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Duodenal gangliocytic paraganglioma showing lymph node metastasis: a rare case report.

Okubo Y, Yokose T, Tuchiya M, Mituda A, Wakayama M, Hasegawa C, Sasai D, Nemoto T, Shibuya K - Diagn Pathol (2010)

Bottom Line: Histological examination confirmed the immunohistochemical identification of three components comprising epithelioid cells, spindle-shaped cells, and ganglion-like cells.Epithelioid cells showed positive reactivity for synaptophysin, somatostatin, and CD56.Furthermore, we found lymph node metastasis despite lack of bcl-2 and p53 expression.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Surgical Pathology, Toho University School of Medicine, 6-11-1 Omori-Nishi, Ota-Ku, Tokyo, 143-8541, Japan.

ABSTRACT
We describe a case of duodenal gangliocytic paraganglioma showing lymph node metastasis. A 61-year-old Japanese man underwent pylorus preserving pancreaticoduodenectomy to remove a tumor at the papilla of Vater. The section of the tumor extending from the mucosa to submucosa of the duodenum was sharply demarcated, solid, and white-yellowish. Neither necrosis nor hemorrhage was present. Histological examination confirmed the immunohistochemical identification of three components comprising epithelioid cells, spindle-shaped cells, and ganglion-like cells. Epithelioid cells showed positive reactivity for synaptophysin, somatostatin, and CD56. In contrast, spindle-shaped cells showed positive reactivity for S-100 protein, but not for synaptophysin, somatostatin or CD56. Furthermore, we found lymph node metastasis despite lack of bcl-2 and p53 expression. In addition to the rarity of the tumor, we are describing here the present case suggests the malignant potency of the tumor despite lack of acceptable prognostic indicators for neuroendocrine tumor.

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The surgical specimen. (A) A solid tumor measuring 25 × 30 × 25 mm in size was found at the papilla of Vater which was lobulated and covered by attenuated mucosa with ulcer formation at the center of elevation. (B) The section of the tumor extending from the mucosa to submucosa of the duodenum was sharply demarcated, solid, and white-yellowish.
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Figure 1: The surgical specimen. (A) A solid tumor measuring 25 × 30 × 25 mm in size was found at the papilla of Vater which was lobulated and covered by attenuated mucosa with ulcer formation at the center of elevation. (B) The section of the tumor extending from the mucosa to submucosa of the duodenum was sharply demarcated, solid, and white-yellowish.

Mentions: The surgical specimen, an en-bloc comprising duodenum, bile duct, gallbladder, and head of pancreas, was fixed with 10% buffered formalin. A solid tumor 25 × 30 × 25 mm in size was found at the papilla of Vater whose surface was lobulated and covered by attenuated mucosa showing ulcer formation at the center of elevation (Fig. 1A). The section of the tumor extending from the mucosa to submucosa of the duodenum was sharply demarcated, solid and white-yellowish (Fig. 1B). Neither necrosis nor hemorrhage was present. Sections of paraffin-embedded tissue were prepared and stained with hematoxylin and eosin (HE) double stain for light microscopic observation. Histological examination showed that a large body of the tumor was present in the submucosa and invaded a part of the muscularis propria, but the bile duct and pancreas were not involved. Histological observation also revealed that the tumor consisted of three identical cellular components: epithelioid cells, spindle-shaped cells, and ganglion-like cells. Tumor cells of an epithelioid cell type usually nested and had a round to oval-shaped nucleus with an inconspicuous nucleolus, as well as a clear and eosinophilic cytoplasm (Fig. 2A). Tumor cells of a spindle-shaped cell type encompassed the nests of epithelioid cells with alignment of a single cell layer and had an elongated and plump nucleus, including an attenuated eosinophilic cytoplasm (Fig. 2A). Tumor cells of a ganglion-like cell type were rarely seen and had a round nucleus with conspicuous nucleolus, as well as a polyhedral amphophilic cytoplasm (Fig. 2B). In addition to the absence of mitosis among these cells, neither necrosis nor hemorrhage was found. However, tumor cells of an epithelioid cell type showed regional lymph node metastasis (Fig. 2C and 2D).


