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Background: Alveolar soft part sarcoma (ASPS) occurs most often in the deep muscles or fascia of the extremities in adults, with only 3.4% of these tumours originating from the head, face and neck. To date, only 17 cases of buccal ASPS have been reported, including the case presented here. Only one case of ASPS recurrence at the primary site, similar to our case, has been reported thus far. Immune checkpoint inhibitors (ICPis)-associated diabetes, with an estimated incidence of 0.43%, is usually seen in older cancer patients and has not been reported in younger people or in patients with ASPS., Case Presentation: A 24-year-old male patient presented with a slowly progressing right cheek mass with a clinical history of approximately 28 months. Sonographic imaging revealed a hypoechoic mass, which was considered a benign tumour. However, a pathological diagnosis of ASPS was made after excision of the mass. Five days later, functional right cervical lymph node dissection was performed. No other adjuvant therapy was administered after surgery. In a periodic follow-up of the patient six months later, blood-rich tumour growth was noted at the primary site, and Positron emission tomography-computedtomography (PET-CT) ruled out distant metastasis in other areas. The patient was referred to the Ninth People's Hospital of Shanghai Jiaotong University. Due to the large extent of the mass, the patient received a combination of a Programmed Cell Death Ligand 1(PD-L1) inhibitor and a targeted drug. Unfortunately, the patient developed three episodes of severe diabetic ketoacidosis after the administration of the drugs. A confirmed diagnosis of ICPis-associated diabetes was confirmed. After the second operation, the postoperative pathological diagnosis was ASPS, and the margins were all negative. Therefore, we made a final clinical diagnosis of ASPS recurrence at the primary site. Currently in the follow-up, the patient is alive, has no distant metastases, and undergoes multiple imaging examinations every 3 months for the monitoring of their condition., Conclusions: In analysing the characteristics of all previously reported cases of buccal ASPS, it was found that the clinical history ranged from 1 to 24 months, with a mean of approximately 3 to 9 months. Tumour recurrence at the primary site has been reported in only one patient with buccal ASPS, and the short-term recurrence in our patient may be related to the extraordinarily long 28-month history. ICPis-associated diabetes may be noted in young patients with rare tumours, and regular insulin level monitoring after use is necessary., (© 2024. The Author(s).)

Alveolar soft-part sarcoma (ASPS) is a rare soft tissue tumor that primarily involves the extremities. We report a case of a 30-year-old never-smoker man who presented with hematuria, dysuria, and constipation at an outside hospital. He was diagnosed with and treated for multiple episodes of urinary tract infection. However, he continued to have voiding symptoms for which a cystoscopy was performed and revealed a bladder neck mass. He underwent transurethral resection of a bladder tumor and was diagnosed with muscle-invasive urothelial carcinoma, nested variant, at an outside hospital. Subsequent to this diagnosis he transferred his care to our center. In-house imaging revealed a large vascular mass involving the prostate and pushing against the bladder base. Prostate needle biopsies were performed and revealed an epithelioid neoplasm with a nested growth pattern composed of cells with a moderate amount of eosinophilic cytoplasm, mildly pleomorphic nuclei, and occasional prominent nucleoli. Since the findings were not classic for urothelial carcinoma or for prostate cancer, we included a wider differential of poorly differentiated carcinoma, sarcoma, and paraganglioma. A wide panel of keratin stains was negative, ETS (erythroblast transformation-specific)-related gene highlighted an extensive vascular network and neuroendocrine stains were all negative. A transcription factor E3 fluorescent in-situ hybridization was positive and subsequently, an ASPSCR1 gene rearrangement was demonstrated. The outside hospital transurethral resection of bladder tumor was obtained for review and the tumor was morphologically similar to that seen on the in-house prostate needle biopsies. Based on the above findings a final diagnosis of primary ASPS of the prostate with involvement of the bladder was made. The patient was later diagnosed with bilateral lung metastases. He was treated with pazopanib, radiation therapy, and cystoprostatectomy and is symptom-free on a 15-month follow-up., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Alveolar soft part sarcoma (ASPS) is a very rare mesenchymal malignancy of uncertain origin. It mostly affects young people, with about a quarter of cases being diagnosed in children., Case: An 11-year-old girl had a painless subcutaneous "lump" in the left elbow area. Imaging exams revealed a solid soft-tissue intramuscular mass of suspicious appearance. A surgical excision of lesion was performed. The biopsy consisted of a lobular tumor measuring 35 × 20 × 12 mm. Histology revealed an epithelioid-cell population arranged in organoid pseudoalveolar pattern. It immunohistochemically expressed TFE3 and harbored the ASPSCR1:: TFE3 gene fusion. A diagnosis of ASPS was established. Subsequently, a wide re-excision of the scar was performed without microscopic residual tumor. The patient is currently without evidence of local recurrence or metastasis., Conclusion: ASPS is considered an aggressive and prognostically unfavorable chemoresistant neoplasm. Children have a better prognosis compared to adults. Early detection of tumor in a localized stage with complete surgical removal remains a mainstay therapeutic option. Due to its tendency to late metastases, a long-term thorough follow-up of the patient is necessary.

