scholarly journals Rare Adult Subtype of Rhabdomyosarcoma, a Common Childhood Soft Tissue Carcinoma

2021 ◽  
Vol 9 ◽  
pp. 232470962110422
Author(s):  
Manasa Dondapati ◽  
Jonathan Vincent M. Reyes ◽  
Saad Ahmad ◽  
Aaron S. Stern ◽  
Joseph J. Lieber
Keyword(s):  
Soft Tissue ◽  
Treatment Guidelines ◽  
Pediatric Population ◽  
Adult Population ◽  
Survival Rates ◽  
Alveolar Rhabdomyosarcoma ◽  
High Rate ◽  
Childhood And Adolescence ◽  
Advanced Stages ◽  
Poorly Differentiated

Rhabdomyosarcoma is a malignant soft tissue sarcoma of primitive mesenchymal cells, showing varying degrees of striated skeletal muscle cell differentiation. It is a very common cancer of childhood and adolescence, but rarely seen in the adult population. Here, we present a case of a 33-year-old male presented with a poorly differentiated desmin positive alveolar rhabdomyosarcoma in the left arm. The prognosis of alveolar rhabdomyosarcoma in adults is very poor, frequently detected at advanced stages or with metastases. The alveolar subtype in particular has been found to have a more aggressive course with a high rate of metastasis. Recent studies have shown that using pediatric treatment guidelines resulted in better survival outcomes and local control, but the survival rates are still below that of the pediatric population. Newer studies are looking into using specific molecular markers for more targeted therapy in hopes of further improving survival rates in the adult population.

The pathological spectrum of solid CNS metastases in the pediatric population

2014 ◽  
Vol 14 (2) ◽  
pp. 129-135 ◽  
Author(s):  
Andrea L. Wiens ◽  
Eyas M. Hattab
Keyword(s):  
Soft Tissue ◽  
Pediatric Population ◽  
Survival Rates ◽  
Cns Tumors ◽  
Limited Information ◽  
Cns Metastases ◽  
Metastatic Tumors ◽  
Cns Metastasis ◽  
Malignant Diagnosis ◽  
Pediatric Cns Tumors

Object Collectively, metastatic tumors are the most common malignancy encountered in the adult central nervous system (CNS), arising most often from lung, breast, skin, and gastrointestinal tract carcinomas. Limited information is available in the literature regarding solid nonhematopoietic CNS metastases in children. The authors carried out a retrospective study of pediatric metastatic neoplasms to the CNS treated in a 30-year period to characterize their frequency, common histological subtypes, and sites of origin. Methods The archival pathology files were searched (1981–2011) for metastatic tumors to the CNS in patients 21 years of age and younger. Pathology material was reviewed, tumors were classified by site of origin and histological subtype, and survival was evaluated. Results The authors identified 26 patients with solid nonhematopoietic CNS metastases out of 1135 pediatric CNS tumors diagnosed from 1981 to 2011. Patients ranged in age from 1.5 to 20.3 years and were equally divided between sexes. Most CNS metastases were supratentorial (85%) and solitary (65%). The mean interval from primary malignant diagnosis to CNS metastasis was 27 months. Sites of origin included kidney/adrenal, bone/soft tissue, gonads, head and neck, lung, and liver. Mean survival after CNS involvement was 36.6 months. Overall 1-year and 5-year survival rates were 52% and 16%, respectively. Conclusions In neuropathology practice, nonhematopoietic pediatric CNS metastases are far less common than are nonhematopoietic adult CNS metastases, accounting for approximately 2% of all pediatric CNS tumors. The most common tumors to exhibit CNS metastasis are of kidney/adrenal origin, followed by those from bone/soft tissue. As expected, prognosis is dismal, despite aggressive therapy.

