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Primary adenocarcinoma of the thymus: an immunohistochemical and molecular study with review of the literature.

Maghbool M, Ramzi M, Nagel I, Bejarano P, Siebert R, Saeedzadeh A, Daneshbod Y - BMC Clin Pathol (2013)

Bottom Line: Primary adenocarcinoma of thymus is extremely rare.This is a case of primary adenocarcinoma with intestinal differentiation and focal mucin production in the thymus.Array comperative genomic hybridization (CGH) analysis showed a complex pattern of chromosomal imbalances including homozygous deletion at the HLA locus in chromosomal region 6p21.32.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Molecular Pathology, Dr Daneshbod Pathology Laboratory, Shiraz, Iran. y@daneshbod.com.

ABSTRACT

Background: Primary adenocarcinoma of thymus is extremely rare.

Case presentation: This is a case of primary adenocarcinoma with intestinal differentiation and focal mucin production in the thymus. Thymic cyst was associated with this tumor. Intestinal differentiation was confirmed by immunohistochemical stain with positivity for CDX-2, CK20, villin, MOC31 and focal positivity of CK7. Array comperative genomic hybridization (CGH) analysis showed a complex pattern of chromosomal imbalances including homozygous deletion at the HLA locus in chromosomal region 6p21.32.

Conclusion: This rare tumor shows a similar genetic aberration with other studied thymic epithelial tumors.

No MeSH data available.


Related in: MedlinePlus

Representative images of radiology, histology, immunostaining as well as results of array-comparative genomic hybridization (array-CGH). A: Chest X ray showed mediastinal widening. B: chest CT scan revealed anterior mediastinal mass that invade pericardium. C: tumoral sheets and glandular structures in desmoplasticstroma (H&E stain, × 400). D: Malignant glands showed diffuse membranous positivity for CD5. (Immunoperoxidase). E: nuclear positivity for CDX-2 in malignant glands (Immunoperoxidase). F: Malignant glands showed diffuse positivity for CK20 (Immunoperoxidase). G: benign columnar linningthymic cyst adjacent to neoplastic glands (H&E stain, × 200). H: neoplastic glands arising in the vicinity of thymic cyst (H&E stain, × 200). I: Array-CGH-results displayed in a whole genome view showed deletion of chromosome 6. J: Homozygously deleted region around the HLA-DRB5-locus in chromosomal region 6p21.32.
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Figure 1: Representative images of radiology, histology, immunostaining as well as results of array-comparative genomic hybridization (array-CGH). A: Chest X ray showed mediastinal widening. B: chest CT scan revealed anterior mediastinal mass that invade pericardium. C: tumoral sheets and glandular structures in desmoplasticstroma (H&E stain, × 400). D: Malignant glands showed diffuse membranous positivity for CD5. (Immunoperoxidase). E: nuclear positivity for CDX-2 in malignant glands (Immunoperoxidase). F: Malignant glands showed diffuse positivity for CK20 (Immunoperoxidase). G: benign columnar linningthymic cyst adjacent to neoplastic glands (H&E stain, × 200). H: neoplastic glands arising in the vicinity of thymic cyst (H&E stain, × 200). I: Array-CGH-results displayed in a whole genome view showed deletion of chromosome 6. J: Homozygously deleted region around the HLA-DRB5-locus in chromosomal region 6p21.32.

Mentions: A 28 year old woman presented with neck and right upper extremity pain accompanied by dyspnea of two years duration. Chest X- ray revealed mediastinal widening (Figure 1A). Chest computed tomography (CT) scan (Figure 1B) showed an anterior mediastinal mass invading in pericardium without extramediastinal extension. An initial clinical impression of a mediastinal germ cell tumor was considered but serum tumor markers such as alpha-feto protein (AFP), Beta-human chorionic gonadotropin (β-hCG), carcinoembryonic antigen (CEA) and CA-125 were normal. CA19-9 level was 2420 U/ml (normal: 0.37U/ml). A mediastinal biopsy was done and showed histologic features of adenocarcinoma. Endoscopy, colonoscopy, abdominopelvicsonography and imaging studies were negative for primary origin. The patient underwent mid-sternotomy. The mass was resected. The patient received chemotherapy (GEMOX) and radiotherapy with two recurrences in 2 years follow up. She is doing well and free of tumor after 6 months.