Objective: Female genital alveolar soft part sarcoma (ASPS) is rare and has a favourable prognosis compared to ASPS from other sites. We reported our experience to manage a case with uterine corpus ASPS (UC ASPS) and conducted a literature review on prognosis of ASPS from different sites of female genital tract., Case Report: This report represented a 33-year-old woman who had UC ASPS. She received tumor excision with uterine preservation and had the longest follow-up time (155 months) without recurrence in the literature., Conclusion: UC ASPS has better prognosis than ASPS from the uterine cervix, the low uterine segment, vulvovaginal area and perineum. We recommended conservative treatment for young women with UC ASPS., Competing Interests: Declaration of competing interest The authors declare no conflict of interest., (Copyright © 2023. Published by Elsevier B.V.)

Alveolar soft part sarcoma (ASPS) commonly involves extremities and head and neck regions. Primary intracranial ASPS is rare. We report a series of 3 primary intracranial ASPS. These were not suspected clinically and histopathology with immunohistochemistry proved to be diagnostic in all 3 tumors.

Competing Interests: Competing interests: None declared.

Background: Alveolar soft-part sarcoma (ASPS) is a rare soft tissue sarcoma subtype, occurring mainly in young people, with poor prognosis., Materials and Methods: We conducted a retrospective analysis of localized or metastatic ASPS patients admitted to the First Affiliated Hospital of Zhengzhou University (China) from 2012 to 2020, focusing on treatment and prognosis., Results: The median age at diagnosis was 24 years (range: 1.4-78 years). Women ( n  = 29, 58%), especially those aged <30 years, dominated this series. The most common metastasis site was lung. Thirty-one (62%) patients developed lung metastasis (localized: n  = 9 [18%]; metastatic: n  = 22 [44%]). Only a tumor maximum diameter ≥ 5 cm was associated with a high lung metastasis rate ( p  = 0.039). The mean follow-up time was 37.5 months (1-108 months), and the 5-year overall survival (OS) rate was 84.7%. Univariate analysis indicated that distant metastasis observed at the initial visit and incomplete resection of the primary tumor were associated with poor OS. For localized cases, neither surgery plus radiotherapy ( p  = 0.486) nor surgery plus chemotherapy ( p  = 0.536) improved progression-free survival compared to surgery alone. Among the metastatic cases, the disease control rate (PR + SD) was higher for targeted therapy (60%) and combined immunosuppressive therapy (100%) than for conventional cytotoxic chemotherapy (26%)., Conclusions: Postoperative adjuvant radiotherapy and chemotherapy do not provide good local control for patients with localized disease. Although there is no standard treatment strategy for patients with advanced or metastatic disease, they can benefit from targeted therapy and/or immunosuppressive therapy.

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.

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 (ASPS) is a rare malignancy with low sensitivity to chemotherapy. While localized ASPS has a very good prognosis after resection, the 5-year overall survival rate drops substantially in metastatic disease. We report the case of an 80-year-old male patient with ASPS of the left elbow and metastasis to the lung, lymph nodes and peritoneum. After weighing the benefits and risks, systemic treatment with the anti-PD-1 checkpoint inhibitor pembrolizumab combined with the vascular endothelial growth factor receptor tyrosinkinase inhibitor axitinib was initiated in this patient with a history of psoriasis and Crohn's disease. After only two cycles of therapy, a significant size reduction of the nodal cervical metastasis became apparent. A partial response of all metastases was then confirmed in the first computed tomography restaging. So far, side effects have remained manageable, especially with regard to the development or worsening of autoimmune adverse events. The patient continued to have a high quality of life, while also remaining in ongoing partial response for 15 months at the time of submission. While sarcomas generally have low sensitivity to immunotherapies, ASPS is an exception, and checkpoint inhibition is an integral part of its systemic therapy., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

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., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.)

