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Salivary Gland Hyalinizing Clear Cell Carcinoma.

Lin JC, Liao JB, Fu HT, Chang TS, Wang JS - J Pathol Transl Med (2015)

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology and Lab Medicine, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan.

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Hyalinizing clear cell carcinoma (HCCC) is a rare malignant salivary gland tumor that was characterized as a distinct entity by Milchgrub et al. in 1994... The diagnosis was soon revised to HCCC after recognizing this new entity... The clear cells contained abundant glycogen highlighted by a diastase sensitive periodic acid-Schiff positive reaction (Fig. 1F, G)... The result of the mucicarmine stain for mucin was negative... HCCC was first described by Milchgrub et al. in 1994 as a rare salivary gland carcinoma made up of clear cells forming cords and nests in a hyalinized stroma... HCCC is currently classified by the World Health Organization (WHO) as a diagnosis of exclusion: “clear cell carcinoma, not otherwise specified. ” Many small case series and case reports have been described, but none added new insights into HCCC until Antonescu et al. recently identified a consistent EWSR1-ATF1 fusion in HCCC... In the differential diagnosis, EMC has a prominent ductal structure of tumor cells and does not show characteristic dual hyalinizing and fibrocellular stroma of HCCC... Molecular genetic methods have become an integral part of modern oral pathology and play an important role in helping to categorize cases that defy traditional morphologic analysis... In the molecular era, “NOS” is no longer fitting for HCCC... The term “NOS” is at odds with the presence of the specific EWSR1-ATF1 fusion that clearly sets HCCC apart from other salivary neoplasia... Adding “hyalinizing” is also recommended, even though this feature is not present in all cases... The benefit of its inclusion is the mental association with a salivary clear cell malignancy.

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(A) Hyalinizing clear cell carcinoma (HCCC) showing cords and nests of tumor cells with perineural invasion. The tumor cells have a clear cytoplasm (B) and are embedded in a characteristic dual hyaline and fibrocellular stroma (C). (D) Almost all clear cells are positive for p63. (E) The tumor cells are negative for CD10 while the myofibroblastic spindle cells in the stroma are positive for CD10. (F, G) Tumor cells with clear cytoplasm contain abundant glycogen which is positive for periodic acid–Schiff and sensitive to diastase digestion. (H) Fluorescence in situ hybridization shows a break-apart signal pattern in 25% of the tumor cells confirming the presence of EWSR1 rearrangement.
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f1-jptm-49-4-351: (A) Hyalinizing clear cell carcinoma (HCCC) showing cords and nests of tumor cells with perineural invasion. The tumor cells have a clear cytoplasm (B) and are embedded in a characteristic dual hyaline and fibrocellular stroma (C). (D) Almost all clear cells are positive for p63. (E) The tumor cells are negative for CD10 while the myofibroblastic spindle cells in the stroma are positive for CD10. (F, G) Tumor cells with clear cytoplasm contain abundant glycogen which is positive for periodic acid–Schiff and sensitive to diastase digestion. (H) Fluorescence in situ hybridization shows a break-apart signal pattern in 25% of the tumor cells confirming the presence of EWSR1 rearrangement.

Mentions: The tumor comprised of cords and nests of tumor cells with skeletal muscle infiltration and perineural invasion (Fig. 1A). The tumor cells had clear and pale eosinophilic cytoplasm (Fig. 1B), fine nuclear chromatin with occasional small nucleoli, and no mitotic figure. The tumor cells were embedded in a characteristic dual hyaline and fibrocellular stroma (Fig. 1C). No ductal formation was seen. On immunohistochemical stains, the tumor cells were positive for CKAE1/3 and p63 (Fig. 1D), but negative for myoepithelial cell markers, such as SMA, CD10, S100, myosin, calponin, glial fibrillary acidic protein (GFAP), muscle specific actin (MSA), and desmin. The spindle cells in the fibrocellular stroma were positive for SMA and CD10 (Fig. 1E) but negative for CKAE1/3, p63, myosin, calponin, GFAP, MSA, S100, and desmin. The clear cells contained abundant glycogen highlighted by a diastase sensitive periodic acid-Schiff positive reaction (Fig. 1F, G). The result of the mucicarmine stain for mucin was negative. Fluorescence in situ hybridization for EWSR1 showed a break-apart signal pattern in 25% of the tumor cells (Fig. 1H), confirming the presence of EWSR1 gene rearrangement. Since the margin was focally involved by the tumor, the patient received further excision with safe resection margin, which showed no residual tumor.


Salivary Gland Hyalinizing Clear Cell Carcinoma.

