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Clear Cell Sarcoma

DEFINITION AND PATHOGENESIS

  • AKA malignant melanoma of soft parts, clear cell sarcoma of tendon and aponeuroses
  • Not a malignant melanoma, although carries the term as a synonym, having a translocation between chromosomes 12 and 22 (or structural aberration of 22)
  • Along with alveolar soft parts sarcoma, clear cell sarcoma is driven by chromosomal translocations that activate members of the microphthalmia transcription factor (MITF) family
  • First described by Enzinger in 1965

IMPORTANCE

  • Although rare, a common soft tissue sarcoma of the foot and ankle
  • Reported as accounting for 5% of pediatric renal tumors and up to 7% of all renal tumors

CLINICAL FEATURES

  • Usually affects patients 20-40 years of age (6-85 yrs reported)
    • Mean age 36 years of age
  • F>M
  • Foot and ankle lesions are most common, followed by knee, thigh, and hand
  • Toes may become spread apart by mass affect
  • LE 75%, UE 22%
  • Mean size 4.5 cm in diameter at presentation
  • Painless in 50%
  • 20 month - 9.5 months to 2 year average duration of sxs
  • ± reddish discoloration of overlying skin
  • Unusual locations

RADIOLOGIC FEATURES

  • X-rays may show a soft-tissue mass, without calcification, ± underlying bony erosions
  • MRI most helpful in delineating soft tissue extent and proximity to neurovascular structures
    • T1-weighted image: non-homogenous mass nearly isointense to hyperintense (due to melanin--52%) with muscle
    • T2-weighted image: high signal intensity
    • Primary involvement of a tendon, ligament, or aponeurosis with fusiform growth proximally and distally along and within the fibrous structure best demonstrated with MR

GROSS PATHOLOGY

  • Usually grows along tendons or aponeuroses (like epithelioid sarcoma)
  • Lobulated and circumscribed but not usually encapsulated, with a grayish-white (±?hemorrhagic areas) rubbery surface, usually 2-6cm in size, usually sparing the skin, although in larger lesions, invading into subcuta-neous or subcuticular tissues
  • Rubbery to firm

HISTOLOGIC/MOLECULAR FEATURES

  • Distinct from melanoma, lacking epidermal involvement and junctional changes, with round or polygonal to fusiform or spindle cells with clear cytoplasm (due to intracellular glycogen)(although the cytoplasm frequently is not clear) arranged in nestlike aggregatesand few mitoses (not numer-ous)
  • Delicate fibrocollagenous stroma blends into adjacent tendons or fascial structures
  • Nests of cells enveloped by fibrous septae
  • Scattered multinucleated (wreath-like) giant cells (2/3) have peripherally placed nuclei appearing like the mononuclear tumor cells
  • PAS/diastase highlights fibrous septae
  • Recurrent lesions may show more pleomorphic changes
  • Cytology
    • Highly cellular with discohesive round/polygonal cells with eccentric nuclei/prominent nucleoli
    • Foamy-granular appearance reminiscent of a "tigroid" background (seen in seminomatous germ cell tumors) reported on Diff Quik staining
  • Special stains:
    • intracellular mucin
    • ±?alcian blue, hyaluronidase-sensitive for mucoid material in the stroma
    • + reticulin staining
    • 50% with intracellular melanin with Fontana's stain (60-75% with Warthin-Starry stain), may be more prominent in metastases; hemosiderin may cause stain to be falsely + so Fe+ staining is needed
  • Immunohistochemistry stains:
    • + S-100 (usually)
    • + HMB-45
    • + MITF
    • Alcian blue + for mucin extracellularly
    • Diastase-sensitive PAS + (glycogen contributes to the "clear cell")
    • + for melanoma-associated antigen
    • ± neuron-specific enolase
    • ± Leu-7
    • ± LN3
    • Vimentin weakly +
    • Cytokeratin weakly +
    • Cyclin D1 +
      • Reported helpful in distinguishing clear cell sarcoma in renal location
        • (From Wilm's tumor, rhabdoid tumor, and some congential mesoblastic nephroma)
        • (Not from Ewing sarcoma and neuroblastomas)
  • Cytogenetics
    • 60-75% with t(12;22)(q13;q12-3) (distinct from melanoma which most commonly involves chromosomes 1, 5, and 6)
    • Chimeric product of EWS/ATF-1 (Ewing's sarcoma oncogene-activating transcription factor 1 fusion transcripts) is produced
      • Activates the melanocyte transcription factor MITF
        • Activates transcription of c-Met
          • (Oncogenic receptor tyrosine kinase)
      • Soft tissue tumors associated with the EWSR1translocation and associated translocation
        • Angiomatoid fibrous histiocytoma
          • t(12;22)(q13;q12), EWSR1-ATF1
            • Type 1 chimeric transcript (EWSR1 exon 8/ATF 1 exon 4)
            • Type 2 chimeric transcript (EWSR1 exon 7/ATF1 exon 5)
            • Type 3 chimeric transcript (EWSR1 exon 10/ATF1 exon 5)
        • (Gastrointestinal clear cell sarcomaalso reported with EWSR1-ATF1)
          • Stomach location reported in a 10-yr-old girl
        • Extraskeletal myxoid chondrosarcoma

