bromochloroacetic-acid and Paraganglioma

Paraganglioma of the urinary bladder is a rare pathologic entity with no definitive histological, immunohistochemical or molecular features to determine its malignant potency. Malignancy is essentially determined by the presence of deep local invasion, invasion of adjacent structures, and lymph node or distant metastases. So far, up to 180 cases of paraganglioma have been reported, with less than 30 being malignant. We present a case of malignant paraganglioma of the urinary bladder in a 44-year-old woman. The patient's symptoms were painless hematuria and micturitional headache. The tumor presented the characteristic "zellballen" pattern of growth and immunohistochemically was positive for all neuroendocrine markers. The patient underwent partial cystectomy and the following two postoperative years were uneventful. The literature on paraganglioma of the urinary bladder, analyzing the histological, immunohistochemical and molecular characteristics, is reviewed.

Topics: Adult; Biomarkers, Tumor; Female; Humans; Immunohistochemistry; Keratins; Paraganglioma; Treatment Outcome; Urinary Bladder; Urinary Bladder Neoplasms

To review the clinical and pathological picture of laryngeal paragangliomas and compare laryngeal paragangliomas with the three other types of laryngeal neuroendocrine neoplasms.. The study presents a case review of a patient with a subglottic laryngeal paraganglioma treated at our institution, with a literature review of the laryngeal neuroendocrine neoplasms. Included is a review of all four neuroendocrine neoplasms, taking a close look at a comparison between laryngeal paragangliomas and atypical carcinoids tumors. All cases of subglottic laryngeal paragangliomas reported in the literature were also compiled and tabulated.. The patient's record was reviewed for age, symptomatology, workup, and surgical procedures performed.. The patient was disease free 36 months after undergoing a tracheostomy for airway control, followed by a cricoid split for complete excision of the tumor.. It is important to differentiate among the four laryngeal neuroendocrine neoplasms, especially between atypical carcinoids and paragangliomas. Each tumor requires different treatments, with the former requiring a more aggressive approach. Paragangliomas must be completely excised to prevent their recurrence, and this approach should be considered a curative treatment. In addition, the immunohistochemical battery is of paramount importance in obtaining the correct pathological diagnosis.

Topics: Aged; Calcitonin; Carcinoma, Neuroendocrine; Endoscopy; Female; Humans; Immunoenzyme Techniques; Immunohistochemistry; Keratins; Laryngeal Neoplasms; Paraganglioma; Tracheal Neoplasms; Tracheostomy

A case of gangliocytic paraganglioma (GP) of the ampulla of Vater is reported and the literature reviewed, with special attention to immunohistochemical studies. The present case, which occurred in a 56-year-old woman, shows the typical histological admixture of epithelioid, ganglion and spindle cells. Immunohistochemistry reveals strong reactivity for synaptophysin, Leu-7, somatostatin, S-100 protein and vimentin. A few ganglion cells are reactive for neurofilaments. Chromogranin A, myelin basic protein, desmin and cytokeratin are absent. Immunohistochemical data from literature regarding the cytoskeletal composition of GPs are not unequivocal: cytokeratin and neurofilament positivity is reported by some authors and denied by others. More uniformity is reported concerning the peptides produced by GPs: somatostatin and pancreatic polypeptide are the most frequently found antigens, followed by serotonin. General neuroendocrine markers like neuron specific enolase and protein gene product 9.5 are always positive, whereas chromogranins are rarely found. S-100 protein is always positive in the spindle cell component. Our data are in keeping with those previously reported and add the diffuse positivity for the Leu-7 antigen and the positivity of ganglion cells for synaptophysin. The nature of the tumour is still a matter of debate and it is difficult to agree with either of the proposed hypotheses--hamartoma/choristoma versus true neoplasm. However the recent reports of the occasional malignant evolution of GPs may support their true neoplastic nature.

