scholarly journals Dermatofibrosarcoma Protuberans: Update on the Diagnosis and Treatment

2020 ◽  
Vol 9 (6) ◽  
pp. 1752 ◽  
Author(s):  
Xingpei Hao ◽  
Steven D. Billings ◽  
Fangbai Wu ◽  
Todd W. Stultz ◽  
Gary W. Procop ◽  
...  
Keyword(s):  
Targeted Therapy ◽  
Soft Tissue ◽  
Malignant Tumors ◽  
Dermatofibrosarcoma Protuberans ◽  
Surgical Excision ◽  
Clinical Staging ◽  
Surveillance Program ◽  
Fusion Transcripts ◽  
Complete Surgical Excision ◽  
High Local Recurrence Rate

Dermatofibrosarcoma protuberans (DFSP) is a slow growing, low- to intermediate-grade dermal soft-tissue tumor. It has a high local recurrence rate but low metastatic potential. It is characterized by a uniform spindle cell arrangement, classically with a storiform pattern and CD34 immunoreactivity. The histomorphology and immunophenotype overlap with a broad range of other neoplasms. The standard treatment is complete surgical excision. The surgical procedures include wide local excision (WLE) with tumor free margins, Mohs micrographic surgery (MMS) and amputation. Unresectable DFSPs are treated with radiation therapy and/or targeted therapy. DFSP has characteristic t(17; 22) (q22; q13), resulting in a COL1A1- PDGFB fusion transcripts in more than 90% of DFSPs. Molecular detection of the gene rearrangement or fusion transcripts is helpful for the diagnosis of patients with atypical morphology and for screening candidates for targeted therapy with tyrosine kinase inhibitors. The aims of the present review are to update the clinical presentation, tumorigenesis and histopathology of DFSP and its variants for diagnosis and differential diagnosis from other benign and malignant tumors, to compare the advantages and drawbacks of WLE and MMS, to propose the baseline for selecting surgical procedure based on tumor’s location, size, stage and relationship with surrounding soft tissue and bone structures, and to provide a biologic rationale for the systemic therapy. We further propose a modified clinical staging system of DFSP and a surveillance program for the patients after surgical excision.

scholarly journals Chondrosarcoma of the Nasal Septum—A Rare Subsite: Case Report with Review of Literature

2021 ◽  
Vol 42 (05) ◽  
pp. 506-509
Author(s):  
Nidhi Gupta ◽  
Awadhesh Kumar Pandey ◽  
Kislay Dimri ◽  
Surinder K Singhal ◽  
Neeraj Rathee ◽  
...  
Keyword(s):  
Nasal Septum ◽  
Malignant Tumors ◽  
Surgical Excision ◽  
Metastatic Potential ◽  
Young Female ◽  
Resection Margins ◽  
Diagnostic Dilemma ◽  
Complete Surgical Excision ◽  
Grade Ii ◽  
Common Primary Malignant Bone

AbstractChondrosarcomas are the second most common primary malignant bone tumors. Head and neck chondrosarcomas constitute less than 10% of these tumors, rarely arising from the nasal septum. These are locally aggressive malignant tumors arising from the cartilaginous framework of the nasal septum. Rarity of the tumor coupled with nonspecific symptoms makes it a diagnostic dilemma. Diagnosis requires endoscopy, radiology, and final histopathology for confirmation. Treatment is mainly surgical, requiring complete surgical excision with clear margins. Radiation has a role in unresectable tumors or for tumors with positive margins after surgery. Survival depends on the grade of tumor that predicts the metastatic potential of the tumor. We present a rare case of chondrosarcoma arising from the nasal septum in a 29-year-old young female presenting with complaints of nasal obstruction. Computed tomography was suggestive of a calcified cartilaginous tumor arising from the nasal septum. Endoscopic excision was done and postoperative histopathology showed grade II chondrosarcoma with clear margins. No adjuvant treatment was given to our patient and 2 years post-excision patient is disease free.To conclude, chondrosarcoma of the nasal septum is a rare tumor, with nonspecific symptoms. Surgery with clear margins remains the treatment of choice. Prognosis depends on the extent of tumor at presentation, resection margins, and grade of tumor.

scholarly journals Liposarcoma of mesorectum presenting as chronic intestinal obstruction: a rare case

