingival Lipoma: A Rare Case Report

2017 ◽  
Vol 1 (1) ◽  
pp. 37-39
Author(s):  
Sweta Shrestha ◽  
Shaili Pradhan ◽  
Ranjita Shrestha Gorkhali
Keyword(s):  
Rare Case ◽  
Surgical Excision ◽  
Soft Tissue Tumors ◽  
Rare Case Report ◽  
Postsurgical Complication ◽  
Benign Tumours ◽  
One Year ◽  
Slow Growing ◽  
Oral Soft Tissue

Lipomas are benign tumours of mesenchymal origin (mature adipocytes) that are comparatively uncommon in the oral cavity corresponding to less than 4.4% of all benign oral soft tissue tumors. Clinically, they present as slow growing, soft, asymptomatic masses. Histopathologically, they appear as thinly encapsulated lesion composed of mature adipocytes with inconspicuous vascularity. The pathogenetic mechanisms of oral lipomas are still unclear. They are usually treated by surgical excision and bear excellent prognosis. Here we report a case of intraoral lipoma in 54 year old male patient in the left lower lingual alveolar mucosal region that was treated by surgical excision using electrocautery without any postsurgical complication. One-year follow-up showed no evidence of recurrence.

scholarly journals Low-Grade Fibromyxoid Sarcoma of the Lateral Skull Base: Presentation of Two Cases

2019 ◽  
Vol 2019 ◽  
pp. 1-6
Author(s):  
Evgenia Chetverikova ◽  
Priit Kasenõmm
Keyword(s):  
Malignant Tumour ◽  
Low Grade ◽  
Loss Of Consciousness ◽  
First Case ◽  
Clinical Presentations ◽  
Lateral Skull Base ◽  
Fibromyxoid Sarcoma ◽  
Gag Reflex ◽  
The Right ◽  
Slow Growing

Low-grade fibromyxoid sarcoma (LGFMS) is a rare slow-growing malignant tumour with a deceptively benign histologic appearance. Herein, we report two cases of LGFMS with variable clinical presentations. The first case was a 17-year-old female who referred to our department due to deaf ear on the right together with ipsilateral gag reflex impairment and globus sensation in the pharynx. The second case was a 35-year-old female with recurrent LGFMS, suffering from headaches, vertigo, and episodes of loss of consciousness. LGFMS of the temporal bone is a rare pathology, and to the best of our knowledge, no such cases have been reported before.

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.

scholarly journals A Low-Grade Fibromyxoid Sarcoma of the Internal Abdominal Oblique Muscle

2016 ◽  
Vol 2016 ◽  
pp. 1-4
Author(s):  
Masakazu Hashimoto ◽  
Kei Koide ◽  
Michinori Arita ◽  
Koji Kawaguchi ◽  
Yoshihiro Mikuriya ◽  
...  
Keyword(s):  
Radical Resection ◽  
Oblique Muscle ◽  
Low Grade ◽  
First Case ◽  
Positron Emission ◽  
Muc4 Expression ◽  
Highly Sensitive ◽  
Fibromyxoid Sarcoma ◽  
Fluorine 18 Fluorodeoxyglucose

A low-grade fibromyxoid sarcoma (LGFMS) is a rare tumor, with a benign histologic appearance but malignant behavior. This report describes a 74-year-old man with an internal abdominal oblique muscle mass. The tumor appeared as a well-defined ovoid mass on computed tomography, with mild uptake on fluorine-18-fluorodeoxyglucose positron-emission tomography images. Radical resection with wide safe margins was performed. Histologically, the tumor was composed of spindle-shaped cells in a whorled growth pattern, with alternating fibrous and myxoid stroma. MUC4 expression, a highly sensitive and specific immunohistochemical marker for LGFMS, was detected. Therefore, we diagnosed the tumor as LGFMS. At the 3-month follow-up, there was no sign of recurrence or metastasis. We report the first case of LGFMS arising from the internal abdominal oblique muscle.

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