Primary alveolar rhabdomyosarcoma of the brain: a case report.

BACKGROUND: Primary brain rhabdomyosarcoma is a rare primary brain malignancy with few case reports. The vast majority of cases of primary brain rhabdomyosarcoma occur in pediatric patients, and immunohistochemistry can distinguish it from embryonal subtypes; however, few cases of primary brain rhabdomyosarcoma in adults have been reported in the literature. CASE PRESENTATION: We report the case of a 26-year-old White male patient who was found to have primary brain alveolar rhabdomyosarcoma after developing headaches for several months. A brain MRI revealed a mixed cystic and solid tumor along the vermis of the cerebellum. The patient underwent a gross total surgical resection, which confirmed the diagnosis of alveolar rhabdomyosarcoma. Further staging workup for another primary focus or disseminated disease yielded negative results, confirming the diagnosis of primary alveolar rhabdomyosarcoma of the brain. CONCLUSION: The standard of care for managing this rare type of brain tumor involves surgery with adjuvant chemoradiotherapy. Further studies should be conducted for a better diagnostic and therapeutic understanding.

18 F-FDG PET/CT of Alveolar Rhabdomyosarcoma of Prostate in a 17-Year-Old Boy.

ABSTRACT: Rhabdomyosarcoma of the prostate is a rare malignancy that usually presents in childhood and adolescence with symptoms of bladder outlet obstruction. Serum prostate-specific antigen is usually normal, and 18 F-FDG PET/CT has a crucial role to rule out distant metastases. We present a case of alveolar rhabdomyosarcoma of the prostate in a 17-year-old boy.

Parameningeal alveolar rhabdomyosarcoma in a child.

This is the case of a parameningeal alveolar rhabdomyosarcoma (ARMS) in a 13-year-old boy who presented with painless loss of vision in the right eye, but very few other physical signs. The ARMS diagnosis was confirmed with imaging and molecular characterisation of the tumour. Despite tolerating the initial chemotherapy and radiotherapy regimens, there was leptomeningeal recurrence and the patient unfortunately passed away. Parameningeal ARMS occurs in an area of the body, which leads to a wide variety of possible presenting symptoms, creating a long list of differentials that can delay treatment. This tumour subtype has a poor prognosis, and due to the location of the tumour around vital structures in the head, treatment toxicities must be taken into account. This highlights the necessity for having a strong index of suspicion for this tumour in atypical presentations in children, and the necessity for prompt treatment to prevent leptomeningeal disease from occurring.

Bilateral alveolar rhabdomyosarcoma of breast: a rare entity.

Sarcomas of breast constitute less than 1% of all malignant breast tumours. Alveolar rhabdomyosarcoma (RMS) is very rare in breast with limited case reports in literature, and primary alveolar RMS arising from breast is still less common than metastatic RMS. Here, we report a case of primary bilateral alveolar RMS of breast in an adolescent female where the correct diagnosis was obfuscated by an overlap in the histological features of RMS and high-grade invasive ductal carcinoma.

Alveolar rhabdomyosarcoma metastatic to the brain.

Alveolar rhabdomyosarcoma (ARMS) has a predilection for the peripheral extremities, and brain metastases are rare, with only a few cases reported after the initial diagnosis. We present a 22-year-old male patient with a right orbital-ethmoidal ARMS who presented with a recurrence to the brain 1 year after the initial diagnosis. He was referred to our institution due to acute neurological deterioration. A brain MRI was performed, showing an enhancing bilateral parafalcine lesion centred about the bilateral cingulate gyri with extension into the corpus callosum. The patient was taken to the operating room for a stereotactic biopsy under general anaesthesia, which was compatible with metastatic ARMS. Our case is exceedingly rare, considering the initial diagnosis of an orbital/ethmoidal ARMS, its subsequent metastasis to the brain and its clinical sequelae after a biopsy. Prognosis after cerebral metastatic ARMS is dismal, with most patients expiring due to central nervous system metastatic disease.

