Presentation
We present a case of RMS prostate in a young adult presenting as BOO. Case Report A 19-year-old boy presented with one month’s history of dysuria and increased frequency of urination followed by acute urinary retention. [ncbi.nlm.nih.gov]
Entire Body System
- Pathologist
Edited by four leading surgical pathologists - Noel Weidner, MD, Richard J. Cote, MD, Saul Suster, MD and Lawrence M. [books.google.com]
A biopsy is the removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. [my.clevelandclinic.org]
Biopsy: The only way to determine the exact tumor type is by removing a piece of the tumor and having a pathologist examine it under the microscope. This is known as a biopsy. [curesearch.org]
- Weight Loss
Most presents with obstructive symptoms; also hematuria and weight loss. LMS may grow large (mean 9 cm) and replace entire prostate; often is difficult to completely resect the tumor. [auanet.org]
He had no fever or weight loss or any history of high-risk behaviour. On examination, he had grade 3 prostatomegaly with no lymphadenopathy or hepatosplenomegaly. Blood counts, urine microscopy, and renal and liver function tests were normal. [ncbi.nlm.nih.gov]
- Anemia
Severe megaloblastic anemia: Vitamin deficiency and other causes Daniel S. Socha, MD, Sherwin I. DeSouza, MD, Aron Flagg, MD, Mikkael Sekeres, MD, MS and Heesun J. [ccjm.org]
Investigations showed normocytic normochromic anemia, hypercalcemia and hyperuricemia. Routine urine examination was normal. No laboratory evidence of tuberculosis, immuno-ompromised state or an autoimmune disorder was observed. [peertechz.com]
Patient’s symptoms were treated with analgesic to relieve pain, hematinic and blood transfusion for anemia. [article.sapub.org]
Investigations showed normocytic normochromic anemia, hypercalcemia and hyperuricemia. Routine urinalysis was normal. No laboratory evidence of tuberculosis, immuno-compromised state or an autoimmune disorder was observed. [casesjournal.biomedcentral.com]
If metastatic disease is present, symptoms of bone pain, respiratory difficulty (secondary to lung nodules or to pleural effusion), anemia, thrombocytopenia, and neutropenia may be present. [indiacancersurgerysite.com]
- Fishing
Fluorescence in situ hybridisation (FISH) studies in all three cases revealed a heterozygous microdeletion of their entire NF1 gene. [adc.bmj.com]
Urol. (2002) [ Pubmed ] Detection of aneuploidy and possible deletion in paraffin-embedded rhabdomyosarcoma cells with FISH. Lee, W., Han, K., Harris, C.P., Meisner, L.F. Cancer Genet. [wikigenes.org]
Use of a novel FISH assay on paraffin-embedded tissues as an adjunct to diagnosis of alveolar rhabdomyosarcoma. Lab Invest 2006;86:547–556. Google Scholar | ISI 37. [journals.sagepub.com]
[…] pediatric Embryonal Rhabdomyosarcoma is thought to be linked to the following factors and disorders: Inherited genetic defects Use of drugs like cocaine, marijuana; by the parent mother Carcinogens (some have been found on mice and certain types of fish [dovemed.com]
Genetic studies Fluorescent in situ hybridization (FISH) Reverse transcriptase–polymerase chain reaction (RT-PCR) assay: When FISH is unavailable or uninformative Imaging studies Plain radiography Computed tomography (CT) scanning Magnetic resonance imaging [emedicine.medscape.com]
- Feeding Difficulties
Case 2 : A male with a history of polyhydramnios, severe feeding difficulties, developmental delay and typical facial features for CS had a germline HRAS G12S mutation. [ashg.org]
Gastrointestinal
- Constipation
[…] pain when urinating constipation Rhabdomyosarcoma around the testicles can cause painless enlargement of the scrotum. [cancer.ca]
A 23-year-old male was referred to our department with symptoms of acute retention of urine, hematuria and constipation. [ccij-online.org]
- Pelvic Mass
Embryonal rhabdomyosarcoma of the prostate in a 19 year old Posted by: Philip Alex | Posted on: May 27th, 2006 | 0 Comments A 19 year old boy presented to us with edema, difficulty in passing urine and a pelvic mass extending till the umbilicus. [manalihospital.com]