Lin JC, Liao JB, Fu HT, Chang TS, Wang JS - J Pathol Transl Med (2015)

(A) Hyalinizing clear cell carcinoma (HCCC) showing cords and nests of tumor cells with perineural invasion. The tumor cells have a clear cytoplasm (B) and are embedded in a characteristic dual hyaline and fibrocellular stroma (C). (D) Almost all clear cells are positive for p63. (E) The tumor cells are negative for CD10 while the myofibroblastic spindle cells in the stroma are positive for CD10. (F, G) Tumor cells with clear cytoplasm contain abundant glycogen which is positive for periodic acid–Schiff and sensitive to diastase digestion. (H) Fluorescence in situ hybridization shows a break-apart signal pattern in 25% of the tumor cells confirming the presence of EWSR1 rearrangement.
© Copyright Policy
Related In: Results  -  Collection

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Show All Figures
getmorefigures.php?uid=PMC4508576&req=5

f1-jptm-49-4-351: (A) Hyalinizing clear cell carcinoma (HCCC) showing cords and nests of tumor cells with perineural invasion. The tumor cells have a clear cytoplasm (B) and are embedded in a characteristic dual hyaline and fibrocellular stroma (C). (D) Almost all clear cells are positive for p63. (E) The tumor cells are negative for CD10 while the myofibroblastic spindle cells in the stroma are positive for CD10. (F, G) Tumor cells with clear cytoplasm contain abundant glycogen which is positive for periodic acid–Schiff and sensitive to diastase digestion. (H) Fluorescence in situ hybridization shows a break-apart signal pattern in 25% of the tumor cells confirming the presence of EWSR1 rearrangement.
Mentions: The tumor comprised of cords and nests of tumor cells with skeletal muscle infiltration and perineural invasion (Fig. 1A). The tumor cells had clear and pale eosinophilic cytoplasm (Fig. 1B), fine nuclear chromatin with occasional small nucleoli, and no mitotic figure. The tumor cells were embedded in a characteristic dual hyaline and fibrocellular stroma (Fig. 1C). No ductal formation was seen. On immunohistochemical stains, the tumor cells were positive for CKAE1/3 and p63 (Fig. 1D), but negative for myoepithelial cell markers, such as SMA, CD10, S100, myosin, calponin, glial fibrillary acidic protein (GFAP), muscle specific actin (MSA), and desmin. The spindle cells in the fibrocellular stroma were positive for SMA and CD10 (Fig. 1E) but negative for CKAE1/3, p63, myosin, calponin, GFAP, MSA, S100, and desmin. The clear cells contained abundant glycogen highlighted by a diastase sensitive periodic acid-Schiff positive reaction (Fig. 1F, G). The result of the mucicarmine stain for mucin was negative. Fluorescence in situ hybridization for EWSR1 showed a break-apart signal pattern in 25% of the tumor cells (Fig. 1H), confirming the presence of EWSR1 gene rearrangement. Since the margin was focally involved by the tumor, the patient received further excision with safe resection margin, which showed no residual tumor.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology and Lab Medicine, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan.

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

Hyalinizing clear cell carcinoma (HCCC) is a rare malignant salivary gland tumor that was characterized as a distinct entity by Milchgrub et al. in 1994... The diagnosis was soon revised to HCCC after recognizing this new entity... The clear cells contained abundant glycogen highlighted by a diastase sensitive periodic acid-Schiff positive reaction (Fig. 1F, G)... The result of the mucicarmine stain for mucin was negative... HCCC was first described by Milchgrub et al. in 1994 as a rare salivary gland carcinoma made up of clear cells forming cords and nests in a hyalinized stroma... HCCC is currently classified by the World Health Organization (WHO) as a diagnosis of exclusion: “clear cell carcinoma, not otherwise specified. ” Many small case series and case reports have been described, but none added new insights into HCCC until Antonescu et al. recently identified a consistent EWSR1-ATF1 fusion in HCCC... In the differential diagnosis, EMC has a prominent ductal structure of tumor cells and does not show characteristic dual hyalinizing and fibrocellular stroma of HCCC... Molecular genetic methods have become an integral part of modern oral pathology and play an important role in helping to categorize cases that defy traditional morphologic analysis... In the molecular era, “NOS” is no longer fitting for HCCC... The term “NOS” is at odds with the presence of the specific EWSR1-ATF1 fusion that clearly sets HCCC apart from other salivary neoplasia... Adding “hyalinizing” is also recommended, even though this feature is not present in all cases... The benefit of its inclusion is the mental association with a salivary clear cell malignancy.

No MeSH data available.


Related in: MedlinePlus