Case No.: D-004

Diagnosis: Adult rhabdomyoma

Organ: Tongue

Last Updated: 12/21/2011

 

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Hematoxylin & eosin

Area 1: The tumor cells are large, polygonal. Cells with solid, granular cytoplasm and cells with large vacuoles intermingled with each other and forms a check board pattern. The nuclei are small, bland, and eccentrically placed.

Hematoxylin & eosin

Area 2: Occasional cells with brightly eosinophilic, cytoplasmic rod shape crystaline material (arrow). These rods probably represent hypertrophic Z-bands.

History: The patient was a 75 year-old man who presented with a pedunculated, submucosal tumor at the base of the tongue as illustrated in the CT scan. A fine needle aspiration was performed and an excision was subsequently performed. The excised specimen was a 6.0 x 3.5 x 2.0 cm mass with solid cut surface that was free of cystic changes or necrosis.

CT Scan

 

Histologic Highlights of this Case:

  • Histologically, the tumor is a well circumscribed neoplasm with pushing margin. The tumor cells are large and polygonal with one or two small, bland, and eccentrically placed nuclei. While a significiant number of the tumor cells have a fine granular, amphophilic to eosinophilic cytoplasm , many tumor cells are dominated by a large centrally located vacule that pus the cytoplasm to the periphery to become a rim (Area 1). In some cells, bright eosinophilic rods can be seen (Area 2) and these material represents Z-band material. Prominent nucleoli are noted in some of the tumor cells. Mitoses are not readily seen and there is no hemorrhage or necrosis.

  • Some of the vacuolated cells has a central mass of stellate cytoplsm with thin strands connected to a condensed rim of cytoplasm at the periphery (spider cells).

  • The tumor cells are separated by thin fibrous septa and narrow vascular challesls.

  • Results of immunohistochemistry and special stain are as follow:

          Immunohistochemistry:

 

·         Muscle specific actin and smooth muscle actin: Positive in tumor cells.

·         Desmin and myogenin: Negative in tumor cells.

·         Vimentin: Negative in tumor cells.

·         CD163: Negative in tumor cells.

·         PGP9.5: Negative in tumor cells.

·         S100 protein: Negative in tumor cells.

·         Cytokeratins : Negative in tumor cells

·         Epithelial membrane antigen: Negative in tumor cells.

 

          Special stain:

 

·         Periodic Acid Schiff (PAS): Negative in tumor cells

·         PAS with diastase: Negative in tumor cells

 

Comment:

  • Striations can be seen in most case but does not seem to be a prominent structure in this case.

  • The major differential diagnosis of this case is granular cell tumor. Tongue is a common location for granular cell tumor. Although granular cell tumors also have fine, granular cytoplasm and large polygonal cells with small nuclei, they do not have the large cytoplasmic vacuoles. Granular cell tumors are strongly positive for PAS stain. Also, granular cell tumors are strongly positive for S100 protein. Also, since granular cell tumor has substantial amount of lysosomes, these tumors are also positive for CD163. Immunohisochemically, adult rhabdomyoma are positive for muscle-specific actin and desmin and less commonly for vimentin, S-100 protein, and Leu-7. The other less common entities for differential diagnosis include hibernoma (with numerous small cytoplasmic vacuoles), crystal-storing histiocytosis associated with lymphoplasmacytic neoplasm (crystal storing cells and histiocytes are positive for PAS and CD68 but negative for skeletal muscle markers and S-100 protein), paraganglioma, and cardiac rhabdomyoma.

Original slide is contributed by Dr. Kar-Ming Fung, University of Oklahoma Health Science Center, Oklahoma, U.S.A.

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