Primary adenocarcinoma of the thymus: an immunohistochemical and molecular study with review of the literature.

Maghbool M, Ramzi M, Nagel I, Bejarano P, Siebert R, Saeedzadeh A, Daneshbod Y - BMC Clin Pathol (2013)

Representative images of radiology, histology, immunostaining as well as results of array-comparative genomic hybridization (array-CGH). A: Chest X ray showed mediastinal widening. B: chest CT scan revealed anterior mediastinal mass that invade pericardium. C: tumoral sheets and glandular structures in desmoplasticstroma (H&E stain, × 400). D: Malignant glands showed diffuse membranous positivity for CD5. (Immunoperoxidase). E: nuclear positivity for CDX-2 in malignant glands (Immunoperoxidase). F: Malignant glands showed diffuse positivity for CK20 (Immunoperoxidase). G: benign columnar linningthymic cyst adjacent to neoplastic glands (H&E stain, × 200). H: neoplastic glands arising in the vicinity of thymic cyst (H&E stain, × 200). I: Array-CGH-results displayed in a whole genome view showed deletion of chromosome 6. J: Homozygously deleted region around the HLA-DRB5-locus in chromosomal region 6p21.32.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3672093&req=5

Figure 1: Representative images of radiology, histology, immunostaining as well as results of array-comparative genomic hybridization (array-CGH). A: Chest X ray showed mediastinal widening. B: chest CT scan revealed anterior mediastinal mass that invade pericardium. C: tumoral sheets and glandular structures in desmoplasticstroma (H&E stain, × 400). D: Malignant glands showed diffuse membranous positivity for CD5. (Immunoperoxidase). E: nuclear positivity for CDX-2 in malignant glands (Immunoperoxidase). F: Malignant glands showed diffuse positivity for CK20 (Immunoperoxidase). G: benign columnar linningthymic cyst adjacent to neoplastic glands (H&E stain, × 200). H: neoplastic glands arising in the vicinity of thymic cyst (H&E stain, × 200). I: Array-CGH-results displayed in a whole genome view showed deletion of chromosome 6. J: Homozygously deleted region around the HLA-DRB5-locus in chromosomal region 6p21.32.
Mentions: A 28 year old woman presented with neck and right upper extremity pain accompanied by dyspnea of two years duration. Chest X- ray revealed mediastinal widening (Figure 1A). Chest computed tomography (CT) scan (Figure 1B) showed an anterior mediastinal mass invading in pericardium without extramediastinal extension. An initial clinical impression of a mediastinal germ cell tumor was considered but serum tumor markers such as alpha-feto protein (AFP), Beta-human chorionic gonadotropin (β-hCG), carcinoembryonic antigen (CEA) and CA-125 were normal. CA19-9 level was 2420 U/ml (normal: 0.37U/ml). A mediastinal biopsy was done and showed histologic features of adenocarcinoma. Endoscopy, colonoscopy, abdominopelvicsonography and imaging studies were negative for primary origin. The patient underwent mid-sternotomy. The mass was resected. The patient received chemotherapy (GEMOX) and radiotherapy with two recurrences in 2 years follow up. She is doing well and free of tumor after 6 months.

Bottom Line: Primary adenocarcinoma of thymus is extremely rare.This is a case of primary adenocarcinoma with intestinal differentiation and focal mucin production in the thymus.Array comperative genomic hybridization (CGH) analysis showed a complex pattern of chromosomal imbalances including homozygous deletion at the HLA locus in chromosomal region 6p21.32.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Molecular Pathology, Dr Daneshbod Pathology Laboratory, Shiraz, Iran. y@daneshbod.com.

ABSTRACT

Background: Primary adenocarcinoma of thymus is extremely rare.

Case presentation: This is a case of primary adenocarcinoma with intestinal differentiation and focal mucin production in the thymus. Thymic cyst was associated with this tumor. Intestinal differentiation was confirmed by immunohistochemical stain with positivity for CDX-2, CK20, villin, MOC31 and focal positivity of CK7. Array comperative genomic hybridization (CGH) analysis showed a complex pattern of chromosomal imbalances including homozygous deletion at the HLA locus in chromosomal region 6p21.32.

Conclusion: This rare tumor shows a similar genetic aberration with other studied thymic epithelial tumors.

No MeSH data available.


Related in: MedlinePlus