11q21

MAM-3,MAM2,MAM3,MLL-MAML2

mucoepidermoid carcinoma with t(11;19)(q21-22;p13).

- Most common type of malignant salivary gland tumor;
The t(11;19) was found in samples from the three different sites. - Rare tumour in the thyroid.

- Mucoepidermoid carcinomas have an unpredictable behaviour.
- The CRTC1-MAML2 fusion transcript was found equally in low, intermediate and high grade tumours; however, tumours lacking the fusion transcript were significantly associated with metastases; they may represent a subset of aggressive tumours.
- In another study, the median survival for fusion-positive patients was greater than 10 years compared to 1.6 years for fusion-negative patients.

CRTC1-MAML2; exon 1 of CRTC1 fused to exons 2-5 of MAML2. Note: CRTC1 is also known as MECT1, or WAMTP1.

CRTC1-MAML2. In the fusion protein, the first 171 aa including the basic domain of MAML2 are replaced by 42 aa of CRTC1; there are no sequence similarities in the N-terminal domains of MAML2 and CRTC1. The fusion protein activates transcription of the Notch target gene HES1 independently of both Notch ligand and CSL.
Transforming activity of CRTC1-MAML2 fusion oncoprotein is mediated by mimicking constitutive activation of cAMP signaling, by activating CREB directly.

Warthins tumor with t(11;19)(q21-22;p13).

In rare instances mucoepidermoid carcinoma may arise from or coexist with Warthins tumors.

Warthins tumor is a salivary gland neoplasm consisting of benign epithelial and lymphoid components; malignant transformation is extremely rare.

CRTC1-MAML2

Clear Cell Hidradenomas of the skin with t(11;19)(q21-22;p13)

Clear Cell Hidradenomas of the skin are benign sweat gland tumors of eccrine duct origin.

CRTC1-MAML2; exon 1 of CRTC1 fused to exons 2 of MAML2.

therapy-related acute leukemia and MDS.

MLL-MAML2; exon 1-7 of MLL fused to exons 2-5 of MAML2.

Hybrid transcript MLL/MAML2 contains the following domains: from MLL: AT-hook, DNA-Methyltransferase; from MAML2: Q rich domain, acidic domain.