Topics: Antigens, Differentiation; CD57 Antigens; Duodenal Neoplasms; Duodenum; Female; Ganglia; Humans; Immunohistochemistry; Intestinal Mucosa; Keratins; Membrane Proteins; Middle Aged; Paraganglioma; S100 Proteins; Somatostatin; Synaptophysin; Vimentin

Paragangliomas are rare neuroendocrine tumors originating from neural crest-derived paraganglion cells. Primary cauda equina paraganglioma (CEP) pose both diagnostic and surgical challenges. We report 12 cases of CEP to characterize the diagnostic and operative approach to these rare tumors. 12 cases with primary CEP were studied; 5 patients were male (41.7%) and 7 were female (56.3%). The median age was 44 years (range: 15-64 years). The most common symptom was lower back pain of variable duration. Radiologically, the lesions were intradural and extramedullary with well-defined margins, and ranged from 1 to 4.5 cm. in diameter (mean: 1.65 cm). 9 tumors were composed of sheets and nests of cells with a neuroendocrine pattern and intense vascularity and displayed a characteristic Zellballen pattern. Interestingly, CAM 5.2 was diffusely or focally positive with a dot-like or membrane pattern in 8/11 cases (72.7%). Similarly, CK was diffusely or focally positive with membrane and cytoplasmic staining or with a dot-like pattern in 7/11 (63.6%) and 2/11 cases (18.2%). None of the cases showed deletion of SDHB nor expression of GATA3. CEP can display aberrant keratin positivity, and this should be considered in the differential diagnosis of these lesions. This finding also raises the possibility that CEP may be an entirely different entity than non-spinal paragangliomas.

Topics: Adult; Cauda Equina; Female; Humans; Keratins; Male; Neuroendocrine Tumors; Paraganglioma; Peripheral Nervous System Neoplasms

Paraganglioma is a rare neuroendocrine tumor arising from undifferentiated cells of the primitive neural crest. We report a case of renal paraganglioma in a 67-year-old patient. Computed tomography demonstrated a solid mass in the middle and lower pole of the right kidney. Sonography revealed an enlarged right kidney with an irregular shape but distinct border. Renal cell carcinoma was diagnosed provisionally; the tumor was completely resected and submitted for pathological examination. Unexpectedly, histopathology and immunohistochemistry confirmed paraganglioma arising from the renal parenchyma. In this study, we report the exceptional occurrence of Paired box gene 8 (PAX-8) expression in a renal paraganglioma. In addition, we demonstrated diffuse cytokeratin positivity in this renal paraganglioma. Although our report of a paraganglioma originating from the kidney is not unique, our finding expands the known immunophenotypic spectrum of this tumor. The awareness of the possible occurrence of cytokeratin diffuse positivity in paraganglioma is relevant to avoiding misdiagnosis of paraganglioma.

Topics: Aged; Diagnosis, Differential; Histological Techniques; Humans; Keratins; Kidney; Kidney Neoplasms; Male; Paraganglioma; PAX8 Transcription Factor; Tomography, X-Ray Computed

The absence of cytokeratin expression in paraganglioma helps to differentiate it from other neuroendocrine neoplasms such as carcinoid tumor. Although rare cytokeratin positive paragangliomas have been reported, there are no large systematic studies of this phenomenon. The aim of this study was to determine the frequency and extent of cytokeratin expression in paragangliomas using a large cohort of cases from multiple anatomic sites. Immunohistochemical staining for keratin AE1/AE3 (mouse monoclonal, MAB3412; Millipore) and CAM 5.2 (mouse monoclonal, 349 205; Becton-Dickinson) was performed on whole-tissue sections from 60 resected paragangliomas from the head and neck (36), thorax (10), abdomen (8), intradural/epidural spine (5) and bone, left iliac (1). Cytokeratin expression was identified in only 2/60 (3.3%) cases. One was a mediastinal paraganglioma with moderate to strong expression of keratin AE1/AE3 and CAM 5.2 in <5% tumor cells. The other was a lumbar intradural paraganglioma positive for CAM 5.2 (moderate to strong, 80% of tumor cells) but negative for keratin AE1/AE3. All other paragangliomas (58/60, 96.7%) were negative for keratin AE1/AE3 and CAM 5.2. This study - the largest series of cytokeratin-stained whole-tissue sections of paragangliomas to date - supports the dictum that most paragangliomas are cytokeratin negative. Rare exceptions may be site-related.

