伴有淋巴结转移的十二指肠节细胞性副神经节瘤一例

节细胞性副神经节瘤目前被认为是一种罕见的良性肿瘤,通常发生在十二指肠黏膜下层,具有特征性的形态学特征。该文报道1例51岁男性发生在十二指肠的节细胞性副神经节瘤伴有淋巴结转移,描述其组织病理学特征、免疫表型及电镜特点、诊断和鉴别诊断要点,并结合文献复习其组织学特征及预后。     

Duodenal gangliocytic paraganglioma: report of two cases and review of literature

We report two cases of Gangliocytic paraganglioma (GP), one of which was accompanied by lymph node metastasis. Histologically, the tumor was composed of three morphologically distinct cell populations: spindle cells, ganglion-like cells and epithelioid cells. The epithelioid cells were positive for cytokeratin (AE1/AE3), synaptophysin (Syn), chromogranin A (CgA), CD56 and progesterone receptor (PR). Ganglion-like cell types showed positive reactivity for Syn and CD56. In contrast, the spindle-shaped cells showed positive reactivity for S-100. The patient with lymph node metastasis has a good prognosis. Nonetheless, close surveillance is still necessary for patients with GP because a few cases of GP with regional lymph node metastasis and even distant metastasis have been published, including a malignant case of GP showing a lethal course.     

Duodenal gangliocytic paraganglioma with regional lymph node metastasis and a glandular component

Gangliocytic paraganglioma (GP) is generally considered to be a benign periampullary lesion, although it is unclear whether it should be classified as a hamartoma or as a neoplasm. Here, we present a GP case with lymph node metastasis. A 16-year-old boy complained of exertional dyspnea. Upper endoscopy and imaging studies revealed a polypoid ampullary tumor. Pancreaticoduodenectomy with lymph node dissection was performed due to swelling of peripancreatic lymph nodes. Histologically, the tumor consisted of three cell types: epithelioid; spindle; and ganglion cells. In addition to these typical components of GP, a distinct glandular component was also present. There was substantial invasion of tumor cells into the lymphovascular vessels, associated with lymph node metastases. These lymph node metastases were histologically similar to the primary tumor. To judge from these findings GP may be a true neoplasm with metastatic capacity. Pre- and intraoperative investigations for lymph node or distant metastases are required for adequate resection of this kind of tumor.     

Duodenal gangliocytic paraganglioma. Clinicopathologic and immunocytochemical study of 11 cases

The clinicopathologic features of 11 cases (8 in men) of duodenal gangliocytic paraganglioma are presented. The patients averaged 56 years of age; none showed evidence of phakomatosis. Ten tumors occurred in the second portion of the duodenum, and one arose in the third portion. All tumors were polypoid, and half presented with gastrointestinal bleeding. The neoplasms were composed of paraganglioma and carcinoid-like elements, neurons, and Schwann as well as sustentacular cells. All tumors behaved in a benign fashion after local resection or snare polypectomy; long-term follow-up (1-25 years; mean, 8.3 years) showed no recurrence in any case. Immunocytochemical examination demonstrated the presence of somatostatin, serotonin, and human pancreatic polypeptide within endocrine cells and neurons.     

Nasopharyngeal gangliocytic paraganglioma

Gangliocytic paraganglioma (GP) is a rare neoplasm described almost exclusively in the gastrointestinal tract, especially the periampullary region. However, several examples have been reported at various sites, including the stomach, jejunum, and appendix. Herein we report a case of GP involving the nasopharynx. To our knowledge, this is the first report of GP at this site. A 44-year-old woman presented with headaches and symptoms of fullness and pressure related to mass effect. An initial endoscopic biopsy was followed by surgical excision of the nasopharyngeal mass. The triphasic tumor fulfilled the morphologic and immunohistochemical criteria for GP. The histogenesis of GP is uncertain, and the current belief is that it arises from the embryonic ventral pancreas. This concept is based largely on the location of most cases, which is along the embryologic migration route of the ventral pancreas, as well as the expression of pancreatic polypeptide by the tumor. The nasopharyngeal location of our case clearly refutes the pancreatic origin of GP. We propose that the tumor probably arises from totipotential adult stem cells, which in the right microenvironment differentiate along nonnative cell lineages.     