Alveolar soft part sarcomas (ASPSs) are rare malignant tumors representing ∼1% of all soft tissue sarcomas. Most ASPS occurring in the central nervous system are metastases. In contrast, primary intracranial ASPSs are extremely rare and only 8 cases have been previously reported in English literature. Here, we report a case of primary alveolar soft part sarcoma in a 16-year-old female patient with no evidence of primary extracranial tumors. Histologically this case fulfilled the criteria of ASPS, and a molecular confirmation has been archived. To date, only 9 primary intracranial ASPS cases, including ours, have been reported in the literature. This report highlights the clinical and pathological characteristics, differential diagnosis, and molecular analysis of primary ASPS of the central nervous system.

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 (ASPS) is a rare soft tissue sarcoma characterized by an alveolar or organoid arrangement of polygonal tumour cells separated by fibrovascular septa. A specific fusion gene [ASPS critical region 1 (ASPSCR1)-TFE3] was detected in ASPS. Despite being a slow-growing tumour without pain and dysfunction, ASPS is characterized by early metastasis, which leads to poor prognosis. Herein, we report a rare case of primary ASPS of the cheek harbouring ASPSCR1 (exon 7)-TFE3 (exon 5) fusion gene in a 21 year-old woman. This tumour was a well-circumscribed, smooth, round mass that was clinically suspected as a benign tumour. However, histologically, it was observed that the polygonal tumour cells were arranged in solid and alveolar growth patterns. Post-operative examination of the whole body excluded the possibility of metastasis at other sites. Thus, careful immunohistochemical and genetic analyses, as well as whole-body examination, demonstrated that the tumour was a primary ASPS of the cheek., (© 2021. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Alveolar soft part sarcoma (ASPS) has the highest incidence of brain metastasis amongst sarcomas. There is a paucity of literature published focusing on radiation therapy for this condition. This is a single centre retrospective review of the treatment of three patients with 12 ASPS brain metastasis using single dose stereotactic radiosurgery (SRS). Five lesions were treated with low (<25 Gy) and seven with high (≥25 Gy) dose. Four lesions had a volume of >1.5 cm 3 and were defined as large, while seven had a volume of ≤0.5 cm 3 and were defined as small. The local tumor control as well as the clinical complication rates were studied. There was a statistically significant relation between treatment dose and tumor control rate. All large tumors treated with low dose recurred and required surgical removal within two months following SRS, while the large lesion treated with high dose recurred after 11 months. Five of the six small tumors treated with high doses were controlled, while the sixth required retreatment and was stable thereafter. No patient suffered from undue symptomatic radiation effects. The success rate following SRS for small ASPS metastases treated with high doses seems to be sufficient to justify the treatment. The short time for large tumor to recur, significant increase in tumor size requiring surgical removal of the tumors, makes low dose SRS unattractive. Based on this limited patient population, it seems that high dose SRS should be used for all ASPS brain metastases except for large tumors deemed surgically accessible., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

BACKGROUND Alveolar soft-part sarcoma is an uncommon mesenchymal tumor accounting for approximately 0.7% of soft tissue sarcomas in adults. It mainly affects young adults, with a peak incidence between 15 and 35 years old. Available data indicate that surgical resection with adjuvant therapy using tyrosine kinase inhibitor may be considered the standard treatment. The rarity of the disease and resultant data scarcity makes it difficult to establish treatment guidelines. CASE REPORT We present the 9-year follow-up of a 24-year-old patient with an initially advanced (stage IV), huge, 21-cm alveolar soft-part sarcoma of the retroperitoneum. During the observation period, the patient developed pulmonary, brain, and bone metastases. In the course of treatment, she underwent excision of the main tumor, excision of satellite tumors, and brain metastasectomies, and was treated with sunitinib, pazopanib, and radiotherapy. No similar case reports were found in the PubMed database. CONCLUSIONS Our multimodal approach resulted in a long period of stable disease. Late progression may occur; therefore, frequent and thorough imaging evaluation of such patients is crucial. Our case is one of the largest ASPS tumors reported, and her long-term successful treatment makes this report valuable, considering the scarcity of data regarding treatment of ASPS. Further large-cohort, multi-center studies are necessary to establish the best treatment.