Retrospective Analysis of the Survivability of Absorbable Versus Nonabsorbable Subtalar Joint Arthroereisis Implants

2012 ◽  
Vol 6 (1) ◽  
pp. 36-44 ◽  
Author(s):  
Jeffrey R. Baker ◽  
Erin E. Klein ◽  
Lowell Weil ◽  
Lowell Scott Weil ◽  
Jessica M. Knight
Keyword(s):  
Overall Survival ◽  
Subtalar Joint ◽  
Pediatric Population ◽  
Adult Population ◽  
Survival Rates ◽  
Level Of Evidence ◽  
Absorbable Implants ◽  
Combined Procedures ◽  
Pediatric Populations ◽  
Overall Survival Rates

Subtalar joint arthroereisis is a surgical modality that has been shown to be an effective procedure for flexible flatfoot in both pediatric and adult populations. Despite advances in understanding its mechanics and function, complication and implant removal rates remain as high as 30% to 40%. Analysis was performed to determine the survivability of 2 subtalar joint arthroereisis implants, absorbable and nonabsorbable, used alone and in combination with other procedures in both the adult and pediatric populations. The 95 total arthroereisis procedures were analyzed in several major categories: absorbable implants versus nonabsorbable implants and adult versus pediatric patients. Each major group was then further subdivided to create further subgroups: absorbable isolated procedures, absorbable combined procedures, nonabsorbable isolated procedures, and nonabsorbable combined procedures. The overall survival rates were 83% for absorbable implants and 81% for nonabsorbable implants. A total of 11 (17%) absorbable implants and 6 (19%) nonabsorbable implants were removed, respectively, at an average of 9 months and 23 months postoperatively. When used alone and in combination with other procedures, 36% and 13% of absorbable implants and 18% and 19% of nonabsorbable implants, respectively, were removed. When comparing adult versus pediatric populations, the overall survival rates of the absorbable and nonabsorbable implants were 81% for absorbable implants and 79% for nonabsorbable implants in the adult population and 85% for absorbable implants and 100% for nonabsorbable implants in the pediatric population. Level of Evidence: Therapeutic, Level III; Retrospective comparative series

scholarly journals Survival Analysis in Pediatric Osteosarcoma

2019 ◽  
Vol 13 (2) ◽  
pp. 21
Author(s):  
Mururul Aisyi ◽  
Ayu Hutami Syarif ◽  
Nur Asih Anggraeni ◽  
Adhitya Bayu Perdana ◽  
Hutomo Rezky ◽  
...  
Keyword(s):  
Overall Survival ◽  
Survival Probability ◽  
Pediatric Population ◽  
Prognostic Significance ◽  
Childhood And Adolescence ◽  
Cancer Awareness ◽  
Therapeutic Modalities ◽  
Patient Variables ◽  
Advanced Stages ◽  
Pediatric Osteosarcoma

Background: Osteosarcoma is the most common bone malignancy in childhood and adolescence. Despite significant advances in diagnostic and therapeutic modalities, osteosarcoma has generally poor prognosis. Several studies highlighted the prognostic significance of demographic and clinical parameters in the pediatric population. Controversy exists about which obvious factors of mortality in some institutions. Meanwhile, little is known about the overall survival (OS) of pediatric osteosarcoma in Dharmais Cancer Hospital.The aim of this study is to estimate the OS in pediatric osteosarcoma.Methods: This retrospective cohort study enrolled 41 children with osteosarcoma during the period of January 2010-September 2017. Patients were selected using inclusion and exclusion criteria. Overall survival (OS) and patient variables were plotted using Kaplan-Meier.Results: Our result showed that all patients admitted to our hospital in advanced stages (IIB and III). Their median OS was 23 months (12.3-33.7 months) with the survival probability was 29.4%.Conclusions: Median OS of pediatric osteosarcoma in our institution was 23 months and the survival probability was 29.4%. It can be used as evidence showing lack of cancer awareness and early detection in our country.

Assessment of viable cells as a predictor of response in soft tissue sarcomas treated with neoadjuvant therapy.

2013 ◽  
Vol 31 (15_suppl) ◽  
pp. e21507-e21507
Author(s):  
Ranyell Spencer Sobreira Batista ◽  
Samuel Aguiar ◽  
Isabela Werneck Cunha ◽  
Ademar Lopes ◽  
Fernando A. Soares ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Tumor Necrosis ◽  
Survival Rates ◽  
Soft Tissue Sarcomas ◽  
Disease Free Survival ◽  
Viable Cell ◽  
High Rate ◽  
High Grade ◽  
Response Parameter ◽  
Viable Cells

