[Analysis of ultrasound images features and diagnostic model establishment of alveolar soft part sarcoma and intramuscular capillary-type hemangiomas].

Objective: The ultrasonography features of alveolar soft part sarcoma (ASPS) and intramuscular capillary-type hemangiomas (ICTH) were analyzed, and the diagnostic model of ASPS was established. Methods: A cross-sectional study was carried out. The clinical data of 52 patients [28 males and 24 females, aged (20.7±15.1) years] with pathologically confirmed ASPS and ICTH admitted to People's Hospital of Henan Province from January 2005 to February 2023 were included in the study. According to pathological types, the patients were divided into ASPS group and ICTH group. Clinical data of patients were retrospectively collected, and meaningful indicators in the univariate analysis were included in the regression analysis for screening. After comprehensive consideration of clinical significance and statistical significance, eligible indicators were selected for inclusion in the regression analysis. Binary logistic regression analysis was used to screen the factors that distinguished the pathological types of ASPS and ICTH, and the diagnostic model was established. The area under receiver operating characteristic (ROC) curve (AUC) was used to evaluate the diagnostic effectiveness of the diagnostic model in distinguishing ASPS from ICTH. Results: There were 20 patients in ASPS group, 10 males and 10 females, aged (26.9±13.5) years, and 32 patients in ICTH group, 18 males and 14 females, aged (16.8±15.0) years. The age difference between the ASPS group and the ICTH group was statistically significant (P<0.05), and there were statistically significant differences in the ultrasound imaging features of "clear boundary" "peripheral lobe" "thin blood vessels inside the lesion are straight and out of shape" "intra-lesion liquification" "peripheral thick blood vessels" and "peripheral muscle fiber disruption" between the two groups (all P<0.001).Variables with clinical and statistical significance were selected as independent variables. Binary logistic regression analysis showed that peripheral muscle fiber interruption (OR=97.358, 95%CI:6.833-1 387.249) and internal thin blood vessels were flat and out of shape (OR=0.052, 95%CI:0.003-0.921) was the correlation factor to distinguish the pathological types of ASPS and ICTH. Two ultrasonic image features of "peripheral muscle fiber interruption" and "internal thin blood vessels are straight and out of shape" were used to establish the diagnostic model. The sensitivity of "peripheral muscle fiber interruption" diagnostic model was 81.3%, and the specificity was 95.0%. The AUC was 0.811(95%CI: 0.761-0.954). The sensitivity, specificity and AUC of the diagnosis model of "internal thin vessels with flat misshape" were 90.0%, 96.9% and 0.934(95%CI: 0.830-0.984). The sensitivity, specificity and AUC of the combined diagnosis model of "peripheral muscle fiber interruption" and "internal thin blood vessel straight out of shape" were 96.9%, 90.0% and 0.974(95%CI:0.877-0.999). Conclusion: Ultrasonography can be used to distinguish ASPS from ICTH, and the combined diagnostic model based on the two ultrasonic imaging features of "peripheral muscle fiber interruption" and "internal thin blood vessel straight out of shape" can further improve the diagnostic efficiency.

Severe immediate hypersensitivity to gadolinium contrast agent after targeted treatment in a patient with alveolar soft part sarcoma: A case report and review of literature.

RATIONALE: Gadolinium-based contrast agents (GBCAs), benefiting from good tolerance and safety, become the priority contrast agents in magnetic resonance imaging. Serious hypersensitivity reactions caused by GBCAs are rare, but occur occasionally. The "immune surveillance" theory proposes that lowered immune function exists in patients with malignance, which decrease the occurrence of atopy. Natural immunosurveillance that enhanced by effective treatment of malignance may increase the risk of hypersensitivity. PATIENT CONCERNS: A 29-year-old female patient suffering from intensive pain with left leg mass was admitted in our hospital. DIAGNOSES: The patient was diagnosed with alveolar soft part sarcoma by histopathology and revealed destruction of the left fibula and lung metastasis by computed tomography scan, and treated with anlotinib hydrochloride, a multi-targeted tyrosine kinase inhibitor. After 4 cycles of effective targeted therapy, the patient developed severe immediate hypersensitivity due to gadopentetate dimeglumine-enhanced magnetic resonance imaging. INTERVENTIONS AND OUTCOMES: The vital signs of the patient returned to normal after rescue. Since then, the patient has not used gadolinium contrast agent again, and currently the condition is stable and still alive. LESSONS: Severe immediate hypersensitivity might be occurred by gadolinium contrast agent in patients with malignance after effective treatment. We explored the potential mechanism of GBCA-inducing hypersensitivity in detail, by especially focusing on the changes of immune environment. Furthermore, we propose new ideas for the safe use of GBCAs in patients with malignancies.

Treatment of a case of primary alveolar soft part sarcoma of the lung: Case report and literature review.