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., (Copyright Journal of Radiology Case Reports.)

Alveolar soft part sarcoma (ASPS) is a rare soft tissue sarcoma, often occurs in adolescents and young adults with a particular predilection for the deep soft tissue of extremities. Occurrence of ASPS in the female genital tract is very uncommon and poses a significant diagnostic challenge. A case of ASPS of the uterine corpus is described in a young unmarried female, who presented to the out-patient clinic of our Institute with complaints of abnormal uterine bleeding for the past 9 mo. She was being treated with oral contraceptive pills, progesterone and tranexamic acid. Following radiological imaging and hysteroscopy, a therapeutic curettage of the lesion was done. The histopathologic features raised a differential diagnosis of a myriad of morphologic mimickers. The diagnosis was clinched by exclusion of mimickers by relevant immunohistochemical markers and strong nuclear expression of TFE3 on immunohistochemistry. The patient is on regular follow-up with oral contraceptives and antifibrinolytic medication. Despite being infrequent at this location, ASPS should be kept in differential diagnosis in young females presenting with abnormal uterine bleeding., Competing Interests: The authors declare no conflict of interest., (Copyright © 2020 by the International Society of Gynecological Pathologists.)

Alveolar soft part sarcoma (ASPS) is a rare malignancy that typically arises in the trunk or extremities and preferentially metastasises to the brain. Radical resection is generally recommended for cranial metastatic ASPS, but stereotactic radiosurgery (SRS) is a recognised alternative for tumours in surgically challenging locations. Here, we present the case of a 22-year-old female, who underwent SRS and systemic therapy with pazopanib for a metastatic ASPS in the left temporal bone. The tumour was successfully controlled without further intervention over 23 months following SRS, which should be considered for metastatic ASPS when surgical resection is not appropriate.

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.

Background: Alveolar soft part sarcoma is a rare, histologic subtype of soft tissue sarcoma that remains poorly defined. We aimed to describe patient characteristics and treatment patterns and to examine factors associated with survival for patients with alveolar soft part sarcoma., Methods: After identifying patients with alveolar soft part sarcoma in the National Cancer Database, we recorded their clinicopathologic characteristics. Univariable log-rank survival analysis and Cox proportional hazards model were employed. For context, survival comparisons were included for patients with other sarcoma subtypes., Results: Overall, 293 patients with alveolar soft part sarcoma were identified. Interestingly, patients with head and neck tumors were least likely to present with distant disease (40%, P = .025). The majority of patients underwent resection (n = 183, 63%). Among those, no predictors of lesser survival were identified other than the presence of metastases (hazard ratio 6.04, P ≤ .001). Patients with stage IV alveolar soft part sarcoma who underwent resections experienced improved survival relative to similar patients with more common subtypes of soft tissue sarcomas (P ≤ .001)., Conclusion: Alveolar soft part sarcoma is exceedingly rare, and patients often present with metastases. Primary tumors can occur anywhere in the body, and location impacts the rates of metastases at presentation. Resection is associated with a favorable survival advantage when compared to other, more common histologic subtypes of soft tissue sarcomas., (Published by Elsevier Inc.)

Alveolar soft part sarcoma (ASPS) is a very rare soft tissue sarcoma. Primary ASPS of the gastrointestinal tract is especially rare. Due to the scarcity of cases, neither its clinicopathologic features nor its mutational background has been clarified. Here, we report a case of ASPS arising from the rectum, which was completely resected by endoscopic submucosal dissection. The lesion was a 17 × 16 × 15 mm semi-pedunculated mass in the upper portion of the rectum in a 46-year-old female. In terms of histology, tumor cells exhibited confluent eosinophilic cytoplasm, forming a sheet-like architecture. Periodic acid Schiff-positive diastase-resistant intracytoplasmic crystals were observed in the tumor cells. Fluorescence in situ hybridization revealed TFE3 rearrangement, and reverse transcription polymerase chain reaction revealed an ASPSCR1-TFE3 type 1 fusion. Negative PAX8 immunostaining and the absence of other massive lesions in postoperative imaging studies led to a diagnosis of primary ASPS of the rectum. The potential oncogenic role of the canonical ASPSCR1-TFE3 fusion transcript in gastrointestinal ASPS was indicated. Primary gastrointestinal ASPS remains a diagnostic pitfall in routine surgical pathology., (© 2020 Japanese Society of Pathology and John Wiley & Sons Australia, Ltd.)

