RHABDOMYOSARCOMA (MALIGNANT TUMOR) IS PRESENTED BY K.TRIVENI FROM CHILKUR BALAJI COLLEGE OF PHARMACY HYDERABAD,TELANGANA

1. RHABDOMYOSARCOMARHABDOMYOSARCOMA
(MALIGNANT TUMOR)
Under the guidance of:Under the guidance of:
K.K. SEETARAMSEETARAM SWAMYSWAMY, M.Pharm.,, M.Pharm.,
ASSISTANT PROFESSORASSISTANT PROFESSOR
Presented by:
TRIVENI KARLAPUDI
13GD1R0026
IVth B-PHARMACY

2. WHAT IS RHABDOMYOSARCOMAWHAT IS RHABDOMYOSARCOMA
 Rhabdomyosarcoma or Rms, is a cancer made up of cells that normally
develop into skeletal muscles.
 The body has 3 main types of muscles.
Skeletal (voluntary) muscles are muscles that we control to move parts
of our body.
Smooth muscle is the main type of muscle in internal organs (except for
the heart). For example, smooth muscles in the stomach and intestines
push food along as it is digested. We do not control this movement.
Cardiac muscle is the main muscle type in the heart.

3. EPIDEMIOLOGY OF RHABDOMOYSARCOMA:EPIDEMIOLOGY OF RHABDOMOYSARCOMA:
• Rhabdomyosarcoma is the most common soft-tissue sarcoma in children as
well as the third most common solid tumor in children.
• RMS has been correlated with familial cancer syndromes and congenital
abnormalities including
 neurofibromatosis type -1,neurofibromatosis type -1,
 cardio-facio-cutaneous syndrome, and Costello syndromecardio-facio-cutaneous syndrome, and Costello syndrome.
• Nearly 80% of genitourinary tract (GU) RMS are embryonal in nature The
botrytis variant of RMS, characterized by a protuberant mass arising from the
bladder or vagina, is found almost exclusively in infants.

4. PATHOLOGY OF RHABDOMOYSARCOMA:PATHOLOGY OF RHABDOMOYSARCOMA:
• RMS falls into the broader category of small, round, blue-cell tumors of
childhood.
• To identify the characteristics features of RMS:
• To identify the features of RMS ,we use
Light microscopy,
A) Alveolar RMS. Small, round cells lined up along spaces
reminiscent of pulmonary alveoli.
B) Embryonal. Spindle-shaped cells with a stroma-rich appearance.
 C) Solid alveolar. Alveolar variant lacking in septations.
D) Leiomyomatous. Embryonal variant predominantly paratesticular
in origin. Reprinted with permission from.
Immunohistochemistry,
electron microscopy,
and molecular genetic techniques
• Those can be used to identify characteristic features of RMS consistent
with a myogenic lineage.

5. Signs and SymptomsSigns and Symptoms
 RMS can occur in almost any soft-tissue site in the body.
 The most common primary sites of RMS are
 genitourinary (24%),
 parameningeal (16%),
 extremity (19%), orbit (9%),
 other head and neck (10%),
 and miscellaneous other sites (22%).

6. TYPES OF RMS:TYPES OF RMS:
• Photomicrograph showing nodules of tumor cells separated by hyalinised
fibrous septae (50×, HE stain).
• Rhabdomyosarcoma can be generally divided into
three histological subsets:
1.Embryonal rhabdomyosarcoma:1.Embryonal rhabdomyosarcoma:
Embryonal rhabdomyosarcoma (ERMS) is the most common
histological variant, comprising approximately 60-70% of childhood
cases
It is most common in children 0–4 years old, with a maximum
reported incidence of 4 cases per 1 million children.
ERMS is characterized by spindle-shaped cells with a stromal-rich
appearance
ERMS also has two defined subtypes, boytroid and spindle cell
ERMS, and these subtypes are associated with a favorable prognosis

7. 2.Alveolar rhabdomyosarcoma:2.Alveolar rhabdomyosarcoma:
It is the second most common type. ARMS comprises approximately
20-25% of RMS-related tumors, and it is equally distributed among
all age groups.
 it is the most common form of RMS observed in young adults and
teenagers, who are less prone to the embryonal variant.
 This type of RMS is characterized by densely-packed, round cells
that arrange around spaces similar in shape to pulmonary alveoli.
 It is also typically more aggressive than ERMS.
3.Anaplastic rhabdomyosarcoma:3.Anaplastic rhabdomyosarcoma:
Anaplastic rhabdomyosarcoma is defined by the presence of
anaplastic cells with large, lobate hyperchromatic nuclei and
multipolar mitotic figures
The anaplastic cells may be diffuse or localized, with the diffuse
variation correlating to a worse prognosis. It occurs most often in
adults, rarely in children