Prostate tumors Prostate tumors produce large pelvic masses, sometimes with urethral strangury and constipation. Tend to occur in infants and in older age groups. Prostate tumors have a greater tendency to infiltrate adjacent tissues (than bladder). [pedsoncologyeducation.com]
It is characterized by a high degree of aggressiveness, rapid local growth with the formation of large pelvic masses, often leading to urethral obstruction, and systemic spread, commonly to the lungs, liver, and bone. [ccij-online.org]
- Failure to Thrive
Costello syndrome (CS) is a rare autosomal dominant, multiple congenital anomaly syndrome comprising failure to thrive, short stature, mental retardation, congenital heart disease, and a predisposition for embryonal rhabdomyosarcoma (ERMS). [ashg.org]
Eyes
- Diplopia
Embryonal 68%, alveolar 31%, others rare Different distribution in adults Pleomorphic 43%, embryonal 34%, alveolar 23% Usually presents as a painless mass May be symptomatic depending on organ of involvement (i.e. bladder - urinary dysfunction, orbit - diplopia [surgpathcriteria.stanford.edu]
Psychiatrical
- Aggressive Behavior
CONCLUSION: Coexistence of undifferentiated pleomorphic sarcoma and rhabdomyosarcoma shows highly aggressive behavior and its prognosis is extremely poor. [cancerindex.org]
Urogenital
- Prostatic Mass
The CT findings in the prostatic tumors included an enlarged, inhomogeneous prostatic mass with invasion of the perirectal fat. [journals.lww.com]
The prostatic acid phosphatase (PAP) were also within normal limits.Computed tomography of prostate showed homogenous soft tissue mass measuring 4x5cm in size (Figure 1). [peertechz.com]
After chemotherapy completion, CT and CT/PET showed a complete remission of pulmonary disease and partial response of the prostate mass. [ncbi.nlm.nih.gov]
- Enlargement of the Prostate
Digital rectal examination showed a rather smooth but enlarged prostate without palpable tumor mass. Ultrasound scan showed homogeneous prostate tissue and an enlarged volume of approximately 50–60 cm3. The clinical diagnosis was prostate adenoma. [ncbi.nlm.nih.gov]
[…] hypertrophy of the prostate benign prostatic hypertrophy BPH enlarged prostate nodular prostate polyp of prostate Type 1 Excludes benign neoplasms of prostate (adenoma, benign) (fibroadenoma) (fibroma) (myoma) ( D29.1 ) Type 2 Excludes malignant neoplasm [icd10data.com]
- Urinary Incontinence
Artificial sphincter inserted to cure his urinary incontinence failed because of ischemia secondary to cuff compression and scar tissue. He is alive today with a modified Koch pouch urinary diversion. [ncbi.nlm.nih.gov]
Intractable urinary incontinence in children: Management by implantable artificial urinary sphincter. Transplantation/Implantation Today. [jpedsurg.org]
Workup
Diagnosing rhabdomyosarcoma requires a careful and complete medical workup and examination of your child. [chop.edu]
The workup of these tumors typically includes the following studies: CT and MRI scans of affected region(s). Chest x-ray or CT scan of chest. Bone scan. Bone marrow biopsy. For genitourinary location: Cystoscopy (scope to look inside the bladder). [oncolink.org]
[…] resonance imaging (MRI) Bone scanning Ultrasonography Echocardiography Biopsy Open or core needle biopsy: To obtain tissue sampling for diagnosis and molecular studies Bone marrow aspiration and biopsy: To assess for metastatic spread to bone marrow See Workup [emedicine.medscape.com]
Pediatric Rhabdomyosarcoma Medscape Referenced article by Timothy Cripe MD, covering background, presentation, diagnosis, workup, treatment and follow-up. [cancerindex.org]
Treatment
During treatment for acute lymphatic leukemia at the age of 6 years, the patient had been exposed to a cumulative radiation dose of 10 Gy across his entire body. [ncbi.nlm.nih.gov]
Intensive chemotherapy as primary treatment for rhabdomyosarcoma of the pelvis. [jpedsurg.org]
1 Patterns of failure and toxicity profile following proton beam therapy for pediatric bladder and prostate rhabdomyosarcoma. 61 31397065 2019 2 Current treatment of pediatric bladder and prostate rhabdomyosarcoma (bladder preserving vs. radical cystectomy [malacards.org]