Adult parameningial alveolar rhabdomyosarcoma: Case report and literature review.

Alveolar rhabdomyosarcoma (ARMS) represents the most common childhood soft tissue sarcoma, but they are rarely seen among adults. Most of the protocols for adults are adapted from pediatric protocols. Here we report a case of a 53-year-old woman diagnosed with a nasal alveolar rhabdomyosarcoma, stage IV at diagnosis, treated by chemotherapy (a regimen inspired from the pediatric protocole pEpSSG RMS 2005) which led to partial response followed by chemo-radiotherapy. We performed a systematic review of adult head and neck ARMS and found 29 cases. Primary chemotherapy with different protocols (VAC, VAI or VIE) should be done followed by surgery and/or external beam radiotherapy (preferably with IMRT). EBRT seems beneficial to every ARMS with a dose around 50Gy in a conventional fractionation, eventually completed with a boost on residual tumor. The target volume must be defined on pre-chemotherapy imaging. Brachytherapy and proton therapy are under evaluation.

Treatment strategies and outcomes for spinal rhabdomyosarcoma: A series of 11 cases in a single center and review of the literature.

OBJECTIVES: Rhabdomyosarcoma (RMS) involving the spine is rare. The aim of the present study is to explore the clinicopathological features, surgical treatments and outcomes of this rare disease. PATIENTS AND METHODS: Eleven patients with spinal RMS who received surgery in our institution between 2012 and 2018 were retrospectively investigated. The literature on spinal RMS was also reviewed. RESULTS: Our study consisted of 7 cases of primary RMS and 4 cases of metastatic RMS. Seven primary and one metastatic spinal RMS received radical resection, the remaining three metastatic patients received palliative resection. Eight patients died with a median survival time of 8 months. The mean value of Ki-67 positivity was 48.2 %. Literature review revealed a total of 22 previously reported cases. 54.5 % of the pooled cases of the 33 patients were under the age of 18. Of the 20 patients with primary spinal RMS, 9 cases were diagnosed as embryonal, while 6 of the 13 metastatic patients were diagnosed as alveolar. Multiple modalities, including surgery and concurrent adjuvant therapy were performed in 19 patients. The median overall survival (OS) for 28 patients with detailed follow-up information was 10 months. Radical resection offered a significant longer median OS than non-radical resection (18 vs. 6 months, p = 0.027). CONCLUSION: Spinal RMS mainly affects young patients. The embryonal form and alveolar form is the most frequent subtype for primary and metastatic spinal RMS respectively. Spinal RMS is highly aggressive with dismal prognosis. Multimodality therapies are the mainstay of treatment. Radical resection is strongly recommended in eligible patients.

Alveolar Rhabdomyosarcoma Following Mandibular Distraction Osteogenesis.

The authors present a rare case of anaplastic alveolar rhabdomyosarcoma (ARMS) occurring after Mandibular Distraction Osteogenesis. A 16-month-old male presented with a rapidly enlarging left lower jaw mass after removal of mandibular distractors placed shortly after birth for Pierre Robin Sequence and severe obstructive sleep apnea. Incisional biopsy of the mass revealed ARMS with anaplastic features. Although ARMS is an extremely rare entity, craniofacial surgeons should have a low threshold for seeking further diagnostic modalities of a growing mass postmandibular distraction.

Primary cutaneous alveolar rhabdomyosarcoma in an adolescent - A challenging diagnosis.

Primary cutaneous alveolar rhabdomyosarcoma is an extremely rare and highly aggressive soft tissue sarcoma that predominantly arises on the extremities and perineum of adolescents and young adults. Dermatologists should be aware of these tumors in order to promptly make the diagnosis and initiate treatment.

Outcome and Patterns of Relapse in Childhood Parameningeal Rhabdomyosarcoma Treated With Proton Beam Therapy.