8. DIAGNOSIS OF RHABDOMOYSARCOMA:DIAGNOSIS OF RHABDOMOYSARCOMA:
• Difficult to diagnosis due to its similarities to other cancers and varying
levels of differentiation. It is loosely classified as one of the “small,
round, blue-cell cancer of childhood” due to its appearance on an H&E
stain
• The defining diagnostic trait for RMS is confirmation of malignant
skeletal muscle differentiation with myogenesis (presenting as a plump,
pink cytoplasm) under light microscopy.
• The alveolar type of RMS tends to have stronger muscle-specific
protein staining
• Classification into types and subtypes is accomplished through further
analysis of cellular morphology (alveolar spacings, presence of
cambium layer, aneuploidy, etc.) as well as genetic sequencing of tumor
cells.
• Radiologic evaluation should include plain radiographs of the primary
site as well as a computed tomography (CT) scan of the primary and
surrounding structures

9. STAGING OF RHABDOMOYSARCOMA:
• Following diagnosis and histopathological analysis, the patient will usually
under go magnetic resonance imaging (MRI), ultrasonography, and a
bone scan in order to determine the extent of local invasion and metastasis.
• It utilizes a modified TNM (tumor-nodes-metastasis) system originally
developed by the IRSG.
• This system accounts for tumor size (> or <5 cm), lymph node
involvement, tumor site, and presence of metastasis and It grades on a
scale of 1 to 4 based on these criteria.
• The current Children's Oncology Group protocols for the treatment of
RMS categorize patients into one of four risk categories based on tumor
grade and clinical group, and these risk categories have been shown to be
highly predictive of outcome.

10. Treatment of RhabdomyosarcomaTreatment of Rhabdomyosarcoma
• Treatment of rhabdomyosarcoma is a multidisciplinary practice
involving the use of surgery, chemotherapy, radiation, and
possibly immunotherapy.
• Surgery is generally the first step in a combined therapeutic approach
• There are two main methods of chemotherapy treatment for RMS.
The VAC regimen, consisting of vincristin, actinomyocin-D,
and cyclophosphamide, and
The IVA regimen, consisting of ifosfamide, vincristin, and
actinomyocin - D.
• Radiation therapy, which kill cancer cells with focused doses of
radiation, is often indicated in the treatment of RMS and this therapy is
used when resecting the entirety of the tumor would involve
disfigurement or loss of important organs

11. Future ChallengesFuture Challenges
• For patients with localized disease who have an excellent chance of cure,
the development of less toxic therapy has the potential for decreasing
long-term morbidity and reducing the risk for secondary neoplasms.
• Patients with metastatic disease continue to do poorly despite dose
intensification, the use of multiagent chemotherapy, aggressive local
control, as well as other strategies, such as ABMT.
• Anti-angiogenic agents or agents aimed at specific targets involved in
metastatic behavior are potential modalities that are being explored.
• In animal models, the anti-angiogenic agent TNP-470 and an antibody to
vascular endothelial growth factor have been shown to inhibit RMS tumor
• Immunotherapy is a more recent treatment modality that is still in
development. This method involves recruiting and training the patient's
immune system to target the cancer cells.
TREATMENT:TREATMENT:

12. Conclusion:Conclusion:
• Rhabdomyosarcoma is a rare aggressive tumor manifesting in
children and young adults
• Localized forms have a good prognosis whereas metastatic
tumors show very poor results. A well-defined treatment
based on surgery and chemotherapy yields good results
• Radiotherapy is indicated in cases of residual foci and
retroperitoneal lymphnodes and strict follow-up has to be
instituted for all patients

13. REFERENCE:
 American Cancer Society. Cancer Facts & Figures 2014. Atlanta, Ga: American Cancer
Society; 2014
 Breitfeld PP, Meyer WH. Rhabdomyosarcoma: New windows of opportunity.
Oncologist. 2005;10:518-527.
 Dome JS, Rodriguez-Galindo C, Spunt SL, Santana VM. Chapter 95: Pediatric solid
tumors. In: Niederhuber JE, Armitage JO, Doroshow JH, Kastan MB, Tepper JE, eds.
Abeloff’s Clinical Oncology. 5th ed. Philadelphia, Pa: Elsevier; 2014.
 Hawkins DS, Spunt SL, Skapek SX; COG Soft Tissue Sarcoma Committee. Children’s
Oncology Group’s 2013 blueprint for research: Soft tissue sarcomas. Pediatr Blood Cancer.
2013:60:1001-1008.
 Meza JL, Anderson J, Pappo AS, Meyer WH. Analysis of prognostic factors in patients
with nonmetastatic rhabdomyosarcoma treated on Intergroup Rhabdomyosarcoma Studies
III and IV: The Children’s Oncology Group. J Clin Oncol. 2006;24:3844-3851.

15. THANK YOUTHANK YOU