Three patients had an unsuccessful treatment attempt with sildenafil. 8 patients, including the three failures of oral PDE-5-inhibitors, were successfully treated with intracavernous injection of Alprostadil (mean post treatment SIEF-NS=38.25). [spuonline.org]
Commonly used terms in treatment Back to top Side effects Side effects and complications of treatment Treatment often causes side effects and these will be discussed before treatment starts. [childrenwithcancer.org.uk]
Prognosis
Prognosis is related to histologic type and tumor stage. Alveolar RMS has poorer prognosis. In contrast to pediatric RMS, adult RMS does not appear to respond to multimodal therapy and has a poorer prognosis. [auanet.org]
In adults prognosis is very poor, therefore early diagnosis is crucial. To date, only three cases of spindle cell subtype of embryonal rhabdomyosarcoma of the prostate in an adult have been published. [ncbi.nlm.nih.gov]
In contrast to the relatively good prognosis for treated rhabdomyosarcoma in children, the prognosis in adults is poor with most dying of disease in <2 years, despite multimodality therapy. [peertechz.com]
Etiology
Use Additional Use Additional Help Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. [icd10data.com]
Etiology The cause of rhabdomyosarcoma is unknown, but a translocation t(2;13) is very often associated with the alveolar type. Diagnostic methods Diagnosis is based on positive muscular markers and immunohistochemistry. [orpha.net]
Etiology of Prostate Sarcoma Li-Fraumeni syndrome: Li-Fraumeni syndrome is associated with mutation of the p53 suppressor gene: this leads to children with sarcoma whose mothers have a high risk of breast cancer and siblings with an increased cancer risk [urology-textbook.com]
Epidemiology
Back to 2016 Fall Congress Pediatric Bladder and Prostatic Sarcomas: A Surveillance, Epidemiology, and End Results (SEER) Analysis Casey A. Seideman, MD, Andrew Jacobs, MD, Lane S. Palmer, MD, Richard Glick, MD. [fallcongress.spuonline.org]
Essential features Small round cell to spindle cell tumor, variable cellularity Occurs in young patients and requires aggressive treatment Epidemiology Most common prostatic malignancy in children / infants Clinical features Firm, smooth enlargement of [pathologyoutlines.com]
It covers relevant literature by leading experts in basic science, molecular biology, epidemiology, cancer prevention, cellular imaging, staging, treatment, targeted therapeutics and innovative technologies. [books.google.ro]
Summary Epidemiology The annual incidence is 1/170,000. In children younger than 15 years, the annual incidence is estimated at 1/244,000. Clinical description The median age of diagnosis is 5 years. [orpha.net]
Pathophysiology
It brings together the pathophysiology of disease with detailed clinical guidance on diagnosis and management for the full range of childhood cancers, including aspects important in optimal supportive care. [books.google.com]
Internal Medicine (477) Paediatrics, Neonatology (424) Pathology and Forensic Medicine (323) Neurology (308) Physiology and Pathophysiology (276) Genetics (255) Surgery, Traumatology and Orthopaedics (219) Medical Chemistry and Biochemistry (216) Infectology [portal.mefanet.cz]
The National Rosacea Society Expert Committee updated its guidelines in 2017 on standard classification and pathophysiology of rosacea. [ncbi.nlm.nih.gov]
Standard classification and pathophysiology of rosacea: The 2017 update by the National Rosacea Society Expert Committee. Journal of the Academy of Dermatology. 2017;78:148. Briggs JK. Facial skin problems. In: Triage Protocols for Aging Adults. [mayoclinic.org]
Prevention
Treating allergies Avoiding cosmetics and skin products that cause allergic reactions can prevent a reddened nose. [medicalnewstoday.com]
Prevention Rhabdomyosarcoma can't be prevented. In rare cases, it's possible that a family history of cancer at a young age may increase the risk of RMS, but RMS is not inherited. And there is no known link to any lifestyle or environmental issues. [mayoclinic.org]
There’s no known way to prevent rhinophyma. However, some factors can increase blood flow to the surface of your skin and aggravate rosacea symptoms. [healthline.com]