PURPOSE: The standard of care of childhood parameningeal rhabdomyosarcoma (pRMS) is chemotherapy and local radiation therapy. Protons are increasingly being used to decrease late effects. The aim of the present study is to analyze the pattern of relapse and the correlation with dosimetric factors in pRMS treated with proton therapy. METHODS AND MATERIALS: This retrospective evaluation includes children treated in our institution for pRMS. Information on demographics, treatment, tumor characteristics, and toxicities and outcome was prospectively collected within the in-house registry. For patients presenting with local relapse, a fusion of the dosimetry with magnetic resonance imaging displaying site and geometry of recurrence was performed. RESULTS: Median follow-up time was 2.9 years (0.5-4.7). Forty-six patients were identified in our institution between July 2013 and November 2017. Main characteristics of patients were as follows: 56.5% male, median age 5.1 years (1.3-17.5), 39.1% alveolar histology, 26.1%, 52.2%, 8.7%, and 13% patients with subgroup risk classification D, E/F/G, H, or metastatic, respectively, median total prescribed dose 55.8 Gy (50.4-56.4). Estimated 2-year local control, metastasis-free survival, event-free survival, and overall survival were 83.8%, 87.8%, 76.9%, and 88.9%, respectively. No acute or late local toxicity exceeding grade 3 was observed. Risk-group was identified as prognostic factor for metastasis-free survival in univariate analysis but not in multivariate analysis (trend: P = .09). In this cohort, dosimetric factors did not correlate with outcome. Isolated local failure happened in 5 of the 11 relapses. Local relapses were matched with dosimetry for 6 patients: 4 of them occurred in the high dose volume and 2 in the intermediate or low dose volume. CONCLUSIONS: Proton therapy was effective and well feasible even in a critical cohort. Still, local relapse within the target volume of the radiation therapy remains an important issue in pRMS and new treatment strategies are needed.

An Adult Patient With Rare Primary Intracranial Alveolar Rhabdomyosarcoma.

We report a rare case of primary intracranial alveolar rhabdomyosarcoma (ARMS) in the right temporal lobe of a 51-year-old male. ARMS is one of 3 histological subtypes of rhabdomyosarcoma that most commonly presents in older children and younger adults. To our knowledge, there have been no prior published reports of primary intracranial ARMS in adults. Known cases of intracranial ARMS in adults are due to central nervous system (CNS) metastases from the head and neck and extremities. Diagnostic workup did not reveal any primary source outside the CNS. Given that risk factors for ARMS have not been studied in adults, it is difficult to ascertain what aspects of this patient's clinical history may have contributed to his diagnosis. Interestingly, he had prior history of traumatic brain injury requiring evacuation of a right fronto-temporal intraparenchymal hematoma.

Primary alveolar rhabdomyosarcoma of retrorectal-presacral space in an adult patient: A case report of an uncommon tumor with rare presentation.

RATIONALE: Rhabdomyosarcoma (RMS) has known as a highly malignant soft tissue sarcoma, representing 5% to 10% of all solid tumors in childhood. Alveolar rhabdomyosarcoma (ARMS) of the retrorectal-presacral space is an extremely rare lesion for adult, no study has been reported in the English literature. PATIENT CONCERNS: A 51-year-old male presented with abdominal pain for 1 month, significantly worse when having a bowel movement. DIAGNOSIS: Computed tomography (CT) and magnetic resonance imaging (MRI) of the pelvis showed a solid-cystic, enhancing lesion of dimension located in retrorectal-presacral space. The surgical specimen was reported as ARMS after pathological evaluation. INTERVENTIONS: The tumor was complete surgical resection, and after surgery, the patient was treated with combination chemotherapy. OUTCOMES: At 23 months follow up, the patient was asymptomatic with no evidence of metastases or local recurrence. LESSONS: Improvements in imaging in addition to early surgical intervention and chemotherapy treatment are crucial to improve survival chances against RMS.

Solitary Breast Metastasis From Rhabdomyosarcoma Shown on FDG PET/CT.

Rhabdomyosarcoma rarely metastasizes to breast. A 15-year-old adolescent girl underwent FDG PET/CT scan for staging a newly diagnosed alveolar rhabdomyosarcoma. The images showed not only the abnormal activity in the region of the right maxillary sinus, consistent with the known primary tumor, but also an intense focal activity in the right breast. The right breast lesion was excised, and the pathological results showed alveolar rhabdomyosarcoma.