Background: Alveolar soft part sarcoma (ASPS) is a clinically and morphologically distinct malignant soft tissue tumour. It occurs mostly in the lower extremities in adults. The purpose of our study was to describe the related clinicopathologic factors, treatment and prognosis of recurrent orbital ASPS in children., Case Presentation: Three children aged from 1 to 12 years presented with unilateral proptosis, restricted ocular motility and impaired visual acuity of the affected eye. Periodic acid Schiff (PAS) -positive diastase-resistant crystalline granules were found in all cases. Immunostaining of TFE-3, INL1 and Ki67 was positive in the tumour cells of two patients. The time until local recurrence of primary tumor in patient 2 and patient 3, who only underwent tumour resection, was shorter than that of patient 1, who underwent tumour excision and postoperative radiotherapy. The recurrent masses were successfully treated with complete tumour excision followed by adjuvant radiotherapy. Patient 1 presented metastasis at 11 years after radiotherapy., Conclusions: Orbital ASPS in children is easily misdiagnosed due to its rare occurrence and atypical clinical findings. Early diagnosis with multidisciplinary, complete surgical resection combined with adjuvant radiotherapy is essential for achieving long-term disease-free survival in orbital ASPS patients.

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.

Rationale: Alveolar soft part sarcoma (ASPS) is a rare malignant soft tissue neoplasm with controversial histogenesis. ASPS accounts for 0.5% to 1% of all soft tissue sarcomas. Because of its rarity, ASPS is easily misdiagnosed, increasing the risk of incorrect treatment., Patient Concerns: A 6-year-old female patient presented with a history of a 2.0 × 2.5 × 3.0-cm mass in the deep soft tissues of her right lower extremity., Diagnoses: Histopathological features indicated the diagnosis of ASPS. Microscopically, a diffuse arrangement of tumor cells or pseudoalveolar architectures separated by thin and well-vascularized fibrous septa were observed. Immunohistochemical staining of the tumor cells indicated positivity for transcription factor E3, myogenic determination factor 1, and periodic acid-Schiff-diastase (PAS-D) and showed a Ki-67 proliferating index of approximately 20%., Interventions: The patient underwent enlarged resection of the tumor and was treated with radiotherapy., Outcomes: During the 3-year follow-up, the patient has remained in good condition, with no symptom recurrence, distant metastatic spread, or significant toxicity during or after treatment. The patient remains under regular surveillance., Lessons: Its low incidence, lack of characteristic clinical manifestations, and atypical location often lead to ASPS misdiagnosis and subsequent incorrect treatment. Nuclear expression of transcription factor E3 is of diagnostic value for ASPS. At present, there is no consensus on the treatment for ASPS. In-depth pathological analysis is needed to better understand the characteristics of this tumor.

Objective: To describe different types of primary extraocular muscle (EOM) tumours based on the results of imaging studies, peroperative clinical picture and their histopathological diagnosis., Study Design: Case series., Place and Duration of Study: Al-Shifa Trust Eye Hospital, Rawalpindi, from July 2001 to January 2017., Methodology: A retrospective analysis of 640 diagnosed orbital tumours was carried out using non-randomised sampling technique, and the prevalence of primary EOM tumours was determined. Based on the results of imaging studies, the clinical picture observed during surgery (orbitotomy) and the histopathological diagnosis, primary EOM tumours were divided into different types, accordingly., Results: Nineteen (n=19) primary EOM tumours (frequency of 2.96%) had 12 types of histopathological diagnoses, and were categorised into inflammatory tumours (n=8, 42%), vascular tumours (n=4, 21%), lymphoproliferative tumours (n=3, 16%), neurogenic tumours (n=2, 10.5%) and myogenic tumours (n=2, 10.5%). The recti were involved more frequently than obliques (n=15, 78.94% and n=4, 21.06%, respectively). All the patients presented with proptosis of varying degree with some degree of globe rotation and had surgical excision/appropriate management. Visual acuity was not affected in any of the patients. Four (n=4, 21.05%) tumours were malignant (NHL, ASPS, myeloid sarcoma and rhabdomyosarcoma) and these patients underwent chemotherapy and/or radiotherapy., Conclusion: Biopsy-proven primary EOM tumours were devisable into five broad categories. Patients with primary EOM tumours presented with proptosis and impaired ocular motiliy. The primary EOM tumours involved both the recti and the obliques and were excised surgically with favourable outcomes in most cases.

