Case 205 - Discussion

Uploaded: 2008-04-05, Updated: 2008-04-05

   

CD138

CK8/18

Desmin

Desmin

MSA

MSA

Myoglobin

WT1

WT1

WT1

 

 

  • IMMUNOHISTOCHEMISTRY STAININGS:

    • CK8/18: Positive in epithelial component.

    • Vimentin: Positive in sarcomatous component.

    • CKAE1/3: Positive in epithelial component.

    • Desmin: Positive in sarcomatous component.

    • CD45: Negative

    • CD138: Positive in epithelial component.

    • Myogenin: Positive in majority of cells of sarcomatous component.

    • Myoglobin: Scattered positive in sarcomatous component.

    • SMA: Negative.

    • Cyclin D1: Weak scattered positive in sarcomatous component.

    • S100: Negative.

    • CD10: Weak positive in sarcomatous component.

    • MSA: Strong positive in sarcomatous component.

    • HMB45: Negative (nonspecific reactivity in necrotic tissues).

    • EMA: Positive in epithelial component.

    • SMM (myosin): Negative.

    • WT-1: Strong cytoplasmic positivity in sarcomatous component.

    • p53: Nuclear positivity in sarcomatous and epithelial component (90-100%).

     

  • ELECTRON MICROSCOPY:
    Ultrastructurally, the tumor is composed of loosely-grouped clusters of oval to spindle shaped cells embedded within a collagenous stroma. The nuclei display euchromatin and single, prominent nucleoli. The tumor cell cytoplasm contains abundant intermediate filaments, rough endoplasmic reticulum and scattered mitochondria and lysosomes. Many of the tumor cells contain cytoplasmic straight fibrils composed of thick and thin filaments with structures reminiscent of Z-bands. Rare primitive desmosome-like structures are present between some of these cells. No intracellular or intercellular lumens, microvilli, secretory granules, basal lamina, or well-developed cell junctions are seen.

     

  • CYTOGENETICS REPORT:
    Cytogenetic analysis revealed the presence of a hypertriploid clone characterized by loss of chromosomes 2, 14, 15, 17, 18, and 22; gain of chromosomes 3, 11, 12, 21 and several marker chromosomes; additional unknown material on 1p, 2q, 3p, 6p, 21q and 22q; and deletions of 12p and 17p. Eighteen cells were characterized by a normal female chromosome complement.

     

  • NOMENCLATURE:
    70-74,XXX,add(1)(p36.3),-2,add(2)(q33),+add(3)(p13),add(6)(p25),+11,+12,del(12)(p11.2)x2,
    -14,-15,add(16)(p12)x2,-17,del(17)(p12),-18,-18,+add(21)(q22),-22,add(22)(q13)x2,+8-12mar cp6]/46,XX[18]

     

  • FINAL DIAGNOSIS

    Malignant műllerian mixed tumor (MMMT) with rhabdomyosarcomatous component

Malignant Műllerian Mixed Tumor (MMMT)

The Key Features

 

CLINICAL FEATURES

 

GROSS FINDINGS

 

MICROSCOPIC FINDINGS

 

SUBTYPES

 

DIFFERENTIAL DIAGNOSES

 

IMMUNOHISTOCHEMISTRY AND SPECIAL STAINS

 

ELECTRON MICROSCOPIC FINDINGS

 

CYTOGENETIC STUDIES

 

TREATMENT AND PROGNOSIS

 

REFERENCES