Soft tissue tumors

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Soft Tissue Tumors - The Body's Lumps

  • Mesenchymal origin; classified by tissue type (fat, muscle, vascular).
  • Benign Tumors: "-oma"
    • Lipoma: Most common soft tissue tumor in adults. Mobile, soft, painless.
    • Rhabdomyoma: Benign skeletal muscle tumor. Often in the heart (associated with Tuberous Sclerosis).
  • Malignant Tumors: "-sarcoma"
    • Liposarcoma: Malignant fat tumor; lipoblasts are key.
    • Rhabdomyosarcoma: Most common soft tissue sarcoma in children.

Rhabdomyosarcoma often presents as a "grape-like" mass (sarcoma botryoides) in the vagina or bladder of young girls.

Benign Tumors - Mostly Harmless

Benign soft tissue tumors are common, slow-growing, well-differentiated masses with low metastatic potential. Management is typically surgical excision if symptomatic.

TumorTissue of OriginKey Features
LipomaAdipose TissueMost common soft tissue tumor in adults. Soft, mobile, painless.
FibromaFibroblasticFirm mass. Ovarian fibromas can cause Meigs syndrome.
LeiomyomaSmooth MuscleMost common in the uterus ("fibroids"). Whorled pattern of smooth muscle.
RhabdomyomaSkeletal MuscleRare. Cardiac type associated with Tuberous Sclerosis.
HemangiomaBlood VesselsCommon in infancy (strawberry hemangioma); often regress spontaneously.

Malignant Sarcomas - The Real Villains

  • General Features: Deep-seated, large (>5 cm), aggressive tumors. Tend to spread hematogenously (lungs are a common site of metastasis).
  • Liposarcoma:
    • Most common sarcoma in adults (40-60 years).
    • Key feature: Lipoblasts (cells with vacuolated, lipid-rich cytoplasm and a scalloped nucleus).
    • Well-differentiated type is associated with MDM2 amplification.
    • Myxoid liposarcoma with lipoblasts (H&E)
  • Rhabdomyosarcoma:
    • Most common soft tissue sarcoma in children (<15 years).
    • Key cell: Rhabdomyoblast (tadpole or strap cells; may show cross-striations). Stains for desmin, myogenin.
    • Embryonal subtype is most common; Alveolar subtype has t(2;13) or t(1;13) translocations and a worse prognosis.
  • Synovial Sarcoma:
    • Typically affects adolescents and young adults (15-40 years), often near large joints (e.g., knee).
    • Pathognomonic finding: t(X;18) translocation, resulting in an SS18-SSX fusion protein.

Diagnosis & Genetics - Cracking the Code

  • Initial Dx: H&P, imaging (MRI is key).
  • Tissue is the Issue: Biopsy (core needle > incisional/excisional) is mandatory before treatment.
  • Microscopy: Spindle cells, pleomorphism, mitotic activity.
  • Immunohistochemistry (IHC): Crucial for lineage.
    • Desmin: Muscle (rhabdomyo-)
    • S100: Neural (MPNST)
    • CD34: Vascular (angiosarcoma), GIST

Synovial Sarcoma: Characterized by the t(X;18) translocation, creating an SS18-SSX fusion gene.

  • Lipoma is the most common benign soft tissue tumor in adults; liposarcoma is the most common malignant, linked to MDM2 amplification.
  • Rhabdomyosarcoma is the most frequent soft tissue sarcoma in children, typically the embryonal type in the head, neck, or genitourinary tract.
  • Nodular fasciitis mimics sarcoma with its rapid growth and high cellularity but is a benign, self-limiting process.
  • Synovial sarcoma, despite its name, is unrelated to synovium and shows a characteristic t(X;18) translocation.
  • Desmoid fibromatosis is locally aggressive but non-metastatic, often associated with APC/β-catenin pathway mutations.

Practice Questions: Soft tissue tumors

Test your understanding with these related questions

An 11-year-old boy presents to your clinic after 4 months of pain and swelling in his thigh. His mother states that at first she thought his condition was due to roughhousing, but it hasn’t gone away and now she’s concerned. You perform an X-ray that shows an ‘onion skin’ appearance on the diaphysis of the femur. You are concerned about a malignancy, so you perform a PET scan that reveals lung nodules. Which of the following is most associated with this disease?

1 of 5

Flashcards: Soft tissue tumors

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Which type of esophageal cancer (SCC or adenocarcinoma) is associated with esophageal web (e.g. Plummer-Vinson syndrome)? _____

TAP TO REVEAL ANSWER

Which type of esophageal cancer (SCC or adenocarcinoma) is associated with esophageal web (e.g. Plummer-Vinson syndrome)? _____

Squamous cell carcinoma

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