        • t(9;22)(q22;q12), EWSR1-NR4A3
        • Ewing sarcoma/PNET

          • t(11;22)(q24;q12), EWSR1-FLI1
          • t(23;ww)(q22;q12), EWSR1-ERG
          • t(20;22)(q13;q12), EWSR1-NFATC2
          • t(2;22)(q33;q12), EWSR1-FEV
          • t(7;22)(p22;q12), EWSR1-ETV1
          • t(17;22)(q12;q12), EWSR1-E1AF
          • t(17;22)(q31;q12), EWSR1-SP3
          • t(1;22)(p36.1;q12), EWSR1-ZNF278
          • t(6;22)(p21;q12), EWSR1-POU5F1
        • Myxoid-round cell liposarcoma
        • t(12;22)(q13;q12), EWSR1-DDIT3
  • EM:
    • Chromatin evenly distributed at the nuclear membrane, swollen mitochondria, RER aggregates, glycogen, melanosomes and premelanosomes

DIFFERENTIAL CLINICOPATHOLOGIC DIAGNOSIS

  • Synovial sarcoma
  • Fibrosarcoma
  • Malignant peripheral nerve sheath tumors (eg, epithelioid neurofibrosarcoma)
  • Acral lentiginous melanoma
  • Renal cell carcinoma metastasis
  • Wilms tumor (in renal location)
  • Epithelioid leiomyosarcoma
  • Epithelioid sarcoma
  • GCTTS
  • Malignant melanoma
  • Osteoclast-rich tumor of the gastrointestinal tract with features resembling clear cell sarcoma

DISEASE COURSE AND TREATMENT

  • LR is common prior to metatstases (averages 4.2 yrs, 7.5 yrs reported in children < 18 yrs of age), because of tracking along fibrous planes, unrecognized at the time of resection, and not appreciated on histologic evaluation (may occur even after ?10 yrs)
  • Tumor size (>5cm) predictive of metastases
    • Tumor size ≤ 2.5 cm positive prognostic factor
    • Tumor stage ≤ IIA positive prognostic factor
  • Pulmonary metastases may occur years after the originaly surgical resection (average 3.5 yrs)
  • Metastases also occur in the lymph nodes (17.6%) and skeleton (15-26%)(consider bothskeletal survey and bone scan to detect), liver, occasionally to the heart
  • Unusual location for initial metastasis
  • 5 yr survival survival 67%, 10YS: 33%, 20YS: 10%
  • Wide to radical excision ± XRT (up to 66.2 Gy reported)
  • Carefully evaluated lymph nodes, consider regional lymph node excision
  • Chemotherapy has been utilized
    • Response rate of 4% with ifosfamide reported
      • Secondary agents maintaining stable disease
        • Cisplatin
        • Vinblastine
        • Sorafenib
        • Sirolimus
      • Non-effective agents reported
        • Cisplatin/vinblastine/DTIC
        • Interferon
        • Vincristine
        • Temozolamide/thalidomide
        • IGF1-R antibody
      • Palliative agents with tumor response
        • Sunitinib
          • Radiologic, metabolic and pathologic response reported
      • Novel chemotherapeutic approaches
        • c-Met expression blockade
          • SU11274
            • Small-molecule inhibitor
          • AMG 102
            • Neutralizing antibody to c-Met ligand HGF (hepatocyte growth factor)
      • Immunotherapy
        • Phase 1 clinical trial of vaccination with irradiated, autologous sarcoma cells engineered by adenoviral mediated gene transfer to secrete granulocyte-macrophage cOccolony stimulating factor (GM-CSF)
  • Vascular invasion a poor prognosis
  • Serum neuron-specific enolase has been reported as a useful biomarker of disease progression

SPECIAL CONSIDERATION

  • Ewing-like clear cell sarcoma of soft parts

  • Clear Cell Sarcoma-like Tumor of the Gastrointestinal Tract

    • Occurs in the wall of the small bowel, stomach, or large bowel

    • Predominantly occurs in young adults
    • Often presents with metastatic disease
    • Composed ofmedium-sized primitive ovoid or epithelioid cells with pale or clear cytoplasm
      • Arranged in sheets or in papillary or alveolar architectures
    • S-100 +
    • + for neuroendocrine markers
    • Association with EWSR1-CREB1 gene fusions, less frequently with EWSR1-ATF1 fusions
  • Clear Cell Sarcoma of Bone

    • A primary clear cell sarcoma of the sternum has been reported
  • Animal Model

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