Alveolar soft part sarcoma (ASPS) is an extremely rare type of soft tissue sarcoma. The primary sites of ASPS are mostly located in the extremities and trunk. Primary pulmonary ASPS is extremely rare. A search of the PubMed® database identified only five cases of primary pulmonary ASPS. This current case report describes the sixth case of ASPS in a 15-year-old male that presented with recurrent headaches. Head computed tomography showed space-occupying lesions in the left parietal lobe. Positron emission tomography-computed tomography confirmed the space-occupying lesions in the left parietal lobe and showed multiple nodules and masses in the two lungs and pleura, which were considered to be low-grade malignant mesenchymal tumours. The case report presents the clinical characteristics, diagnosis and treatment process. Programmed cell death protein 1 monoclonal antibody (sintilimab) combined with a tyrosine kinase inhibitor (anlotinib hydrochloride) achieved a good therapeutic effect, indicating that this combination therapy is worth exploring further. Large-scale prospective studies are needed to explore and develop standardized treatments for ASPS.

Primary intracranial alveolar soft part sarcoma in a 22 years old female: a rare case report with literature review.

Alveolar soft part sarcoma is a very rare malignant neoplasm of peripheral muscular, adipose or neural tissue. Its occurrence, as a primary intracranial tumour, is even rarer. To the best of our knowledge, only nine cases of primary intracranial alveolar soft part sarcoma exist in English scientific literature. We attempt to write a comprehensive review on this poorly understood intracranial malignancy, with no evident systemic lesions, such as in the case of our 22 years old patient. In addition to absent definitive proof of benefit of radiologic or chemotherapeutic management, we highlight the role of surgery as the primary treatment. Younger patients with this tumour may have a worse prognosis than elderly patients.

Alveolar Soft Part Sarcoma of Hypopharynx; A Case Report.

Alveolar soft part sarcoma (ASPS) is an aggressive soft-tissue malignancy, notorious for its metastasis to other tissues. A considerable number of cases in the head and neck have been reported but not in the hypopharynx. We describe a 31-year-old man with an incidental finding of a hypopharyngeal mass. Flexible laryngoscopy revealed a fleshy mass 2 × 2 cm2 originating from the left hypopharynx and overlying the epiglottis. Computed tomography scan demonstrated a soft tissue mass in the left wall of the oropharynx measuring about 2.2 × 1.8 cm2, projecting into the hypopharyngeal air space. Magnetic resonance imaging showed a significant thickening of the left hypopharyngeal wall forming a mass lesion occupying the left pyriform sinus and abutting the left aryepiglottic fold. Histopathology indicated that tumor cells were polygonal and epithelioid, with abundant eosinophilic to clear flocculent cytoplasm, eccentric nuclei, and prominent nucleoli. The tumor was positive for smooth muscle actin with rare cells staining for Human Melanoma Black (HMB45). Fluorescence in situ hybridization for transcription factor E3 was also performed and supported the above diagnosis. Our study reports the first case of ASPS in the hypopharynx.

Primary intraosseous alveolar soft part sarcoma: Report of two cases with radiologic-pathologic correlation.

Alveolar soft part sarcoma (ASPS) accounts for less than 1 % of all soft tissue sarcomas. ASPS presents a poor prognosis and develops frequent metastases, especially in the lungs, brain and bones. Current therapies, such as surgery, radiotherapy and chemotherapy, are not fully effective and other alternative treatments are currently being studied. ASPS is predominantly found in the deep soft tissues of the lower extremities. To our knowledge, only thirteen primary intraosseous ASPS have been reported in the literature. In this study, we report two new cases of this exceedingly rare entity. Both cases already had multiple metastases since diagnosis; one of them represents the first case of a primary bone ASPS in the ulna and is also the primary intraosseous ASPS with the longest reported case of survival, after having maintained long periods of stabilization despite not having received any systemic treatment.

Sonographic imaging features of alveolar soft part sarcoma: Case series and literature review.

BACKGROUND: Alveolar soft part sarcoma (ASPS) is a rare tumor but potentially fatal condition. Understanding the imaging and clinical features of ASPS is of certain value for preoperative qualitative diagnosis and clinical treatment of tumors. Nevertheless, there have been only 11 documented case reports describing the sonographic features in the English literature. METHODS: Three patients with confirmed ASPS occurring primarily in the limbs were enrolled in this study. Complete surgical excision was performed with conservative limb function. We pay particular attention to the ultrasonographic features and performed a literature review of ASPS cases. RESULTS: With regular surveillance, one patient had no symptom recurrence and two developed lung and/or breast metastasis later. The specific sonographic findings were heterogeneous hypoechoic, well-circumscribed, and lobulated or round contours on grayscale images, abundant flow signals of intratumoral and extratumoral tubular structures on color Doppler images. CONCLUSION SUBSECTIONS: Its low incidence rate and lack of characteristic clinical manifestations often result in misdiagnosis of ASPS. The specific sonographic findings may add useful diagnostic information.