e21507 Background: Soft Tissue Sarcomas (STS) are an uncommon neoplasm with many histological subtypes with different behaviors, response to treatments and developments. The treatment of these tumors is using surgery, radiotherapy or chemotherapy. The pathological response using degree of tumor necrosis has been implicated in survival rates. However comes to assessing the degree of tumor necrosis in the surgical specimens as response parameter could be confuse because the high-grade sarcomas exhibit a high rate of necrosis even without any treatment. Methods: 31 patients with STS of high grade located in extremities treated with neoadjuvant doxorubicin and ifosfamide were observed prospectively since January 2005 to June 2011. For evaluation of surgical specimens post neoadjuvant chemotherapy, a slice of the specimen in with lower gross of necrosis was represented and after mapped evaluating grade of necrosis, fibrosis and percentage of viable cells. Results: Six patients had ≤5% of viable cells, eight had ≤10% and 12 with 30% or less in the sample. Overall survival (OS) was 100%, 100% and 91% with Disease free survival (DFS) were 100%, 85.7% and 82.5%, respectively. No patient had ≥95% necrosis in the surgical specimens considering that a large part of the tissue had fibrosis in patients with few viable cells, which do not correlate with survival. Conclusions: The viable cell count in the sample is feasible, and relates well with survival. We consider this the ideal method for response evaluation in SPM instead of counting necrosis. In combination with the literature data we suggest cutoff points of 5%, 10% and 30%.

Results of Treatment for Soft Tissue Sarcoma in Childhood and Adolescence: A Final Report of the German Cooperative Soft Tissue Sarcoma Study CWS-86

1999 ◽  
Vol 17 (12) ◽  
pp. 3706-3719 ◽  
Author(s):  
Ewa Koscielniak ◽  
Dieter Harms ◽  
Günter Henze ◽  
Herbert Jürgens ◽  
Helmut Gadner ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Survival Rates ◽  
Local Therapy ◽  
Stage Iv ◽  
Staging System ◽  
Stage Iii ◽  
Final Report ◽  
Childhood And Adolescence ◽  
Primary Tumor Site

PURPOSE: The goal of the second German Soft Tissue Sarcoma Study CWS-86 (1985 to 1990) was to improve the prognosis in children and adolescents with soft tissue sarcoma by means of a clinical trial comprising intensive chemotherapy and risk-adapted local therapy. PATIENTS AND METHODS: There were 372 eligible patients. A staging system based on the postsurgical extent of disease was used. Chemotherapy consisted of vincristine, dactinomycin, doxorubicin, and ifosfamide. Radiotherapy was administered early at 10 to 13 weeks simultaneously with the second chemotherapy cycle (32 Gy or 54.4 Gy). The single dose was reduced to 1.6 Gy and given twice daily (accelerated hyperfractionation). RESULTS: The event-free survival (EFS) and overall survival rates at 5 years were 59% ± 3% and 69% ± 3%, respectively. The 5-year EFS rate according to stage was as follows: stage I, 83% ± 5%; stage II, 69% ± 6%; stage III, 57% ± 4%; and stage IV, 19% ± 6%. The outcome for patients with stage III disease who required radiotherapy was much better in the CWS-86 study compared with the CWS-81 study (5-year EFS, 60% ± 5% v 44% ± 6%; P = .053). The most common treatment failure was isolated local relapse, with 14% of patients relapsing at the primary tumor site. CONCLUSION: The improved design of the study incorporating risk-adapted radiotherapy allowed treatment to be reduced for selected groups of patients without compromising survival.

scholarly journals Genetic Landscape of Papillary Thyroid Carcinoma and Nuclear Architecture: An Overview Comparing Pediatric and Adult Populations

Cancers ◽  
2020 ◽  
Vol 12 (11) ◽  
pp. 3146
Author(s):  
Aline Rangel-Pozzo ◽  
Luiza Sisdelli ◽  
Maria Isabel V. Cordioli ◽  
Fernanda Vaisman ◽  
Paola Caria ◽  
...  
Keyword(s):  
Thyroid Cancer ◽  
Papillary Thyroid Carcinoma ◽  
Thyroid Carcinoma ◽  
Pediatric Population ◽  
Adult Population ◽  
Nuclear Architecture ◽  
High Rate ◽  
Papillary Thyroid ◽  
Molecular Features ◽  
Cancer Pathogenesis

Thyroid cancer is a rare malignancy in the pediatric population that is highly associated with disease aggressiveness and advanced disease stages when compared to adult population. The biological and molecular features underlying pediatric and adult thyroid cancer pathogenesis could be responsible for differences in the clinical presentation and prognosis. Despite this, the clinical assessment and treatments used in pediatric thyroid cancer are the same as those implemented for adults and specific personalized target treatments are not used in clinical practice. In this review, we focus on papillary thyroid carcinoma (PTC), which represents 80–90% of all differentiated thyroid carcinomas. PTC has a high rate of gene fusions and mutations, which can influence the histologic subtypes in both children and adults. This review also highlights telomere-related genomic instability and changes in nuclear organization as novel biomarkers for thyroid cancers.