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.

Alveolar soft-part sarcoma is a rare neoplasm of unknown histogenesis that accounts for less than 1% of all soft-tissue sarcomas. The tumor is highly vascularized with small vascular spaces separating nests of cells, and from cytogenetic point of view, is characterized by chromosome rearrangement der(17)t(X:17)(p11:q25) that results in the ASPL-TFE3 translocation. It can occur at any age, but it is most common between 15 and 35 years of age. The prognosis is poor, despite the relatively slow growth of the tumor. We present here an atypical case of alveolar soft-part sarcoma in which the age of the patient, the location, and the histopathologic characteristics of the lesion represented a diagnostic challenge.

Alveolar soft part sarcoma (ASPS) is a rare soft tissue neoplasm generally affecting adolescents and young adults. Its unique histologic and ultrastructural features have been well-described; however, the cytopathological features of ASPS are less well-characterized, and recognition of this entity's features on cytologic preparations can ensure that the specimen adequacy and appropriate/rapid tissue allocation for additional testing. Herein we report a FNA case of ASPS with emphasis on cytomorphologic characteristics., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Rational: Alveolar soft part sarcomas (ASPSs) with multiple brain metastases in pregnancy is a rare entity., Patient Concerns: We report our experience with a 19-year-old pregnant woman who presented with intermittent headaches and vomiting at 38 weeks gestation., Diagnoses: The patient was initially diagnosed as brain metastasis according to computed tomography and magnetic resonance imaging (MRI) imaging., Interventions: Cesarean section and craniotomy (complete resection of both brain metastatic lesions) was performed sequentially., Outcomes: A healthy baby girl was delivered safely and no neonatal malformations were found. Histological analysis confirmed the diagnosis of ASPS. Follow-up MRI performed 10 months after surgery revealed no residual tumor or signs of recurrence., Lessons: We report a case of ASPS with multiple brain and lung metastases in a pregnant woman. We recommend timely MRI examination for diagnosis and have discussed the approach to the treatment of pregnant women with brain metastasis.

Rationale: Alveolar soft part sarcoma (ASPS) is a rare, malignant neoplasm, which mostly occurs in the upper and lower extremities. This article presents an unusual case of ASPS involving the penis of a 3-year-old boy. To our knowledge, this is the first case of ASPS in the penis of a child., Patient Concerns: The patient complained of slight penile pain for 1 year and a soft tissue mass could be palpated in his penis., Diagnoses: Imaging was performed on the penis. The pathological feature of the mass was evaluated through biopsy examination. It was found that the mass was an alveolar soft tissue sarcoma, which was then confirmed by immunohistochemistry., Interventions: The patient only underwent a partial penectomy because his parents wished to keep the penis. Conventional chemotherapy has been performed for 6 months after the surgery., Outcomes: At 28-month follow-up the mass did not increase apparently, and no signs of metastasis were found., Lessons: ASPS may occur originally in the penis.

Background: Alveolar soft part sarcoma (ASPS) is a rare, malignant soft-tissue neoplasm typically seen in young adults that possesses an unusual tendency to metastasize. Metastases to the intramedullary compartment of the spinal cord, however, are exceptionally rare and have not been described in the literature., Case Description: We report the case of a 23-year-old woman with disseminated ASPS to the lung and brain who presented with progressive lower-extremity weakness and loss of sensation after radiation and chemotherapy. Magnetic resonance imaging revealed a 1.3-cm avidly enhancing lesion within the central thoracic spinal cord at T3. A T2-T4 laminectomy was undertaken and resulted in a gross total resection. Histopathologically, the mass was composed of organoid nests containing epithelioid cells with eosinophilic, granular cytoplasm separated by sinusoidal spaces. Immunohistochemistry demonstrated convincing positive TFE3 staining. Postoperative imaging confirmed the complete resection of the mass, and her examination was notable for intact sensation and impaired motor function that gradually improved., Conclusions: A review of the literature found that the reported case represents the first instance of primary or metastatic ASPS in the spinal cord. Metastatic ASPS should thus be included in the differential diagnosis in patients with known disease and neurologic impairment or back pain. Imaging of the spine should then be considered., (Copyright © 2017 Elsevier Inc. All rights reserved.)