Alveolar soft part sarcoma of the tongue. A case report of a rare entity / Sarcoma alveolar de partes blandas de la lengua. Informe de un caso de una entidad rara

Alveolar soft part sarcoma (ASPS) is a distinct soft tissue sarcoma among adolescent and young individuals. It accounts for 1 % of all sarcomas. It is a rare malignancy of the mesenchymal tissue that possesses an uncertain histologic origin. The increased vascular nature of this lesion leads to the dissemination of tumour cells through the connective tissue along the haematogenous route. Here we present a case of a swelling involving the tongue of a 19-year-old male patient and emphasize on the importance of diagnostic aids and management of this rare and unique lesion. (AU)

El sarcoma alveolar de partes blandas (ASPS, por sus siglas en inglés) es un sarcoma de partes blandas distinto entre los adolescentes y los jóvenes. Representa el 1 % de todos los sarcomas. Es una rara neoplasia maligna del tejido mesenquimatoso que posee un origen histológico incierto. El aumento de la vascularización de esta lesión conduce a la diseminación de células tumorales a través del tejido conjuntivo por vía hematógena. Aquí presentamos un caso de tumefacción en la lengua de un paciente masculino de 19 años y enfatizamos la importancia de las ayudas diagnósticas y el manejo de esta rara y única lesión. (AU)

Microsurgical Management of Early Onset Alveolar Soft Part Sarcoma of the Oral Tongue: Case Report and Review of the Literature.

OBJECTIVES: Alveolar soft part sarcoma is a rare subset of soft tissue sarcomas, typically presenting in subjects 15 to 35 years of age. Usual presentation sites are the trunk, extremities, and the head and neck. Subjects younger than 5 years are rarely affected. METHODS: In this retrospective case report, we present a 16-month old male with a rapidly growing soft tissue mass of the anterior and posterior tongue, found to be alveolar soft part sarcoma. RESULTS: The subject was treated with primary surgical resection and the resulting defect was reconstructed with a radial forearm free flap. CONCLUSIONS: To our knowledge, this is the youngest subject to have been diagnosed with alveolar soft part sarcoma. Surgical extirpation and microvascular reconstruction were successful, and the patient remains disease free 4 years post-operatively.

Alveolar soft part sarcoma of the retroperitoneum: A case report.

Alveolar soft part sarcoma is a rare soft tissue tumor with uncertain histogenesis. It is a slow growing tumor with a high rate of metastasis. The tumor is not easily identified as clinical symptoms are not pronounced. The retroperitoneum is a rare location of tumor, with a few cases published in literature. Surgical excision is the mainstay of treatment. Here we describe a rare case of a large retroperitoneal Alveolar soft part sarcoma in a young female with radiological and histopathological findings.

[A PRIMARY BLADDER ALVEOLAR SOFT PART SARCOMA: A CASE REPORT].

A 32-year-old woman was admitted to our department for hematuria and dysuria.Computed tomography (CT) and cystoscopy revealed a 2-cm pedunculated tumor with rich blood supply and a smooth surface in the bladder trigone. We performed a transurethral resection of bladder tumor. The pathologic diagnosis was alveolar soft part sarcoma (ASPS). CT, bone scintigraphy, positron emission tomography, and pelvic magnetic resonance imaging revealed no other lesions; thus, she was diagnosed as having a primary bladder ASPS. Postoperative follow-up with regular cystoscopies and CTs over 10 years have shown no local recurrence or metastasis.Primary ASPS of the bladder is exceedingly rare, and this case is the 8th case (the 2nd case in Japan) reported in literature.

iCREATE: imaging features of primary and metastatic alveolar soft part sarcoma from the EORTC CREATE study.

BACKGROUND: Alveolar Soft Part Sarcoma (ASPS) is a rare, slow-growing, but highly vascular soft tissue sarcoma, characterised by a high rate of metastases at presentation. Although imaging features of the primary are well described, less detail is available on the imaging pattern of metastatic ASPS. The EORTC 90101 (CREATE) study assessed the efficacy of Crizotinib in patients with metastatic ASPS and presents a unique opportunity to describe the imaging phenotype of primary and metastatic ASPS, based on prospectively collected imaging. METHODS: A retrospective review of the staging CT scans of 32 patients with ASPS from the CREATE study was undertaken and the imaging features of primary and metastatic disease were assessed. RESULTS: Imaging of the primary tumour was available in 7/32 cases (28%). All primary tumours demonstrated marked vascularity with prominent feeding vessels (7/7, 100%). The most frequent sites of metastases included lung (30/32, 94%), nodal (7/32, 22%), bone (5/32, 16%) and muscle/subcutaneous (5/32, 16%). Features of hypervascularity were identified at all sites, more appreciable in the lungs, with feeding vessels frequently demonstrated in pulmonary metastases (21/32, 66%). CONCLUSION: Analysis of imaging from the CREATE cohort of patients with metastatic ASPS demonstrates that metastases from ASPS are predominantly hypervascular and demonstrate feeding vessels comparable to primary ASPS, suggesting potential sensitivity of this rare sarcoma for antivascular/antiangiogenic treatment approaches.

