Connective Tissue Tumors Research Articles

S1957 Perforated Colonic GIST: A Rare Cause of Acute Abdomen

Introduction: Gastrointestinal stromal tumors (GISTs) are the most common connective tissue neoplasms of the gastrointestinal (GI) tract, usually found in the stomach and small intestine. Approximately 10-30% of patients with this malignancy are asymptomatic. Historically, GISTs have been discovered incidentally as patients often present with nonspecific symptoms such as abdominal pain, nausea, and weight loss. Most patients present with symptoms when the tumor grows >5 cm or with acute complications such as perforation or acute peritonitis. We report a rare case of acute abdomen secondary to a perforated colonic GIST treated by surgical resection and chemotherapy. Case description/methods: A 59-year-old female with hypertension and diabetes mellitus presented with abdominal discomfort for one day, associated with episodes of non-bilious, non-bloody emesis. She denied hematemesis, hematochezia, melena, weight loss, and changes to her bowel habits. Physical examination demonstrated diffuse abdominal tenderness without rigidity or guarding. Initial laboratory investigations revealed a leukocyte count of 17.2 × 103/uL. A computed tomography (CT) scan of the abdomen and pelvis without contrast demonstrated pneumoperitoneum. She was taken to the OR for an emergent exploratory and underwent Hartman’s procedure for a perforated colon. Pathology of the surrounding tissue revealed a high-grade GIST (positive for CD117, KI-67 value of 30%, and mitotic index > 5). Patient was started on Imatinib therapy. A subsequent CT scan five months after discharge revealed several new subcapsular liver masses, the largest measuring 2.6 × 2.2 cm along the falciform ligament and ascites in the left paracolic space. The patient follows with Oncology in outpatient clinic. Discussion: GISTs comprise < 3% of all gastrointestinal tumors and are associated with high rates of malignant transformations. While imagine modalities aid in identification, definitive diagnosis requires histopathological examination. Approximately 95% of GISTs express CD117, a component of the cKIT tyrosine kinase receptor, as in our patient. As majority of GISTs are found in the stomach and small intestine, it is very uncommon for this malignancy to manifest in the colon. Furthermore, it is extremely rare for GISTs to cause perforation and acute peritonitis. Clinical outcome in these cases is dependent on early identification and prompt intervention. While GISTs are rare, providers must consider this diagnosis when visualizing abnormal masses with perforations.

Ear and pubic keloids in an adolescent girl, the experience of a successful treatment

Keloids are frequent connective tissue tumors characterized by a significant proliferation of fibroblasts and collagen with a recurrent and extensive tendency. They can be secondary to a solution of cutaneous continuity: surgical wound; vaccination; burn; hard inflammatory lesion. Sometimes spontaneous, and are more frequently observed in black populations [1-3]. We report the case of a 20 year old women, melanoderma, with no known pathological history who presented to our department with multifocal slightly pruritic tumor lesions evolving for 4 years on the auricle and the pubis. The patient and her companions thought of a mystical disease. The interrogation revealed that these lesions occurred after an ear piercing and folliculitis lesions on the pubic area. The examination revealed a good general condition, firm non sensitive normochromic tumor lesions of variable size with smooth surface, protruding and adherent to the deep plane located on the pubis and bilaterally on the auricle.

English

Connective tissue neoplasms or soft tissue tumours as they are popularly called are mesenchymal neoplasms that affect any part of the body. They occur frequently in all organs including the head and neck region and the oral cavity is no exception. The frequency of their occurrence contributes to 15 - 20 % of all head and neck neoplasms and neoplasms of oral cavity form 1 - 4 % of the reported lesions,1,2 representing 2.2 % of all lipomas.3 In a study by Moreno SE et al. in 2016 the most frequently affected sites were the buccal mucosa followed by the lips, tongue, palate, and other areas of the oral cavity.4Here we are documenting a case occurring in the buccal mucosa.

A Rare Adnexal Tumor of Head &amp; Neck: Eccrine Spiradenoma

Eccrine Spiradenoma is a rare connective tissue tumor arising from the dermis of well differentiated eccrine sweat gland. It is considered benign nature, nonetheless, reported malignant cases are extremely rare. The most common presentation symptom is localized intradermal swelling and complete surgical excision is the gold standard in managing these cases.

Breast Sarcomas, Phyllodes Tumors, and Desmoid Tumors: Epidemiology, Diagnosis, Staging, and Histology-Specific Management Considerations.