Duodenal gangliocytic paraganglioma with lymph node metastasis: a case report and review of the literature

A case of duodenal gangliocytic paraganglioma (DGP) in a 67-year-old woman is presented. The DGP arose in the second part of the duodenum. Although most of the reported cases of DGP are considered benign, in the present case, we found regional lymph nodes containing metastatic tumor. Previous reports have documented metastases containing only epithelioid cells. The current case demonstrates metastatic tumor in regional lymph nodes containing all 3 of the DGP components (spindle cells, ganglion-like cells, and epithelioid cells).     

Gastrointestinal haemorrhage from a jejunal gangliocytic paraganglioma.

A case of jejunal gangliocytic paraganglioma is reported in a 54 year old woman who presented with brisk melaena. The tumour was not encapsulated, involved the mucosa and submucosa, and was composed of epithelial nests, spindle cells, and ganglion cells. These cells were mixed, giving carcinoid-like, paraganglioma-like, and ganglioneuroma-like patterns in different areas of the tumour. The lesion was excised locally and recovery was uneventful. Only four previous cases have been reported at this site.     

Immunohistochemical characterization of a case of duodenal gangliocytic paraganglioma

A case of duodenal gangliocytic paraganglloma (DGP) arising in the ampulla of Vater of an 84 year old male is reported. The present case shows the typical hlstological features with a mixture of epithellold, gangllon-like and spindle cells. lmmunohistochemically, the spindle cells were positive with antibodies to neuron specific enorse, S-100 protein and nsurofilament (NF), but negative for anti-glial fibrillary acidic protein (GFAP), synaptophyaln and myelln basic protein (MBP). Both anti-Leu-7 and chromogranin A antibodies gave porritive reactions with a fine granular pattern in the epithellold cells. Most ganglion-like cells showed the cytoplasmic lmmunorsection with GFAP and a few with NF and synapto physin. In these three tumor components, no reactivity was detected In the antibodies to the neurohormonal peptides tested. The present data are fundamentally in keeping with those previously reported, but add the immunohistochemical positivity of gangllon-like cells for GFAP, suggesting different neuroglial differentiation.     

Pulmonary gangliocytic paraganglioma: a case report and review of the literature

Gangliocytic paraganglioma (GP) is a rare histologic type of neuroendocrine tumors. We report a case of pulmonary GP in a 29-year-old male presenting with an asymptomatic endobronchial nodule. Grossly, the tumor showed a 4.0x3.8x3.5 cm well-defined nodule with yellowish cut surface. Microscopically, the tumor was composed of three distinct cellular types: epithelioid cells, ganglion-like cells and spindle cells. Meanwhile, transitional cells, having morphologic features between ganglion-like and epithelioid cells, were also presented. The epithelioid cells arranged in various morphologic architectures, including Zellballen, papillary, cystic and microcystic pattern. The epithelioid cells were positive for AE1/AE3, CAM 5.2, chromogranin A and synaptophysin. Ganglion-like cells showed immunoreactivity for chromogranin A and synaptophysin. A few ganglion-like cells were also positive for AE1/AE3 and/or CAM 5.2. The spindle cells were positive for S-100 protein and neurofilament. The transitional cells showed a similar immunohistochemical profile to the epithelioid cells. The authors believe stem cell theory is a reasonable explanation for the origin of GP. GP probably originate from some kind of mucosa associated stem cell which can differentiate into diverse cellular lineages.     

The cytologic diagnosis of gangliocytic paraganglioma: A case report

Gangliocytic paragangliomas are rare tumors primarily found in the duodenum. We report a case of a woman who presented with a retroperitoneal lymph node involved by metastatic gangliocytic paraganglioma. Subsequently, fine‐needle aspiration (FNA) cytology was used to identify the primary duodenal gangliocytic paraganglioma. The smears of the aspirate material were highly cellular and contained a dominant population of epithelioid cells, a second population of ganglion cells and a third population of small, bland spindled cells. To our knowledge, the cytologic features of gangliocytic paraganglioma have not previously been documented. Diagn. Cytopathol. 2013. © 2011 Wiley Periodicals, Inc.     