Topics: Adolescent; Adult; Aged; Aged, 80 and over; Antibodies, Monoclonal; Female; Humans; Immunohistochemistry; Keratins; Male; Middle Aged; Paraganglioma; Paraganglioma, Extra-Adrenal; Young Adult

Topics: Humans; Keratins; Lumbar Vertebrae; Male; Middle Aged; Paraganglioma; Spinal Neoplasms

The precise diagnosis of thyroid neoplasias will guide surgical management. Primary thyroid paraganglioma has been rarely reported. Data on prevalence, immunohistochemistry (IHC), and molecular genetics in a systematic series of such patients are pending. We performed a multinational population-based study on thyroid paraganglioma and analyzed prevalence, IHC, and molecular genetics. Patients with thyroid paraganglioma were recruited from the European-American-Head-and-Neck-Paraganglioma-Registry. Demographic and clinical data were registered. Histopathology and IHC were re-investigated. All patients with thyroid paraganglioma underwent molecular genetic analyses of the SDHA, SDHB, SDHC, SDHD, SDHAF2, VHL, RET, TMEM127, and MAX genes. Analyses included Sanger sequencing and multiplex ligation-dependent probe amplification (MLPA) for detection of large rearrangements. Of 947 registrants, eight candidates were initially identified. After immunohistochemical analyses of these eight subjects, 5 (0.5%) were confirmed to have thyroid paraganglioma. IHC was positive for chromogranin, synaptophysin, and S-100 and negative for calcitonin in all five thyroid paragangliomas, whereas the three excluded candidate tumors stained positive for pan-cytokeratin, a marker excluding endocrine tumors. Germline variants, probably representing mutations, were found in four of the five confirmed thyroid paraganglioma cases, two each in SDHA and SDHB, whereas the excluded cases had no mutations in the tested genes. Thyroid paraganglioma is a finite entity, which must be differentiated from medullary thyroid carcinoma, because medical, surgical, and genetic management for each is different. Notably, approximately 80% of thyroid paragangliomas are associated with germline variants, with implications for additional tumors and a potential risk for the family. As opposed to sporadic tumors, surgical management and extent of resection are different for heritable tumors, each guided by the precise gene involved.

Topics: Adult; Aged; Calcitonin; Chromogranin A; DNA-Binding Proteins; Electron Transport Complex II; Female; Germany; Humans; Keratins; Male; Middle Aged; Mutation; Paraganglioma; Prevalence; Registries; S100 Proteins; Succinate Dehydrogenase; Synaptophysin; Thyroid Neoplasms; Transcription Factors

Paragangliomas are derived from neurosecretory cells believed to be of neural crest origin. A spinal location of paraganglioma is rare and usually presents as an intradural mass.. A primary intraosseous paraganglioma of sacrum is extremely unusual, and only 6 cases were reported. In this study, we report a rare case of a 44-year-old man with the complaint of low back pain and lower extremity weakness. Imaging workup, including computerized tomography (CT), and magnetic resonance imaging (MRI) presented an intraosseous sacral lesion with invasion of sacrum in the S1-S3 vertebrae, and extension to L4-L5 spinal canal. The patient underwent subtotal tumor resection, followed by radiation therapy.. The morphological and immunohistochemical studies revealed a composite tumor of paraganglioma and ganglioneuroma components, with immunopositivity for cytokeratin.. To the best of our knowledge, this is the first report in the literature demonstrating an intraosseous sacral paraganglioma with these 2 pathological features.

Topics: Adult; Bone Cysts; Ganglioneuroma; Humans; Keratins; Male; Paraganglioma; Sacrum; Spinal Neoplasms

