Carcinosarcoma Parotid in Progress

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    Carcinosarcoma of theParotid Gland: Case Report

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    Case Presentation

    49 y/o white male presented with 3 month

    historyof a rapidly enlarging right parotid

    mass, and a 1 month history offacial

    paralysis. I cant feel the right side of my face and feel

    dizzy

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    History

    PMHx: HTN

    PSHx: ORIF of tibia

    Allergies: NKDA

    Meds: none

    Social Hx: 3 packs/day for 30 years!

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    Physical Exam

    Physical Exam (abnormal findings):

    HEENT/Neuro:

    Facial asymmetry and abnormal facial strength (CN-VII

    palsy) 2 x 4 cm tender palpable mass overlying the right parotid

    gland, adherent/fixed to underlying tissue.

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    Work-up (outside hospital)

    CT scan of neck- Large necrotic mass involving both the superficial

    and deep aspects of the right parotid gland.

    Mass measures almost 4 cm in greatestdimension.

    FNA of the parotid mass-

    Most c/w Mucoepidermoid Carcinoma.

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    MRI Report

    4.5 x 2.7 x 2.9 cm mass partially replacing the

    the superficial and deep lobes of the right

    parotid gland.

    Intermediate signal on T1 and low signal on

    T2 indicating a high cellularity tumor.

    Base of skull (jugular foramen) shows lytic

    lesions with same characteristics as themass involving the parotid gland c/w tumor

    metastasis.

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    Assessment/Plan

    Right Parotidectomy

    Right Neck Dissection (levels I,II,III)

    Right jugular foramen approach with

    excision of posterior fossa lesion

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    Pathology

    02/15/2010

    Received:

    Right parotid gland.

    Right neck dissection, levels I-III.

    Facial nerve biopsy.

    Multiple right mastoid biopsies.

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    Pathology

    2.1 x 1.1 x 0.8 cm ill defined area, that grossly

    appeared confined to the parotid gland

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    IHC Stains

    Pan keratin: +++ neoplastic cells with glandular differentiation

    EMA:

    +++ neoplastic cells with glandular differentiation Mucicarmine

    + luminal material in foci of glandulardifferentiation.

    S-100 + cells in areas of solid growth pattern

    SMA + spindle cells in areas of solid growth pattern

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    Pathology

    Diagnosis:

    Carcinosarcoma of parotid gland.

    2.1 x 1.1 x 0.8 cm- pT2

    Margins uninvolved by tumor and no extension beyondparotid gland.

    + LVI

    + PNI

    Metastatic carcinoma in four of forty-two lymph

    nodes (4/42)- pN2b

    Metastatic carcinoma in right mastoid bone- pM1

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    Malignant Mixed Tumors of

    the Salivary Gland 3 Types

    1) Carcinoma ex pleomorphic adenoma

    Carcinoma arising in a mixed tumor

    Most common form

    2) Carcinosarcoma

    (true malignant mixed tumor)

    3) Metastasizing pleomorphic adenoma

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    Carcinosarcoma

    Definition:

    Biphasic tumor with both a carcinomatous and

    sarcomatous component.

    Thought to arise de novo (vs carcinoma expleomorphic adenoma which arises from pre-

    existent benign mixed tumor).

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    Epidemiology

    Rare and aggressive entity with only 70

    cases documented in literature.

    Ages (15-85 y/o)

    No predilection to gender

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    Etiology

    Two schools of thought

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    Histology

    Carcinomatous component:

    Poorly differentiated adenocarcinoma, undifferentiated

    carcinoma or squamous cell most common.

    May also include adenoid cystic, mucoepidermoid or

    salivary duct carcinoma.

    Sarcomatous component:

    Most common is chondrosarcoma.

    Others include spindle cell sarcomaNOS, fibrosarcoma,

    osteosarcoma, liposarcoma, and rhabdosarcoma.

    The relative proportion of the 2 components is

    variable, and either component may dominate the

    histologic picture.

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    Case Presentation

    49 y/o white male presented with 3 month

    history of a right parotid mass, and a 1

    month history of facial paralysis (described it

    as loss of feeling.

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    Case Presentation

    49 y/o white male presented with 3 month

    history of a right parotid mass, and a 1

    month history of facial paralysis (described it

    as loss of feeling.

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    Case Presentation

    49 y/o white male presented with 3 month

    history of a right parotid mass, and a 1

    month history of facial paralysis (described it

    as loss of feeling.

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    Work-up: FNA

    FNA of the parotid mass done at anotherinstitution was reported as aMucoepidermoid Carcinoma.

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    FNA

    Abundant amorphous debris with

    benign acinar cells and few atypical

    keratinized squamous cells, cannot rule

    out malignancy.

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    Work-up: Imaging

    CT: superior to physical exam and otherimaging modalities.

    Does not provide specific info regardinghistologic diagnosis, but provides info about

    bilateralism and benign vs. malignantmasses.

    MRI superior to CT in demonstrating internalarchitecture.

    Location in relationship to fascial planes andspaces (for operative planning).

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    MRI Report

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    MRI Report

    4.5 x 2.7 x 2.9 cm mass partially replacing the

    the superficial and deep lobes of the right

    parotid gland.

    Intermediate signal on T1 and low signal onT2 indicating a high cellularity tumor.

    Base of skull shows lytic lesions with same

    characteristics as the mass involving the

    parotid gland c/w tumor metastasis.

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    Embriology and Microscopic

    Anatomy

    Ectodermal origin.

    4-6th week of gestation.

    Serous and mucous cells, arranged inacini, drained by series of ducts.

    Parotid: serous acini.

    Submandibular: serous and mucinousacini.

    Minor SG: mucinous acini.

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    Cellular Origins of Salivary

    Gland Neoplasms

    Multicenter theory:

    Each type originates from a distinctive cell

    type.

    E.g.Whartins / oncocytic: striated duct

    cells.

    Acinic cell: acinar cells.

    Mixed: intercalated and myoepithelialcells.

    Dardick I: Mounting evidence against current histogenetic

    concepts for salivary gland tumorigenesis. Eur J

    Morphol 1998; 36:257-261.

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    Cellular Origins of Salivary

    Gland Neoplasms

    Bi-cellular reserve cell theory:

    All tumors arise from the basal cells ofeither the excretory or intercalated ducts.

    These cells act as reserves, with thepotential to differentiate into variousepithelial cell lines.

    E.g. pleomorphic adenomas and oncocytic

    tumors: intercalated ducts. SCC and mucoepidermoid tumors:

    excretory ducts.

    Batsakis JG: Histogenesis of salivary gland neoplasms: a

    postulate with prognostic implications. J Laryngol

    Otol 1989; 103:939-944.

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    Pathology

    Mucoepidermoid carcinoma.

    Low grade.

    Abundant infarction necrosis. 0/18 zone II lymph nodes.

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    Prognosis Mucoepidermoid

    Carcinoma

    Evenson JW. Malgnant neoplasms of the salivary glands.

    In: Thompson LDR (Eds.). Head and neck pathology.

    Churchill Livingston, Philadelphia, PA, pp. 321-370.

    40% incidence of local recurrence.

    15% incidence of spread to regional

    LNs and distant sites.

    5-year survival rate 80%.

    Female>male, mean age 47 years.

    Prognosis depends on clinical stage,site, grading and margins.