Corticotropin-producing pulmonary gangliocytic paraganglioma associated with Cushing's syndrome

Gangliocytic paraganglioma (GP) is an unusual tumor in the wide spectrum of neuroendocrine neoplasms. The biologic behavior and histogenesis of this tumor is uncertain because, despite its benign histologic features, there have been reports of metastases to regional lymph nodes and distant metastases. Although most of these tumors have been reported in the duodenum, examples of GP arising in extra-ampullary sites have been well documented. Herein, we report the case of a lung GP corticotropin producing and presenting as Cushing's syndrome. After removal of the tumor, there was a significant drop in cortisol and corticotropin levels. To our knowledge, this is the first report of a GP causing Cushing's syndrome.     

Duodenal gangliocytic paragangliomas: a study of 10 cases with immunocytochemical neuroendocrine markers

Ten cases of duodenal paraganglioma were studied by conventional histologic and immunocytochemical techniques at both light and electron microscopic levels. Histologically, mixtures of epithelial, ganglion, and spindle cells were seen. In all of the cases immunoreactivity for neuron-specific enolase (NSE) and protein gene product (PGP) 9.5 was seen in each component. Pancreatic polypeptide immunoreactivity was detected in eight cases, mainly in epithelial cells. Somatostatin immunoreactivity was present in epithelial and ganglion cells in nine cases. In seven cases immunoreactivity for neurofilaments, a marker for neurons, was seen in ganglion and spindle cells. However, immunoreactivity for chromogranin, a protein found in endocrine storage granules, was found in only two cases, and the staining was confined to well-granulated epithelial cells. The spindle cells were immunostained only for neuronal markers, NSE and neurofilaments, and the glial marker S-100 protein.     

Immunohistochemical and electron microscopic studies of a case of duodenal gangliocytic paraganglioma

Gangliocytic paraganglioma (GPG) is a rare tumor, occurring almost exclusively in the duodenum. In the present case, a submucosal tumor 2.5 × 2 × 1.5 cm in size was located on the anal side of the papilla of Vater, with clear margins and without capsule on cut-surface examination. Tumor cells included three types of cells: ganglion-like cells (GCs), endocrine cells (ECs), and Schwann cells (SCs). The GCs were large with eccentric nuclei with large nucleoli and clear abundant cytoplasm. ECs were detected in small nests, surrounded by bundles of SCs. Immunohistochemically, GCs were positive for synaptophysin, neuron-specific enolase (NSE), and CD56. ECs were positive for chromogranin A, NSE, somatostatin, pancreatic polypeptide, and CD56, and were associated with S100 protein-positive SCs. On fine structural examination, ECs contained numerous membrane-bounded secretory granules, 250–450 nm in diameter, in their cytoplasm, surrounded by a branched, complex basal lamina. SCs possessed basal lamina along their long interlacing cytoplasmic processes. The histogenesis of GPG most likely involves proliferation and differentiation of pluripotent stem cells in the duodenal crypts in the duodenum as a true tumor, although it is also possible that the retroperitoneal components of both GCs and SCs proliferate, together with ECs, from ventral primordial tissue of the pancreas in the duodenum. The immunohistochemical and ultrastructural findings of a case of GPG are reported, focusing on three major cellular components: GCs, ECs, and SCs.     

Multifocal presentation of gangliocytic paraganglioma in the mediastinum and esophagus

Gangliocytic paraganglioma (GP) is a rare, typically benign tumor that shows neuroectodermal (neurosustentacular or Schwannian and neuronal) and neuroendocrine differentiation. Once thought to arise exclusively in the periampullary region as a solitary lesion, recent reports have documented both origin of GP in a variety of extra-duodenal sites as well as synchronous multifocal presentation of the tumor. Herein, we describe the first reported case of simultaneous occurrence of GP in the superior mediastinum and esophagus. A mass in the mid-distal esophagus and a separate mass in the superior mediastinum at the thoracic inlet were found in a 58-year-old woman by computed tomography scan. Subsequent biopsy of the superior mediastinal mass showed nests of epithelioid tumor cells coexisting with ganglioneuromatous elements, whereas biopsy of the esophageal mass showed nests of epithelioid cells with interspersed ganglion cells. The epithelioid tumor cells showed diffuse immunohistochemical expression of keratin (CAM 5.2), chromogranin, and synaptophysin supporting true neuroendocrine differentiation; ganglion cells expressed S-100 protein and neurofilament protein; and the spindled elements expressed S-100 protein, neurofilament protein, and glial fibrillary acidic protein indicating Schwannian differentiation. The finding of another GP occurring outside the periampullary region bolsters the argument for a stem cell origin of this unusual tumor.