Breast sarcomas arise from connective tissues of the breast and account for fewer than 1% of all breast malignancies. They can be subclassified as primary breast sarcomas, which arise de novo and are histologically diverse, and secondary breast sarcomas, which arise as a result of radiation or lymphedema and are most commonly angiosarcomas. Two other connective tissue neoplasms that occur within the breast include phyllodes tumors and desmoid tumors, which exhibit a spectrum of behaviors. Malignant phyllodes tumors are biologically similar to primary breast sarcomas, whereas desmoid tumors are technically benign but often locally aggressive. Patients with breast sarcomas often present with a rapidly growing mass or, in cases of radiation-associated angiosarcoma, violaceous cutaneous lesions. Core needle biopsy is generally required to confirm the diagnosis of sarcomas. Staging workup includes MRI and chest imaging, although these are not required in the case of benign phyllodes or desmoid tumors. In general, localized breast sarcomas should be resected, with the extent of resection tailored to histologic subtype. Radiation and chemotherapy can be used in the neoadjuvant or adjuvant setting, but data are limited, so treatment decisions should be made on an individualized basis. Systemic therapy options for metastatic disease and refractory breast desmoids mimic those used for the same histologies when present in other sites. Given the rarity and heterogeneity of breast sarcoma, as well as limited literature describing these entities, expert multidisciplinary evaluation is crucial for optimal decision making.

Donkey preputial fibrosarcoma (Equus asinus): case report

Little described in donkeys and of rare occurrence, fibrosarcoma is a malignant tumor of connective tissue. In this report, a 4-year-old donkey of the Pêga breed, used as a breeder, was referred to the Veterinary Hospital with a pendulum neoformation in the prepuce, according to owner with 1 month of evolution. Clinically, the animal was painfull and unconfortable when the tumor was manipulated, and the wound was ulcerated and with a focus of myiasis. An excisional biopsy was performed, and the mass was referred for histopathological examination, where the diagnosis of fibrosarcoma was obtained. Clinical aspects, diagnostic method and histopathological findings will be discussed in this case report.

Low grade mixofibrosarcoma in the mandible: A case report

Introduction: The term myxofibrosarcoma (MFS) and myxosarcoma refers to a neoplasm of connective tissue of fibroblastic origin, formerly called malignant fibrous histiocytoma. Objective: To describe the clinical characteristics and the surgical medical procedure carried out. Clinical case: 61-year-old male patient, with a history of relative previous health and a 2-year history with a lesion in the marginal gingiva. Bulging of both cortices and with an extension of the lower right to the left first premolar. Therefore, they are admitted for a better study and treatment. Conclusions: It is a relatively rare tumor, of large size and wide dissemination at the lymph node levels of both the right and left hemi necks, drawing attention to its anterior location, the dissemination to levels IV and V of both necks, making it necessary to modify the management of the neck in our patient.

Nationwide incidence of sarcomas and connective tissue tumors of intermediate malignancy over four years using an expert pathology review network.

Since 2010, nationwide networks of reference centers for sarcomas (RREPS/NETSARC/RESOS) collected and prospectively reviewed all cases of sarcomas and connective tumors of intermediate malignancy (TIM) in France. The nationwide incidence of sarcoma or TIM (2013-2016) was measured using the 2013 WHO classification and confirmed by a second independent review by expert pathologists. Simple clinical characteristics, yearly variations and correlation of incidence with published clinical trials are presented and analyzed. Over 150 different histological subtypes are reported from the 25172 patients with sarcomas (n = 18712, 74,3%) or TIM (n = 6460, 25.7%), with n = 5838, n = 6153, n = 6654, and n = 6527 yearly cases from 2013 to 2016. Over these 4 years, the yearly incidence of sarcomas and TIM was therefore 70.7 and 24.4 respectively, with a combined incidence of 95.1/106/year, higher than previously reported. GIST, liposarcoma, leiomyosarcomas, undifferentiated sarcomas represented 13%, 13%, 11% and 11% of tumors. Only GIST, as a single entity had a yearly incidence above 10/106/year. There were respectively 30, 64 and 66 different histological subtypes of sarcomas or TIM with an incidence ranging from 10 to 1/106, 1-0.1/106, or < 0.1/106/year respectively. The 2 latter incidence groups represented 21% of the patients with 130 histotypes. Published phase III and phase II clinical trials (p<10-6) are significantly higher with sarcomas subtypes with an incidence above 1/106 per. This nationwide registry of sarcoma patients, with exhaustive histology review by sarcoma experts, shows that the incidence of sarcoma and TIM is higher than reported, and that tumors with a very low incidence (1<106/year) are less likely to be included in clinical trials.