2018 ◽  
Vol 5 (10) ◽  
pp. 3437 ◽  
Author(s):  
Rajesh Goud E. ◽  
Muvva Sri Harsha ◽  
Jakkula Srikanth ◽  
Kanmathareddy Amulya
Keyword(s):  
Soft Tissue ◽  
Local Recurrence ◽  
Rare Case ◽  
Spindle Cell ◽  
Surgical Excision ◽  
Clear Cut ◽  
Complete Surgical Excision ◽  
Neoplastic Lesions ◽  
Cell Variant ◽  
Well Differentiated

The term ‘liposarcoma’ refers to a spectrum of neoplastic lesions that can be benign to malignant and likely to recur or metastasize. Liposarcoma is a common soft tissue sarcoma found in adults and occurs mostly in extremities especially thigh followed by retroperitoneum. Here we present a case of spindle cell variant of well differentiated liposarcoma of mesorectum and underwent Anterior resection for the same. The treatment of choice for liposarcomas of retroperitoneum remains debatable and there are no clear-cut guidelines available regarding the same. However, considering the higher rates of local recurrence we advise a complete surgical excision following anatomical principles.

scholarly journals Nasolabial hamartoma: a rare case report

2020 ◽  
Vol 6 (7) ◽  
pp. 1397
Author(s):  
D. Senthamarai Kannan ◽  
G. Soundara Rajan ◽  
Veerasigamani Narendrakumar ◽  
V. K. Sathiya
Keyword(s):  
Case Report ◽  
Soft Tissue ◽  
Rare Case ◽  
Surgical Excision ◽  
The Body ◽  
Nasolabial Fold ◽  
Tissue Development ◽  
Inborn Errors ◽  
Complete Surgical Excision ◽  
Rare Case Report

<p class="abstract">Nasolabial cysts are rare, nonodontogenic soft tissue developmental cysts occur in the maxillary lip and nasal alar regions. Patients usually presents with an asymptomatic soft swelling with obliteration of the nasolabial fold. Due to it's origin from entrapped epithelium in an embryonic fusion plane developmentally, this cyst is considered to be a Hamartoma. Hamartomas are non-neoplastic malformations, or inborn errors of tissue development. They are characterized by an abnormal mixture of tissues indigenous to that area of the body. Complete surgical excision is the accepted method of treatment. This report aimed to present a case of nasolabial cyst hamartoma, which is rare in presentation.</p>

scholarly journals PLASTIC SURGERY & ADVANCED SCALP TUMORS: EPIDEMIOLOGICAL PROFILE AND SURGICAL MANAGEMENT ABOUT 65 CASES

2021 ◽  
Vol 9 (5) ◽  
pp. 1171-1177
Author(s):  
Sahibi Mohamed Elmehdi ◽  
Mahrouch El Mehdi ◽  
Yafi Imane ◽  
Elgueouatri Mehdi ◽  
Zineddine Ismail ◽  
...  
Keyword(s):  
Squamous Cell Carcinoma ◽  
Cell Carcinoma ◽  
Plastic Surgery ◽  
Surgical Management ◽  
Squamous Cell ◽  
Malignant Tumors ◽  
Dermatofibrosarcoma Protuberans ◽  
Surgical Excision ◽  
Frequent Site ◽  
Epidemiological Profile

The scalp is the most frequent site of occurrence of malignant tumors. As an area that is generally neglected by the patient and not closely monitored during physical examinations. Plastic surgery has a double interest in the management of malignant tumors of the scalp: it allows oncologic tumor excision and coverage of defects induced. The present study reports 65 cases of giant malignant tumors of the scalp and who underwent surgical management, from January 2011 to June 2019. It included oncologic resection and repair of the defect. The average age in our series was 69.3 years, with a sex ratio of 5.1. Disease duration before consultation was on average 21 months. The location of the tumor was mostly parietal in 75%. The histological types were found in our series: 36 cases of squamous cell carcinoma, 16 cases of basal cell carcinoma, 6 cases of melanoma, 3 cases of trichilemmal carcinoma, 2 cases of dermatofibrosarcoma protuberans and one case of sarcoma and one case of adenoid cystic carcinoma . To cover the defect we used: skin graft in 44 cases and by local flaps in 21 cases. 42 patients benefited from radiotherapy and the evolution was marked by a reactivation in 3 cases of squamous cell carcinoma. The management of malignant tumors of the scalp is surgical and consists of two components: surgical excision and coverage. This repair is achieved through multiple techniques, facilitated by the vascular richness of this anatomical region.