A 20-year-old woman with a perineal alveolar soft part sarcoma was referred to our hospital. MRI showed that an irregular oval tumor occupied the perineum. The tumor was contiguous to the vagina, rectum, levator muscle, and pubis and was diagnosed as alveolar soft part sarcoma by transvaginal biopsy. Laparoscopy-assisted total pelvic exenteration combined with a pubic resection was performed, and an R0 resection with a wide margin was achieved. It is well known that only R0 resection improves the outcome of patients with localized alveolar soft part sarcoma. In this case, the perineal manipulation was difficult because the tumor was huge and had a rich blood flow. Massive bleeding occurred during the perineal manipulation. However, we kept the operative field dry thanks to minimal intraoperative blood loss during the laparoscopic phase. The laparoscopic approach might be advantageous for such a demanding surgical procedure for tumors in the distal pelvis and perineum., (© 2016 Japan Society for Endoscopic Surgery, Asia Endosurgery Task Force and John Wiley & Sons Australia, Ltd.)

Laryngeal sarcoma is a rare and potentially aggressive malignancy. In this case report, we present a 23year-old-male with four-years of progressive hoarseness who was found to have a large left paraglottic mass. A partial laryngectomy was successful at completely excising the lesion. Final pathology returned as alveolar soft part sarcoma. Alveolar soft part sarcomas of the larynx are extremely rare with only five cases published in the current literature. This article provides a case presentation with literature review of alveolar soft part sarcoma of the head and neck., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Background: Alveolar soft part sarcoma (ASPS) is a rare entity of soft tissue malignancies with uncommon spinal involvements. Surgical management should be the best choice of cure., Methods: Five patients with spinal ASPS were interviewed retrospectively, where data was collected. The relevant literatures were also systematically examined. Thereafter, patient and surgical data were obtained and pooled for prognostic analysis., Results: A total of five patients with eight surgeries were reviewed retrospectively, and three patients previously reported were also included. All patients were surgically treated, where five of them underwent additional adjuvant therapies such as chemotherapy, radiotherapy, and targeted therapy in order to manage their local and/or systematic diseases. One patient was lost in follow-up. For the remaining seven patients, the mean follow-up period was 19.7 ± 8.8 months, two succumbed to disease while five were alive at the time of the study., Conclusions: Surgical management is shown to be the most important and the most effective treatment strategy for spinal ASPS, whereas adjuvant therapies made little impact. The prognostic factors for spinal ASPS are primary or metastatic lesions, neurological status, disease progression, systematic conditions, and resection approaches.

Background: Alveolar Soft Part Sarcoma is a rare malignant tumor of uncertain histogenesis, representing 0.5-1% of all soft tissue tumors. It occurs predominantly in head and neck regions, especially the orbit and the tongue, in infants, causing no specific symptoms for an extended period of time., Case: A four year old girl was brought to consultation because of persistent swelling of her left upper and lower eyelid for the past six months. A 4 mm proptosis was noted on her left eye with significant upwards displacement of the globe. A firm, not pulsating and non tender mass was palpated, and motility examination revealed deficient abduction and infraduction. Visual acuity was 20/40 and 1/200 in the right and left eye, respectively. The computed tomography revealed an extraconal mass on the inferotemporal aspect of the left orbit, with no bony erosion or globe invasion. An excisional biopsy was made, finding a tumor with nests of clusters of large polygonal cells, separated by fibrous septa and a sinusoidal vascular channel. An Alveolar Soft Part Sarcoma was diagnosed. Our patient had a favorable post-operative follow up, and oncologic evaluations have not shown metastasis or local recurrences. Observations and Conclusions: Alveolar Soft Part Sarcoma is a rare malignant tumor of indolent course, but with propensity to distant metastases, making early diagnose and long term follow up necessary., (© NEPjOPH.)

Objective: The aim of this study was to investigate the magnetic resonance (MR) features of alveolar soft-part sarcoma (ASPS)., Methods: We studied 12 patients with ASPS confirmed by pathology in this retrospective study. MR features were analyzed, especially for the location, morphology, signals, and related enhanced features of the tumor vessels., Results: Flow voids were shown in the central part of the tumor on T2-weighted imaging (T2WI) in all patients; they were arrayed in a radiating mode gathered toward the center (8 cases), designated by us as vascular center-gathered syndrome (VCGS), or scattered like twigs (4 cases). The flow voids were accompanied by high signals in all patients, including tubular (6 cases) and platy (6 cases) signals. Slightly higher signals were shown in the peripheral part of the tumor in all patients. Flow voids in the peripheral part were shown in all patients, and the majority of the flow voids surrounded the tumor (8 cases). The vessels around the tumor in 9 patients showed high signals, and the majority of the vessels were located at the superior and inferior poles (8 cases). 6 patients underwent enhanced scanning, including moderate (5 cases) and significant enhancement (1 case)., Conclusion: Low signals of radiating flow voids accompanied by high signals of slow blood flow or blood sinuses in the center part have high significance for the diagnosis of ASPS., (© 2017 S. Karger GmbH, Freiburg.)