Efficacy and safety of apatinib for patients with advanced extremity desmoid fibromatosis: a retrospective study.

Desmoid fibromatosis (DF) is a locally aggressive connective-tissue tumor arising in deep soft tissues. Although multiple therapeutic modalities have been demonstrated effective for DF, there is no standard systemic treatment for progressive and recurrent DF. As a part of systemic treatment, tyrosine kinase inhibitors have shown promising activity against DF with tolerable toxicity profiles. Thus, the aim of this study was to investigate the efficacy and safety of apatinib, a novel multi-target angiogenesis inhibitor, in patients with DF. We retrospectively analyzed the medical records of patients with advanced extremity DF regularly treated with apatinib between October 2017 and January 2020 in our center. Apatinib was initially administered with a dose of 250mg daily and the dose was adjusted according to the toxicity. Tumor response was assessed by the Response Evaluation Criteria in Solid Tumors 1.1 criteria. The primary endpoint was progression-free survival (PFS); objective response rates and drug-related adverse events were also evaluated. A total of 22 (6 male, 16 female) patients with advanced extremity DF were included. The mean medication time was 17months. None of the patients reached a complete response, but ten (45.5%) patients achieved partial response, and 11 patients (50%) achieved stable disease. One (4.5%) patient developed progressive disease, and the 1-year PFS rate was 95.2%. The disease control rate was 95.4% (21/22) and the objective response rate was 45.5% (10/22). Meanwhile, 18 (81.8%) patients with a tumor shrinkage were accompanied by a decreased signal intensity of lesions in T2-weighted magnetic resonance imaging. The most frequent adverse events included hand-foot syndrome (n = 7, 31.8%), fatigue (n = 6, 27.2%), local pain (n = 4, 18.1%), diarrhea (n = 4, 18.1%). Apatinib is an effective and well-tolerated option for patients with advanced extremity DF. Indeed, further prospective, randomized studies with larger cases are required to fully explore the clinical utility of apatinib in DF.

Focal fibrous hyperplasia beneath the tongue – A rare case report

Focal fibrous hyperplasia is a slow growing connective tissue tumor that frequently occurs in different anatomic sites of the oral cavity. The present case report describes the occurrence of this lesion on the ventral surface of the tongue which is hitherto least reported intraoral site, in a 11 year old male patient, which was successfully excised.

Co-Incidence of Paraesophageal Hernia and Perforated Gastrointestinal Stromal Tumor: A Case Report on Rare Presentation of GIST

Introduction: The most common connective tissue neoplasm of the gastrointestinal tract is gastrointestinal stromal tumors (GISTs). Its presentations are usually abdominal pain and gastrointestinal bleeding. Case Presentation: We report a 31-year-old man with abdominal pain presented to our hospital with 10 days history of abdominal pain. After radiologic investigations, total distended stomach paraesophageal hernia and antral deformity were seen in the CT scan. Subsequently, the patient underwent surgery and after releasing the stomach, a large sealed perforated mass was seen in the antrum near the diaphragmatic hiatus and small localized abscess that subtotal gastrectomy and hernia repair was done. Pathologic examination revealed that the perforated mass is a GIST. Conclusions: We report a rare complication of gist that is perforation and the first report of simultaneous paraesophageal hernia and perforated gist.

Surgery Treatment Improved the Overall Survival Rate in Locoregional Myxoid Leiomyosarcoma than Other Myxosarcomas in the United States.