scholarly journals Nasolabial cyst: case report and review of management options

BMC Surgery ◽  
2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Abdulhakeem Almutairi ◽  
Abeer Alaglan ◽  
Mazyad Alenezi ◽  
Sultan Alanazy ◽  
Osama Al-Wutayd
Keyword(s):  
Soft Tissue ◽  
Nasal Obstruction ◽  
Surgical Excision ◽  
Mass Effect ◽  
Treatment Modalities ◽  
Open Approach ◽  
Management Options ◽  
Case Presentation ◽  
Complete Surgical Excision

Abstract Background Nasolabial cysts are rare, non-odontogenic, soft-tissue cysts that develop between the upper lip and nasal vestibule with an overall incidence of 0.7% out of all maxillofacial cysts. The predominant presentation of a nasolabial cyst is a painless localized swelling with varying degrees of nasal obstruction. Several treatment modalities have described in the management of the nasolabial cyst. In this paper, we present a case of a nasolabial cyst in a 44 years old man with discussions of the treatment modalities in the lights of the literature. Case presentation We present a case of a nasolabial cyst in a 44-year-old man that slowly increased in size through a period of 3 years, with associated mild pain and nasal obstruction. It had caused a mass effect upon the maxilla, resulting in scalloping. The cyst was excised entirely with no evidence of recurrence at the two months follow up. Conclusions The nasolabial cyst is a rare soft-tissue cyst. Complete surgical excision using an open approach performed to our case, which considered with the complete endoscopic removal of the best treatment for the nasolabial cysts with a rare recurrence rate.

Primary Leiomyosarcoma of the Pancreas

2001 ◽  
Vol 125 (1) ◽  
pp. 152-155
Author(s):  
Gabriella Nesi ◽  
Desiree Pantalone ◽  
Ilaria Ragionieri ◽  
Andrea Amorosi
Keyword(s):  
Smooth Muscle ◽  
World Literature ◽  
Soft Tissues ◽  
Malignant Tumors ◽  
Smooth Muscle Actin ◽  
Surgical Excision ◽  
High Rate ◽  
Complete Surgical Excision ◽  
Membrane Components ◽  
Primary Leiomyosarcoma

Abstract Primary leiomyosarcoma of the pancreas is a rare tumor for which only 21 reports appear in the world literature. We describe an additional case of pancreatic leiomyosarcoma in a 76-year-old man, who complained of persistent high fever. Histologic examination revealed a pleomorphic spindle cell tumor. Reactivity for muscle-specific actin, α-smooth muscle actin, and basement membrane components, along with negative staining for epithelial and neural markers, were consistent with a smooth muscle sarcoma. The patient died of disease 1 year after complete surgical excision. This report highlights the need to use a complete antibody panel in order to accurately immunophenotype pleomorphic malignant tumors of the pancreas. A review of the cases compiled in the literature indicates that pancreatic leiomyosarcoma, like its counterpart arising in deep soft tissues, is an aggressive neoplasm characterized by short survival and a high rate of metastases.

scholarly journals Granular Cell Tumor over the Mons Pubis: An Uncommon Tumor

2019 ◽  
Vol 2019 ◽  
pp. 1-3 ◽  
Author(s):  
M. Patabendige ◽  
D. J. Wickramasooriya ◽  
L. Dasanayake
Keyword(s):  
Soft Tissue ◽  
Granular Cell Tumor ◽  
Granular Cell ◽  
Surgical Excision ◽  
Cell Tumor ◽  
The Body ◽  
Complete Surgical Excision ◽  
Neural Origin ◽  
Labium Majus ◽  
Uncommon Tumor

Granular cell tumors are uncommon, usually benign, soft tissue neoplasms of neural origin. They occur throughout the body; vulval involvement is uncommon and labium majus is the commonest site in vulva. Complete surgical excision is the preferred treatment of choice to prevent recurrence. Here, we present a benign granular cell tumor over the mons pubis of vulva in a 27-year-old woman.

scholarly journals Statistically Based Survival Rate Estimation in Patients with Soft Tissue Tumors