18F-FDG PET/TC en la estadificación inicial de pacientes con rabdomiosarcoma alveolar / 18F-FDG PET/CT as staging of alveolar rhabdomyosarcoma

No disponible

Importance of whole-body imaging with complete coverage of hands and feet in alveolar rhabdomyosarcoma staging.

BACKGROUND: Alveolar rhabdomyosarcoma commonly arises in the extremities and is characterized by aggressive biology and high frequency of metastases. Whole-body imaging is increasingly employed in pediatric oncology but not recommended as standard in the staging of soft-tissue sarcomas. OBJECTIVE: After observing patients with a large symptomatic alveolar rhabdomyosarcoma lesion and a smaller silent lesion in the more distal part of an extremity we sought to estimate the frequency of this constellation. MATERIALS AND METHODS: We retrospectively evaluated the data of prospectively registered paediatric patients (age <21 years) with alveolar rhabdomyosarcoma in the SoTiSaR (Soft Tissue Sarcoma Registry) of the Cooperative Weichteilsarkom Studiengruppe (CWS) 09/2011-04/2015 with regard to whole-body imaging. RESULTS: Seventy-five patients were eligible. Images of 57 patients had been submitted for reference consultation, including 80 whole-body examinations in 36 patients. Among them were 5 patients (14%, 95% confidence interval 3-25%) who had been diagnosed because of a symptomatic lesion while an additional silent lesion in the distal part of an extremity had remained unnoticed and had only been detected by later whole-body imaging. It is noteworthy that in 42 (53%) of all 80 whole-body examinations, the hands and feet had been only partially covered or completely excluded. CONCLUSION: In alveolar rhabdomyosarcoma silent lesions can be overlooked when the distal parts of the limbs are not thoroughly examined and not completely covered by imaging. Missing them influences treatment decisions and prognosis. Our results should be considered when evaluating the potential role of whole-body imaging in rhabdomyosarcoma.

Alveolar rhabdomyosarcoma in an adolescent male patient - case report and current perspectives.

Alveolar rhabdomyosarcoma (RMS) is a common pediatric malignant mesenchymal tumor, representing half of soft tissue sarcomas and approximately 5% of all cancers. We present the case of an adolescent male patient treated in our Department for a tumoral mass located in the middle third of the forearm. Magnetic resonance imaging (MRI) and angiography-computed tomography (angio-CT) showed a large mass located in the muscles of the anterior compartment of the forearm. Surgical treatment consisted of tumor ablation including segmental resection of the radial and ulnar arteries and of the median nerve, followed by saphenous autograft vascular bypass. The treatment plan was based on tumor type, histological grading (high), age, tumor size greater than 5 cm, unfavorable location, postoperative tumor, node, metastasis (TNM) stage II, presence of microscopic tumoral tissue in the margins of the resected piece, lymph node metastases (N1) and bone metastases (M1) found on positron-emission tomography (PET)-CT according to the German soft tissue sarcoma study (CWS)-IV 2002 protocol. The chemotherapy used Carboplatin and Topotecan. Survival was less than two years after the initial presentation. Adolescent extremity masses should raise suspicion to exclude serious malignancy. Despite early diagnosis and use of multimodal therapies, alveolar RMS prognostic remains unpredictable.

Pleural Alveolar Rhabdomyosarcoma.

Alveolar rhabdomyosarcoma (ARMS) commonly occurring in children and adolescents, is a rare and aggressive soft tissue malignancy. Here, a rare case of pleural ARMS is reported in a boy presenting dyspnea for two weeks. The pathology of a biopsy specimen from pleura showed ARMS. PAX3-FKHR fusion protein is positive and the PAX7-FKHR fusion protein is negative as detected by fluorescence in situ hybridization. The prognosis of this patient was poor and nonresponsive to chemotherapy.