Persistence of giants: population dynamics of the limpet Scutellastra laticostata on rocky shores in Western Australia

2020 ◽  
Vol 646 ◽  
pp. 79-92
Author(s):  
RE Scheibling ◽  
R Black
Keyword(s):  
Population Dynamics ◽  
Western Australia ◽  
Size Structure ◽  
Survival Rates ◽  
Maximum Size ◽  
High Rate ◽  
Adult Survival ◽  
Rocky Shores ◽  
Adult Size ◽  
Decadal Time Scale

Population dynamics and life history traits of the ‘giant’ limpet Scutellastra laticostata on intertidal limestone platforms at Rottnest Island, Western Australia, were recorded by interannual (January/February) monitoring of limpet density and size structure, and relocation of marked individuals, at 3 locations over periods of 13-16 yr between 1993 and 2020. Limpet densities ranged from 4 to 9 ind. m-2 on wave-swept seaward margins of platforms at 2 locations and on a rocky notch at the landward margin of the platform at a third. Juvenile recruits (25-55 mm shell length) were present each year, usually at low densities (<1 m-2), but localized pulses of recruitment occurred in some years. Annual survival rates of marked limpets varied among sites and cohorts, ranging from 0.42 yr-1 at the notch to 0.79 and 0.87 yr-1 on the platforms. A mass mortality of limpets on the platforms occurred in 2003, likely mediated by thermal stress during daytime low tides, coincident with high air temperatures and calm seas. Juveniles grew rapidly to adult size within 2 yr. Asymptotic size (L∞, von Bertalanffy growth model) ranged from 89 to 97 mm, and maximum size from 100 to 113 mm, on platforms. Growth rate and maximum size were lower on the notch. Our empirical observations and simulation models suggest that these populations are relatively stable on a decadal time scale. The frequency and magnitude of recruitment pulses and high rate of adult survival provide considerable inertia, enabling persistence of these populations in the face of sporadic climatic extremes.

LOCAL RECURRENCES AFTER THE TREATMENT OF SOFT TISSUE MALIGNANT FIBROUS HISTIOCYTOMA (UNCLASSIFIEDPLEOMORPHIC SARCOMA) OF THE LIMBS

2019 ◽  
Vol 72 (8) ◽  
pp. 1523-1526
Author(s):  
Oleksandr O. Lytvynenko ◽  
Volodymyr F. Konovalenko ◽  
Anton Yu. Ryzhov
Keyword(s):  
Soft Tissue ◽  
Malignant Fibrous Histiocytoma ◽  
Fibrous Histiocytoma ◽  
Survival Rates ◽  
Soft Tissue Sarcomas ◽  
Primary Treatment ◽  
Radical Excision ◽  
Local Recurrences ◽  
Results Of Treatment

Introduction: The treatment of patients with malignant fibrous histiocytoma as well as other soft tissue sarcomas is not sufficiently effective up to date, and has largely changed and reflects the alterations, occurred in oncology as a whole. The number of amputation decreased over the last 10-15 years. Some researchers associate the improvement of treatment outcomes with the development of combined and complex methods. The aim of the study is an improvement of the results of treatment of patients with soft tissue malignant histiocytoma on the basis of determination of factors, influencing local recurrence development. Materials and methods: The basis of our study was a comprehensive analysis of examination and treatment results of 130 patients with MFH of the soft tissue of limbs, of them in 84 patients (64.6%) the recurrences developed. The group included 45 (53.6%) males and 39 (46.4%) females. The major part of patients – 82.1% (60 patients) were older than 40 years. Results and conclusions: The number of recurrences after the treatment in general surgical facilities is 86.9%, whereas in the patients after the treatment in the specialized oncological facilities this figure is twice lower (40%). The characteristic of the medical facility where the patient receives his/her primary treatment largely affects the development of local recurrences, patients’ quality of life and overall survival rates. The surgical method remains the leading modality in the treatment of MFH of ST. Wide and radical excision of tumors in the specialized oncological facilities allows achieving better survival outcomes of the patients.

