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A Unique Case of an Aggressive Gangliocytic Paraganglioma of the Filum Terminale.

Akbik OS, Floruta C, Chohan MO, SantaCruz KS, Carlson AP - Case Rep Surg (2016)

Bottom Line: Paragangliomas are rare neuroendocrine tumors that are mostly found in the head and neck.The growth pattern is documented over three years, which to our knowledge has not previously been reported in the literature and is different from the natural history.Clinical, radiological, and pathological characteristics of the tumor are discussed in light of available reports of this rare tumor.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurosurgery, University of New Mexico, Albuquerque, NM 87131, USA.

ABSTRACT
Paragangliomas are rare neuroendocrine tumors that are mostly found in the head and neck. Even less common are gangliocytic variant paragangliomas of the spine for which there are only 7 other documented cases in the literature. We report a case of gangliocytic paraganglioma of the sacral spine in a 68-year-old man. The growth pattern is documented over three years, which to our knowledge has not previously been reported in the literature and is different from the natural history. Clinical, radiological, and pathological characteristics of the tumor are discussed in light of available reports of this rare tumor.

No MeSH data available.


Related in: MedlinePlus

Gangliocytic paraganglioma. (a) Gangliocytic paraganglioma depicting nested arrangement of cells in a Zellballen pattern. H&E stain, 20x. (b) Gangliocytic paraganglioma with ganglion cells (arrow head). H&E stain, 40x. (c) Section of the tumor capsule showing pigmented macrophages (brown) and extracellular hemoglobin breakdown product (yellow). H&E stain, 4x. (d) Focus of remote hemorrhage toward the periphery of the tumor. H&E stain, 20x. (e) Synaptophysin stain positive (brown): positive for synaptic vesicle protein, 20x. (f) S100 stain positive for sustentacular cells (dark brown along edges of lobules), 20x.
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fig4: Gangliocytic paraganglioma. (a) Gangliocytic paraganglioma depicting nested arrangement of cells in a Zellballen pattern. H&E stain, 20x. (b) Gangliocytic paraganglioma with ganglion cells (arrow head). H&E stain, 40x. (c) Section of the tumor capsule showing pigmented macrophages (brown) and extracellular hemoglobin breakdown product (yellow). H&E stain, 4x. (d) Focus of remote hemorrhage toward the periphery of the tumor. H&E stain, 20x. (e) Synaptophysin stain positive (brown): positive for synaptic vesicle protein, 20x. (f) S100 stain positive for sustentacular cells (dark brown along edges of lobules), 20x.

Mentions: Histology revealed islands of uniform tumor cells with neuroendocrine features arranged in a nested Zellballen pattern, which is consistent with a paraganglioma (Figure 4(a)). Additionally, foci of tumor cells with extensive gangliocytic features, including abundant cytoplasm with Nissl-like RNA and prominent nucleoli, were seen (Figure 4(b)). Hemosiderin laden macrophages and extracellular hemoglobin breakdown product were found extensively in the tumor pseudocapsule, with occasional foci of remote hemorrhage within the superficial tumor as well (Figures 4(c) and 4(d)). Immunostaining was negative for glial fibrillary acidic protein (GFAP) and positive for synaptophysin and S100 (Figures 4(e) and 4(f)).


A Unique Case of an Aggressive Gangliocytic Paraganglioma of the Filum Terminale.

Akbik OS, Floruta C, Chohan MO, SantaCruz KS, Carlson AP - Case Rep Surg (2016)

Gangliocytic paraganglioma. (a) Gangliocytic paraganglioma depicting nested arrangement of cells in a Zellballen pattern. H&E stain, 20x. (b) Gangliocytic paraganglioma with ganglion cells (arrow head). H&E stain, 40x. (c) Section of the tumor capsule showing pigmented macrophages (brown) and extracellular hemoglobin breakdown product (yellow). H&E stain, 4x. (d) Focus of remote hemorrhage toward the periphery of the tumor. H&E stain, 20x. (e) Synaptophysin stain positive (brown): positive for synaptic vesicle protein, 20x. (f) S100 stain positive for sustentacular cells (dark brown along edges of lobules), 20x.
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Related In: Results  -  Collection

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Show All Figures
getmorefigures.php?uid=PMC4940518&req=5

fig4: Gangliocytic paraganglioma. (a) Gangliocytic paraganglioma depicting nested arrangement of cells in a Zellballen pattern. H&E stain, 20x. (b) Gangliocytic paraganglioma with ganglion cells (arrow head). H&E stain, 40x. (c) Section of the tumor capsule showing pigmented macrophages (brown) and extracellular hemoglobin breakdown product (yellow). H&E stain, 4x. (d) Focus of remote hemorrhage toward the periphery of the tumor. H&E stain, 20x. (e) Synaptophysin stain positive (brown): positive for synaptic vesicle protein, 20x. (f) S100 stain positive for sustentacular cells (dark brown along edges of lobules), 20x.
Mentions: Histology revealed islands of uniform tumor cells with neuroendocrine features arranged in a nested Zellballen pattern, which is consistent with a paraganglioma (Figure 4(a)). Additionally, foci of tumor cells with extensive gangliocytic features, including abundant cytoplasm with Nissl-like RNA and prominent nucleoli, were seen (Figure 4(b)). Hemosiderin laden macrophages and extracellular hemoglobin breakdown product were found extensively in the tumor pseudocapsule, with occasional foci of remote hemorrhage within the superficial tumor as well (Figures 4(c) and 4(d)). Immunostaining was negative for glial fibrillary acidic protein (GFAP) and positive for synaptophysin and S100 (Figures 4(e) and 4(f)).

Bottom Line: Paragangliomas are rare neuroendocrine tumors that are mostly found in the head and neck.The growth pattern is documented over three years, which to our knowledge has not previously been reported in the literature and is different from the natural history.Clinical, radiological, and pathological characteristics of the tumor are discussed in light of available reports of this rare tumor.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurosurgery, University of New Mexico, Albuquerque, NM 87131, USA.

ABSTRACT
Paragangliomas are rare neuroendocrine tumors that are mostly found in the head and neck. Even less common are gangliocytic variant paragangliomas of the spine for which there are only 7 other documented cases in the literature. We report a case of gangliocytic paraganglioma of the sacral spine in a 68-year-old man. The growth pattern is documented over three years, which to our knowledge has not previously been reported in the literature and is different from the natural history. Clinical, radiological, and pathological characteristics of the tumor are discussed in light of available reports of this rare tumor.

No MeSH data available.


Related in: MedlinePlus