Myxosarcomas are rare malignant tumors of soft connective tissues, classified into various subtypes, including myxoid liposarcoma, myxoid chondrosarcoma, and myxoid leiomyosarcoma. In this study, we proposed to study the demographic, tumor characteristics, and overall survival rate and compared the treatment modalities between these cancers. Patient data collected based on locoregional metastasis presentation of the abovementioned tumors with a cutoff study of survival duration up to 10 years were obtained from the SEER database during 1975-2016. Our results indicated that elderly patients and females were more in locoregional myxoid leiomyosarcoma than myxoid liposarcoma and myxoid chondrosarcoma with locoregional metastasis. The white race represented the most patients who suffered from these cancers than other races. The heart is the primary site for the abovementioned cancers, in addition to the female genitals to the myxoid leiomyosarcoma. Myxoid liposarcoma and myxoid chondrosarcoma patients with locoregional metastasis were suffering from grade II, while locoregional myxoid leiomyosarcoma patients with blank grading were due to missed data. Surgery was the most common treatment modality in this study compared with radiotherapy and chemotherapy. Kaplan-Meier analysis showed a significant difference in survival time between the three subtypes by using histology, and myxoid leiomyosarcoma showed prolonged survival than others. Elderly, female, white, unknown grade, surgery, no radiation, and no chemotherapy variables were independent factors associated with overall survival among these cancers. Multivariate analysis also showed significant differences in overall survival between the three tumors by histology, and myxoid leiomyosarcoma was with a better prognosis than others. Multivariate analysis of locoregional myxoid leiomyosarcoma showed the statistical significance of black race, grade, and radiotherapy, indicating them as independent prognostic factors of locoregional myxoid leiomyosarcoma. We conclude that surgery was the primary treatment modality against these cancers than radiotherapy and chemotherapy. And the locoregional myxoid leiomyosarcomas showed a better prognosis and higher survival rate than locoregional myxoid liposarcoma and locoregional myxoid chondrosarcoma.

Cellular toxicology and mechanism of the response to silver-based nanoparticle exposure in Ewing’s sarcoma cells

Ewing’s sarcoma is the most aggressive connective tissue tumor, mainly affecting children and adolescents; the 5 year survival rate is only 50%. Current treatments have poor effectiveness, and more efficient treatments are being sought. Silver-based nanoparticles, such as silver chloride nanoparticles (AgCl-NPs) and silver/silver chloride (Ag/AgCl-NPs) nanoparticles, can be biologically produced and can release Ag+ ions into solution; however, their antitumor activity has been minimally investigated. The aim of this study was to evaluate the antitumor potential of AgCl-NPs and Ag/AgCl-NPs against Ewing’s sarcoma cells. A673 cells (Ewing’s sarcoma) were treated for 72 h with 0–12.5 μg ml−1 of Ag/AgCl-NPs or 0–40 μg ml−1 of AgCl-NPs. Human cells from the RPE-1 cell line (pigmented retinal epithelium) were used as a model of nontumor cells. The RPE-1 cells were less affected by the administration of AgCl-NPs or Ag/AgCl-NPs, with small reductions in the number of cells and viability and a small increase in apoptosis rates, while lysosomal damage, changes in reactive oxygen species (ROS) production, loss of mitochondrial membrane potential and alterations in microfilaments or cell areas were not observed. A673 tumor cells had significantly reduced number and viability levels when treated with AgCl-NPs, with reductions of 65.05% and 99.17%, respectively, whereas with Ag/AgCl-NP treatment, reductions of 65.53% and 92.51% were observed, respectively. When treated with silver-based nanoparticles, A673 cells also showed a significant increase in ROS production and loss of mitochondrial membrane potential, which culminated in an increase in the percentage of apoptosis among the population. Lysosomal damage was also observed when A673 cells were treated with the highest concentration of AgCl-NPs. In conclusion, the results showed that both AgCl-NPs and Ag/AgCl-NPs had some antitumor activity with minimal effects against healthy cells, which demonstrated the possibility of their use in cancer therapy.

Pediatric soft tissue tumors of head and neck – An update and review

Pediatric malignancies especially sarcomas are the most common and predominant cause of mortality in children. Such ongoing efforts are crucial to better understand the etiology of childhood cancers, get better the survival rate for malignancies with a poor prognosis, and maximize the quality of life for survivors. In this review article we authors aim to discuss relatively common benign and malignant connective tissue tumors (soft tissue tumor), focusing on current management strategies and new developments, as they relate to the role of the otolaryngologist– head and neck surgeon. Other rarer paediatric head and neck tumors beyond the scope of this review