Myxofibrosarcoma: clinical and prognostic value of MRI features

2020 ◽  
Vol 16 ◽  
Author(s):  
Paolo Spinnato ◽  
Andrea Sambri ◽  
Tomohiro Fujiwara ◽  
Luca Ceccarelli ◽  
Roberta Clinca ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Local Recurrence ◽  
Contrast Enhancement ◽  
Imaging Modality ◽  
Soft Tissue Sarcomas ◽  
The Elderly ◽  
High Rate ◽  
Imaging Features ◽  
High Grade ◽  
Mri Features

: Myxofibrosarcoma is one of the most common soft tissue sarcomas in the elderly. It is characterized by an extremely high rate of local recurrence, higher than other soft tissue tumors, and a relatively low risk of distant metastases.Magnetic resonance imaging (MRI) is the imaging modality of choice for the assessment of myxofibrosarcoma and plays a key role in the preoperative setting of these patients.MRI features associated with high risk of local recurrence are: high myxoid matrix content (water-like appearance of the lesions), high grade of contrast enhancement, presence of an infiltrative pattern (“tail sign”). On the other hand, MRI features associated with worse sarcoma specific survival are: large size of the lesion, deep location, high grade of contrast enhancement. Recognizing the above-mentioned imaging features of myxofibrosarcoma may be helpful to stratify the risk for local recurrence and disease-specific survival. Moreover, the surgical planning should be adjusted according to the MRI features

scholarly journals Kaposiform Hemangioendothelioma: clinicopathological characteristics of 8 cases of a rare vascular tumor and review of literature

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Qurratulain Chundriger ◽  
Muhammad Usman Tariq ◽  
Jamshid Abdul-Ghafar ◽  
Arsalan Ahmed ◽  
Nasir Ud Din
Keyword(s):  
Pediatric Population ◽  
Adult Population ◽  
Correct Diagnosis ◽  
Kaposi Sarcoma ◽  
Minor Component ◽  
Vascular Tumor ◽  
Giant Cells ◽  
Clinicopathological Characteristics ◽  
Multinucleated Giant Cells ◽  
Kaposiform Hemangioendothelioma

Abstract Background Kaposiform Hemangioendothelioma (KHE) is a rare vascular tumor of intermediate malignant potential which shows locally aggressive growth but only rarely metastasizes. It is mostly considered to be a tumor of pediatric population but its occurrence in the adults is not uncommon as once considered. Histologically, KHE can mimic other soft tissue neoplasms of different behaviors (e.g. Kaposi Sarcoma, hemangioma) and establishing the correct diagnosis is important for appropriate treatment. Herein, we describe the clinicopathological features of 8 cases of KHE which will be helpful in making their diagnosis. Methods We reviewed pathology reports, microscopy glass slides and obtained follow up information about 8 cases of KHE which were diagnosed at our institution from January 2008 till June 2020. Immunohistochemical stain for HHV8 was also performed. Results Age ranged from 7 months to 25 years. Seven patients were less than 20 years of age and one patient was 25 years old. Equal gender distribution was observed. Extremities were the most common sites of involvement, followed by head and neck, pancreas and ischiorectal region. 2 cases were resection specimen and all others were incisional biopsies. The largest tumor size was 5.5 cm in one of the resections. The incisional/fragmented tissues were all less than 5 cm in aggregate. Most cases showed predominance of nodular growth and a minor component of spindle cell population along with lymphangiomatosis like vascular channels, with evidence of microthrombi in 2 cases. Few multinucleated giant cells were observed in 2 cases. None of the cases exhibited significant nuclear atypia or mitotic activity. One of the cases arising in dermis showed underlying bone involvement. HHV8 was negative in 7/7 cases. Conclusions KHE can also involve adult population and it should always be considered in the differential diagnoses of a vascular lesion. Presence of multinucleated giant cells is a rare finding. Knowledge about histological features and potential mimics is helpful in avoiding misdiagnosis.

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