Duodenal gangliocytic paraganglioma showing lymph node metastasis: a rare case report.

Okubo Y, Yokose T, Tuchiya M, Mituda A, Wakayama M, Hasegawa C, Sasai D, Nemoto T, Shibuya K - Diagn Pathol (2010)

The surgical specimen. (A) A solid tumor measuring 25 × 30 × 25 mm in size was found at the papilla of Vater which was lobulated and covered by attenuated mucosa with ulcer formation at the center of elevation. (B) The section of the tumor extending from the mucosa to submucosa of the duodenum was sharply demarcated, solid, and white-yellowish.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2874790&req=5

Figure 1: The surgical specimen. (A) A solid tumor measuring 25 × 30 × 25 mm in size was found at the papilla of Vater which was lobulated and covered by attenuated mucosa with ulcer formation at the center of elevation. (B) The section of the tumor extending from the mucosa to submucosa of the duodenum was sharply demarcated, solid, and white-yellowish.
Mentions: The surgical specimen, an en-bloc comprising duodenum, bile duct, gallbladder, and head of pancreas, was fixed with 10% buffered formalin. A solid tumor 25 × 30 × 25 mm in size was found at the papilla of Vater whose surface was lobulated and covered by attenuated mucosa showing ulcer formation at the center of elevation (Fig. 1A). The section of the tumor extending from the mucosa to submucosa of the duodenum was sharply demarcated, solid and white-yellowish (Fig. 1B). Neither necrosis nor hemorrhage was present. Sections of paraffin-embedded tissue were prepared and stained with hematoxylin and eosin (HE) double stain for light microscopic observation. Histological examination showed that a large body of the tumor was present in the submucosa and invaded a part of the muscularis propria, but the bile duct and pancreas were not involved. Histological observation also revealed that the tumor consisted of three identical cellular components: epithelioid cells, spindle-shaped cells, and ganglion-like cells. Tumor cells of an epithelioid cell type usually nested and had a round to oval-shaped nucleus with an inconspicuous nucleolus, as well as a clear and eosinophilic cytoplasm (Fig. 2A). Tumor cells of a spindle-shaped cell type encompassed the nests of epithelioid cells with alignment of a single cell layer and had an elongated and plump nucleus, including an attenuated eosinophilic cytoplasm (Fig. 2A). Tumor cells of a ganglion-like cell type were rarely seen and had a round nucleus with conspicuous nucleolus, as well as a polyhedral amphophilic cytoplasm (Fig. 2B). In addition to the absence of mitosis among these cells, neither necrosis nor hemorrhage was found. However, tumor cells of an epithelioid cell type showed regional lymph node metastasis (Fig. 2C and 2D).

Bottom Line: Histological examination confirmed the immunohistochemical identification of three components comprising epithelioid cells, spindle-shaped cells, and ganglion-like cells.Epithelioid cells showed positive reactivity for synaptophysin, somatostatin, and CD56.Furthermore, we found lymph node metastasis despite lack of bcl-2 and p53 expression.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Surgical Pathology, Toho University School of Medicine, 6-11-1 Omori-Nishi, Ota-Ku, Tokyo, 143-8541, Japan.

ABSTRACT
We describe a case of duodenal gangliocytic paraganglioma showing lymph node metastasis. A 61-year-old Japanese man underwent pylorus preserving pancreaticoduodenectomy to remove a tumor at the papilla of Vater. The section of the tumor extending from the mucosa to submucosa of the duodenum was sharply demarcated, solid, and white-yellowish. Neither necrosis nor hemorrhage was present. Histological examination confirmed the immunohistochemical identification of three components comprising epithelioid cells, spindle-shaped cells, and ganglion-like cells. Epithelioid cells showed positive reactivity for synaptophysin, somatostatin, and CD56. In contrast, spindle-shaped cells showed positive reactivity for S-100 protein, but not for synaptophysin, somatostatin or CD56. Furthermore, we found lymph node metastasis despite lack of bcl-2 and p53 expression. In addition to the rarity of the tumor, we are describing here the present case suggests the malignant potency of the tumor despite lack of acceptable prognostic indicators for neuroendocrine tumor.

Show MeSH
Related in: MedlinePlus