Purpose: To perform a joint analysis of data from 3 contributing centers within the intraoperative electron-beam radiation therapy (IOERT)-Spanish program, to determine the potential of IOERT as an anticipated boost before external beam radiation therapy in the multidisciplinary treatment of pediatric extremity soft-tissue sarcomas., Methods and Materials: From June 1993 to May 2013, 62 patients (aged <21 years) with a histologic diagnosis of primary extremity soft-tissue sarcoma with absence of distant metastases, undergoing limb-sparing grossly resected surgery, external beam radiation therapy (median dose 40 Gy) and IOERT (median dose 10 Gy) were considered eligible for this analysis., Results: After a median follow-up of 66 months (range, 4-235 months), 10-year local control, disease-free survival, and overall survival was 85%, 76%, and 81%, respectively. In multivariate analysis after adjustment for other covariates, tumor size >5 cm (P=.04) and R1 margin status (P=.04) remained significantly associated with local relapse. In regard to overall survival only margin status (P=.04) retained association on multivariate analysis. Ten patients (16%) reported severe chronic toxicity events (all grade 3)., Conclusions: An anticipated IOERT boost allowed for external beam radiation therapy dose reduction, with high local control and acceptably low toxicity rates. The combined radiosurgical approach needs to be tested in a prospective trial to confirm these results., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Aim: To study the clinico-radiological and clinic-pathological features of orbital alveolar soft-part sarcomas (ASPS)., Materials and Methods: We retrieved 9 cases of orbital ASPS. Histopathological and immunohistochemical features and clinico-radiological findings were evaluated., Results: Age at presentation varied from 1 to 31 years. Both, children and adults were affected. Proptosis was the most common clinical presentation. PAS-positive diastase-resistant crystals and granules were found in all cases. All patients received adjuvant radiotherapy. Two cases had positive surgical margins and were treated with additional adjuvant chemotherapy. None of the patients had recurrence or metastases till the last follow up., Conclusion: Early diagnosis with a multidisciplinary, multimodal approach of treatment is quintessential in achieving a long disease-free survival in patients with ASPS.

Objective: To describe imaging features of primary and metastatic alveolar soft part sarcoma (ASPS)., Methods: In this institutional review board-approved and Health Insurance Portability and Accountability Act-compliant retrospective study, 25 patients (14 males; mean age, 25 years; range, 18-40 years) with pathologically proven ASPS seen at our institute between 1995 and 2013 were included. Imaging of primary tumours in 5 patients and follow-up imaging in 25 patients were reviewed by 2 radiologists in consensus. Clinical information was obtained from electronic medical records., Results: The most common sites for the primary tumour were extremities (17/25, 68%) and torso (6/25, 24%). Primary tumours (n = 5) were well circumscribed, compared with skeletal muscle, were isodense on CT, hyperintense on T1 and T2 weighted images with intense post-contrast enhancement, prominent feeders on CT and flow voids on MRI. Metastases developed in 23/25 (92%) patients, 18 at presentation. The most common sites of metastases were the lungs (100%), lymph nodes (74%), bones (57%) and brain (43%). Visceral and nodal metastases were hypervascular. At the time of reporting the results, 15 patients have died, 6 are alive and 4 were lost to follow-up. Median survival was 74 months for those without brain metastases (n = 8) and 60 months for those with brain metastases (n = 7). Median survival was shorter for patients with metastases at presentation., Conclusion: ASPS most commonly involves the lower extremities of young adults, is hypervascular on imaging, often metastasizes at presentation, frequently to lung, nodes, bones and brain, and has an indolent course despite metastases. Brain metastases and high tumour burden (number of metastatic sites) at presentation decreased survival in our study., Advances in Knowledge: ASPS has an unusual pattern of metastases to the brain and nodes in addition to lung and bones. It has an indolent course despite metastases.