2018 ◽  
Vol 1 (Supplement) ◽  
pp. 7
Author(s):  
B. Şerban ◽  
Z. Panti ◽  
M. Nica ◽  
M. Pleniceanu ◽  
M. Popa ◽  
...  
Keyword(s):  
Survival Rate ◽  
Soft Tissue ◽  
Healing Rate ◽  
Malignant Tumors ◽  
Surgical Excision ◽  
Soft Tissue Tumors ◽  
Benign Tumors ◽  
Rate Estimation ◽  
Differentiation Degree ◽  
Histopathological Type

Abstract Introduction. Although most soft tissue tumors are benign, with a high healing rate after surgical excision, there is a variety of malignant tumors with differences in progression and prognosis. The study aims to assess the survival rate in patients diagnosed with this pathology, based on the patient’s characteristics (age, gender, race), as well as the tumor’s (histological type, differentiation degree, location and size). Materials and methods. The retrospective study included a group of 103 patients diagnosed in our department during 2010 and 2017. Considering the high healing rate of benign tumors, only the group of neoplastic patients (45 cases) was involved in the survival rate estimation, assessing tumor characteristics and individual comorbidities. Within this lot, we emphasized a predominance of neoplasm in patients aged over 50 years (32 cases), men (29 cases), and localization of the neoplasm in the thigh (23 cases). The predominant histopathological type, liposarcoma, was diagnosed in 67% of the cases, with dimensions over 6 cm and with local extension. Results. There have been significant variations in mortality between the different histological subtypes (liposarcoma vs. synovial sarcoma). Local recurrences were shown in 18 cases of liposarcoma in the first 2 years after the surgical excision, with an increased aggressiveness of this neoplasm in men over 50 years. 12 cases developed distant metastasis, and, until the end of the study, 7 deaths were reported in 3 cases involving associated comorbidities. Conclusions. The five-year survival is inversely proportional to the extent of the tumor and the local invasion, as well as to the age of the patient. It is difficult to appreciate an overall survival rate in the context of a heterogeneous group of tumors so it must be evaluated for every histological subtype taking into account the patient’s particularities.

scholarly journals Statistically based survival rate estimation in patients with soft tissue tumors

2018 ◽  
Vol 1 (2) ◽  
pp. 84-89
Author(s):  
Bogdan Serban ◽  
Zsombor Panti ◽  
Mihai Nica ◽  
Marian Pleniceanu ◽  
Mihnea Popa ◽  
...  
Keyword(s):  
Survival Rate ◽  
Soft Tissue ◽  
Healing Rate ◽  
Malignant Tumors ◽  
Surgical Excision ◽  
Soft Tissue Tumors ◽  
Benign Tumors ◽  
Rate Estimation ◽  
Differentiation Degree ◽  
Histopathological Type

Abstract Although most soft tissue tumors are benign, with a high healing rate after surgical excision, there is a variety of malignant tumors with differences in progression and prognosis. The study aimed to assess the survival rate in patients diagnosed with this pathology, based on the patient’s characteristics (age, gender, race), as well as the tumor’s histological type, differentiation degree, location and size. The retrospective study included a group of 103 patients diagnosed during 2010 and 2017 in our department. Considering the high healing rate of benign tumors, only the group of neoplastic patients (45 cases) was involved in the survival rate estimation, assessing tumor characteristics and individual comorbidities. Within this lot, we emphasized a predominance of neoplasm in patients aged over 50 years (32 cases), men (29 cases), and localization of the neoplasm in the thigh (23 cases). The predominant histopathological type, liposarcoma, was diagnosed in 67% of the cases, with dimensions over 6 cm and with local extension. There have been significant variations in mortality between the different histological subtypes (liposarcoma vs. synovial sarcoma). Local recurrences were showed in 18 cases of liposarcoma in the first 2 years after the surgical excision, with an increased aggressiveness of this neoplasm in men over 50 years. 12 cases developed distant metastasis, and until the end of the study, 7 deaths were reported in 3 cases involving associated comorbidities. The five-year survival is inversely proportional to the extent of the tumor and the local invasion, as well as to the age of the patient. An overall survival rate is difficult to appreciate in the context of a heterogeneous group of tumors so it must be evaluated for every histological subtype taking into account the patient’s particularities.

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