Sarcoma alveolar pulmonar primario. Un caso poco habitual / Primary pulmonary alveolar sarcoma. An unusual case

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Parapharyngeal Alveolar Soft Part Sarcoma in a 5-Year-Old Child.

Alveolar soft part sarcoma (ASPS) is a rare malignancy of head and neck region; orbit and tongue being most common subsites affected by this rare tumor. A 5-year-old female presented with computed tomography scan evidence of right-sided prestyloid parapharyngeal mass. Complete excision was performed through trans-cervical approach. Histopathology, special stain, and immunohistochemistry of the resected specimen confirmed the diagnosis of ASPS. Three-year follow-up did not reveal any local recurrence or distant metastases. Review of the English-language scientific literature did not reveal any documented case of parapharyngeal space ASPS in pediatric age. Only 1 clinical report of ASPS in a 42-year-old female has been documented.

Alveolar soft part sarcoma metastatic to the breast: a case report.

BACKGROUND: Alveolar soft part sarcoma (ASPS) is an extremely rare neoplasm that tends to occur in the lower limbs of children and adolescents. Metastatic breast tumors constitute 0.5-2.0% of all malignant mammary neoplasms, and cases of ASPS with mammary metastases are very rare. CASE PRESENTATION: Three years ago, an 11-year-old girl presented to the hospital with pain in the right jaw after becoming aware of a mass in the right cheek. After detailed examination, the patient was diagnosed with ASPS with the primary tumor in the right cheek and multiple lung metastases, and chemotherapeutic treatment was initiated. One year later, accumulation of fluorodeoxyglucose (FDG) was observed in the right front of the skull (standardized uptake value (SUV)-max 2.8) and left breast (SUV-max 2.4) using FDG-positron emission tomography (PET) / computed tomography (CT). Ultrasonography revealed the mammary tumor as a hypoechoic, internally heterogeneous mass measuring 22.4 × 16.2 × 21.1 mm with a rich blood supply. Using pathological findings of core-needle biopsy, we diagnosed it as ASPS. Based on the above information, we made a diagnosis of ASPS with left mammary and cranial metastases. Due to chemoresistance, surgical excision was selected as the mode of treatment; resection of the metastatic cranial bone was performed first, and partial mastectomy of the left breast was performed in two stages. Postoperative conditions were good, and we are currently performing regular follow-ups (visual palpation every 3 months and semi-annual mammary gland ultrasonography). CONCLUSIONS: We have reported an extremely rare case of ASPS with mammary metastasis with some reference-based discussion. In our case, disease control was obtained by a combination of drug therapy and surgical treatment.

Lingual Alveolar Soft Part Sarcoma in a 1-Year-Old Infant: Youngest Reported Case With Characteristic ASPSCR1-TFE3 Fusion.

Alveolar soft part sarcoma (ASPS) is an exceptionally rare non-rhabdomyosarcomatous soft tissue sarcoma (NRSTS), characterized by the translocation t(X;17) p(11.2;q25). This translocation results in the chimeric ASPSCR1-TFE3 transcription factor which drives tumorigenesis. Complete surgical resection is crucial in allowing a successful outcome in these cases. Here, we describe an 11-month-old female infant who presented with a well-circumscribed lesion of the tongue, with the clinical and radiologic appearances of an infantile hemangioma. This led to an initial plan for surveillance management. However, the mass continued to enlarge and the lesion was therefore biopsied when the infant was 17 months old. Histology showed plump epithelioid tumor cells, in many places lining pseudoalveolar spaces. Occasional Pas-D inclusions were present in the cytoplasm. Immunostaining showed nuclear positivity for TFE-3. Real-time quantitative polymerase chain reaction testing confirmed the presence of ASPSCR1-TFE3 fusion transcripts, characteristic of the translocation t(X;17) p(11.2;q25) observed in ASPS. This represents the youngest reported ASPS case with a confirmed molecular diagnosis. Complete surgical resection was undertaken and a surveillance imaging schedule implemented. This case highlights the need for regular review of the initial diagnosis and the importance of multidisciplinary discussion and early biopsy where the clinical course does not follow that expected for the putative (nonhistologically confirmed) diagnosis.