PANCREATIC CANCER, CURRENT THERAPEUTIC APPROACHES AND POSSIBLE PROSPECTS

Prevalence of pancreatic cancer (PC) is not high in the population, but the aggressive nature of the disease leads to the fact that PC is one of the main causes of death in a group of patients with cancer. The prognosis for PC is significantly worse in the case of metastatic spread. It is proved that pancreatic adenocarcinoma from the very beginning is a systemic disease with early micrometastatic spread, so the question of effective drug treatment is extremely relevant. Chemotherapy is the basis for the treatment of patients with metastatic prostate cancer. However, despite numerous clinical studies using known cytostatic and targeted agents, progress in the treatment of this disease remains relatively modest compared to the progress made in the treatment of other types of tumors. The complexities of prostate cancer therapy are explained by the presence of a dense connective tissue tumor stroma, which is not just a barrier to tumor cells. It has a significant impact on various vital cellular processes, including tumor formation, invasion, metastasis, and contributes to the formation of drug resistance. Pancreatic cancer is heterogeneous in terms of molecular and biological characteristics. Many genetic changes, including germ lines and somatic mutations, contribute to the development of this disease. Recent studies have shown that each sample of prostate cancer includes an average of 63 genetic changes and 12 major signalling pathways. Further studies of tumor microenvironment markers and decoding the heterogeneity of the tumor genome in PC should become the basis for a “personalized” approach to treatment. It is likely 19 4'2020 ТОм 19 vol. 19 РОССИЙСКИЙ БИОТЕРАПЕВТИЧЕСКИЙ ЖУРНАЛ Russian journal of biotherapy Обзоры литературы that in the future, the integration of traditional chemotherapeutic treatments and an immunological approach will be the key to effective treatment of this deadly disease.

Elastofibroma dorsi: a rare connective tissue tumor

Elastofibroma dorsi: a rare connective tissue tumor

Perturbation-Theory Machine Learning (PTML) Multilabel Model of the ChEMBL Dataset of Preclinical Assays for Antisarcoma Compounds.

Sarcomas are a group of malignant neoplasms of connective tissue with a different etiology than carcinomas. The efforts to discover new drugs with antisarcoma activity have generated large datasets of multiple preclinical assays with different experimental conditions. For instance, the ChEMBL database contains outcomes of 37,919 different antisarcoma assays with 34,955 different chemical compounds. Furthermore, the experimental conditions reported in this dataset include 157 types of biological activity parameters, 36 drug targets, 43 cell lines, and 17 assay organisms. Considering this information, we propose combining perturbation theory (PT) principles with machine learning (ML) to develop a PTML model to predict antisarcoma compounds. PTML models use one function of reference that measures the probability of a drug being active under certain conditions (protein, cell line, organism, etc.). In this paper, we used a linear discriminant analysis and neural network to train and compare PT and non-PT models. All the explored models have an accuracy of 89.19–95.25% for training and 89.22–95.46% in validation sets. PTML-based strategies have similar accuracy but generate simplest models. Therefore, they may become a versatile tool for predicting antisarcoma compounds.

Elastofibroma Dorside Ga-68 DOATATE PET-BT İle Gösterilen Somatostatin Reseptör Ekspresyonu

Elastofibroma dorsi is a rare, benign, slowly-growing connective-tissue tumor that occurs most often in the infrascapular area in elderly people. It can be detected with various imaging modalities, like CT, MRI, USG and PET. Somatostatin is an endogenous hormone known by its inhibitory and neurotransmitter effects. Ga-68 DOTATATE which is a radiolabeled somatostatin analogue may show various uptake in physiological structures and also in tumor cells. Here in this case report, we describe the receptor expression of ED and present a case whose Ga-68 DOTATATE PET-CT scan revealed bilateral infrascapular soft tissue lesions, consistent with elastofibroma dorsi. According to our knowledge, this is the second case report in the literature, showing somatostatin receptor expression in elastofibroma dorsi

On the study of vaginal neoplasms

In view of the comparative rarity of connective tissue and muscle neoplasms of the vagina (the number of published cases does not exceed 70), the author reports the following two observations.

Extensive submandibular desmoid fibromatosis in a pediatric patient: A therapeutic challenge

Abstract Desmoid fibromatosis is a progressive benign connective tissue tumor, with a high tendency toward local invasiveness and recurrence. The tumor occurs mostly during childhood, involving the head and neck in 11–15% of the cases. Complete surgical excision with a safe margin is the standard treatment. While adjuvant non-surgical therapy is often considered for incomplete excision or recurrences. A 6-year-old female patient came with a rapidly growing non-painful mass of 6 months duration, involving lower jaw region on the left side. Examination revealed a significantly large and firm non-mobile mass involving the left mandibular and submandibular region, with limited mouth opening. The lesion appeared on imaging as an expansile heterogeneous mass, with evidence of destructive bony changes involving the mandible. Incisional biopsy initially performed followed by surgical excision with segmental resection of the mandible. Without adjuvant therapy, no recurrence observed during >2 years follow up period, and bone grafting subsequently performed. Head and neck pediatric fibromatosis is a rare and aggressive tumor, and its management often constitutes a major challenge to the clinicians. Surgical excision with preservation of cosmetic and functional demands and long-term follow up often result in good outcomes, and adjuvant therapy usually reserved for recurrence or gross residual lesions.