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Adrenal Tumors
Published in Dongyou Liu, Tumors and Cancers, 2017
Pheochromocytoma varies from a small, circumscribed to a large, hemorrhagic, and necrotic lesion (mean 7 cm/200 g). A fresh tumor turns dark brown in the presence of potassium dichromate at pH 5–6 (chromaffin reaction). Microscopically, the tumor shows zellballen (small nests or alveolar pattern), trabecular, or solid patterns of polygonal/spindle-shaped cells in a rich vascular network, with finely granular basophilic or amphophilic cytoplasm, oval nuclei, prominent nucleolus, variable inclusion-like structures, and rare mitotic figures.
Ear Tumors
Published in Dongyou Liu, Tumors and Cancers, 2017
Glomus tumor (paraganglioma) is a benign tumor that arises from the paraganglia situated throughout the temporal bone (including on the jugular dome, the promontory of the middle ear, and along the Jacobson and Arnold nerves). Glomus tumor involving the dome of the jugular bulb and spreading to the neck is referred to as glomus jugulare tumor, whereas that involving the medial wall along the course of the Jacobson nerve but limited to the ear is referred to as glomus tympanicum tumor. Macroscopically, the tumor is a soft, pulsatile, reddish-purple, vascular lobulated mass in the middle ear. On MRI, the tumor is isointense on T1-weighted images and brightly enhanced with gadolinium; and demonstrates increased signal intensity in the solid portions with persistent flow voids in the vascular portions (characteristic “salt-and-pepper” pattern) on T2-weighted images. Histologically, the tumor shows characteristic clusters of chief cells (termed zellballen or “cell balls”) in a highly vascular stroma. Sustentacular cells and nerve axons, seen in normal paraganglia, rarely appear in paraganglioma. Immunohistochemically, the tumor is positive for neuron-specific enolase and chromogranin, and occasionally positive for S100 protein, but negative for cytokeratin.
Surgical pathology of the adrenal gland
Published in Demetrius Pertsemlidis, William B. Inabnet III, Michel Gagner, Endocrine Surgery, 2017
Pheochromocytoma can be grossly distinguished from adrenal cortical neoplasms, as it forms a tan to gray mass, in contrast to the yellow or orange appearance of cortical tumors (Figure 26.29). Pheochromocytomas are usually solid, although larger tumors may undergo cystic degeneration. Microscopically, a nested pattern likened to a ball of cells (zellballen) is characteristic (Figure 26.30). The tumor is composed predominantly of small pleomorphic cells (chief cells) with clear, amphophilic, or basophilic cytoplasm. The nuclei may be round or spindled, with small or large nucleoli, or may be markedly hyperchromatic. Flattened s100-positive sustentacular cells are frequently identified surrounding the nests (Figure 26.31).
The carotid body and associated tumors: updated review with clinical/surgical significance
Published in British Journal of Neurosurgery, 2019
Nasir Butt, Woong Kee Baek, Stefan Lachkar, Joe Iwanaga, Asma Mian, Christa Blaak, Sameer Shah, Christoph Griessenauer, R. Shane Tubbs, Marios Loukas
Grossly, CBTs are well circumscribed and can have a pseudocapsule. The cut surface is typically solid with a smooth, rubbery texture. Histologically, CBTs and paragangliomas in general share a similar “zellballen” growth pattern. This refers to a well-developed nest of chief cells, surrounded by supporting or sustentacular cells at the periphery of the zellballen.17 The tumor cells are predominately chief cells with round, hyperchromatic nuclei and abundant cytoplasm.17 Unfortunately, it is not possible to predict how aggressive a paraganglioma will be based on its presenting histological features. Although the pleomorphism, notable mitotic figures, and atypical nuclear forms are common characteristics of increased malignant activity, in paragangliomas, these cellular morphologies may not necessarily indicate its aggressive behavior.
Metastatic brain carotid body paraganglioma with endocrine activity: a case report and literature review
Published in British Journal of Neurosurgery, 2019
Xiang Wang, Xianglan Zhu, Jinxiu Chen, Yanhui Liu, Qing Mao
The patient underwent a craniotomy, and a total resection of the tumor was performed (Figure 1(f)). The tumor was hard and solid, located on the left parietal lobe, and originated from the cerebral parenchyma with infiltration of the dura matter. The tumor was surrounded by multiple thickened blood vessels with some necrosis inside (Figure 1(g)). Histopathological examination revealed that the tumor tissue consisted of clusters of chief cells surrounded by sustentacular cells arranged in nest-like, trabecular, and adenoidal gland pattern, known as so-called “Zellballen” (Figure 1(h)). The chief cells had abundant pale cytoplasm and vesicular nuclei with conspicuous nucleoli, and some cells showed prominent atypia. Nuclear mitosis was easily detected, and the blood supply of the tumor was abundant (Figure 1(i)). The sustentacular cells tested locally weak-positive to S-100. The chief cells were positive for synaptophysin and chromogranin A, but negative for CK and GFAP (Figure 1(j,k)). Further, the positive percentage of MIB-1was around 15% (Figure 1(l)). The pathological diagnosis was paraganglioma.