Thyroid paragangliomas are extremely rare tumors arising from the inferior laryngeal paraganglia. Most patients are female and present as asymptomatic thyroid nodule. Diagnosing thyroid paraganglioma can be difficult on fine-needle aspiration (FNA) since it could be misdiagnosed as medullary thyroid carcinoma, calcitonin-negative neuroendocrine tumor of the thyroid gland (CNNETT), metastatic neuroendocrine tumor, and intrathyroid parathyroid proliferation. We describe the cytologic features and immunohistochemical staining pattern of thyroid paraganglioma from a 60-year-old woman with a gradually enlarging neck mass for several months. An ultrasound guided fine-needle aspiration was performed which revealed epithelioid, plasmacytoid to occasionally spindle-shaped cells with ovoid to elongated nuclei, fine chromatin pattern and inconspicuous nucleoli. The cells were arranged singly, in sheets, and focally in acinar pattern. Occasional nuclear overlapping, crush artifact, and binucleated cells were identified. Neither colloid nor amyloid was seen. Immunohistochemical stains performed on the cell block showed the tumor cells were positive for neuroendocrine markers and negative for cytokeratin (AE1/AE3), calcitonin, CEA, thyroglobulin, and TTF-1. There were rare S-100 positive cells. Given the difference in management of paragangliomas compared to its cytology mimics and association with familial cancer syndromes in some cases, awareness of this rare tumor, and use of immunohistochemical stains are critical in arriving at the diagnosis.

Topics: Biomarkers, Tumor; Biopsy, Fine-Needle; Calcitonin; Carcinoembryonic Antigen; Cytodiagnosis; Female; Humans; Immunohistochemistry; Keratins; Middle Aged; Nuclear Proteins; Paraganglioma; Thyroglobulin; Thyroid Gland; Thyroid Neoplasms; Thyroid Nodule; Thyroid Nuclear Factor 1; Transcription Factors

Topics: Carcinoid Tumor; Diagnosis, Differential; Humans; Keratins; Lung Neoplasms; Male; Middle Aged; Paraganglioma

Hyalinizing trabecular tumor (HTT) is a rare thyroid tumor of follicular cell origin with a trabecular pattern of growth and marked intratrabecular hyalinization. This tumor is known to share morphological and architectural similarities with paraganglioma and medullary thyroid carcinoma, as well as the nuclear features and RET/PTC1 translocations of papillary thyroid carcinoma. These tumors are not associated with RAS or BRAF mutations. Whether the presence of RET alterations in HTT are sufficient molecular proof of its relationship with papillary thyroid carcinoma (PTC) is still to be defined. Of great interest is the characteristic strong peripheral cytoplasmic and membranous staining of the tumor cells with MIB1 immunostain, not seen in any other thyroid neoplasm. Although cases of malignant HTT have been recorded, HTT should be considered a benign neoplasm or, at most, a neoplasm of extremely low malignant potential.

Topics: Adenoma; Adult; Aged; Biomarkers, Tumor; Calcitonin; Cell Nucleus; Chromogranin A; Female; Humans; Immunohistochemistry; Keratins; Ki-67 Antigen; Male; Middle Aged; Paraganglioma; Sex Characteristics; Thyroid Neoplasms

This study presents a rare case of compound paraganglioma/ganglioneuroma with comprehensive immunohistochemical studies that reveal strong cytokeratin expression in all components. A 74-year-old woman presented with a mass lesion of the cauda equina. The 1.8-cm tumor showed 3 histomorphologically and immunohistochemically distinct components: typical paragangliomatous neuroendocrine areas, mature ganglion cell-like neuronal areas, and a "neuromatous" proliferation of Schwann cells with admixed axons. As often seen in cauda equina paragangliomas, the neuroendocrine cells were cytokeratin-positive. In addition, immunoreactivity for cytokeratins was also observed in the neurons and axons. This tumor illustrates the broad spectrum of divergent differentiation that can be seen in cells of sympathoadrenal lineage.

Topics: Aged; Cauda Equina; Female; Ganglioneuroma; Humans; Immunohistochemistry; Keratins; Paraganglioma

Topics: Back Pain; Biomarkers, Tumor; Cauda Equina; Chromogranins; Female; Glial Fibrillary Acidic Protein; Humans; Immunohistochemistry; Keratins; Magnetic Resonance Imaging; Middle Aged; Pain, Intractable; Paraganglioma; Peripheral Nervous System Neoplasms; S100 Proteins; Spinal Cord; Synaptophysin; Vimentin

We describe a case of primary pulmonary paraganglioma, a tumor that has not been reported in sufficient detail in previous literature. The patient was a 55-year-old woman with hypertension accompanied by an elevated serum norepinephrine level (2651 pg/mL; normal 100-450 pg/mL). Computed tomography revealed a well-circumscribed solid mass, 3.5 cm in diameter, located in the lower lobe of the left lung. In the lobectomy specimen, the tumor had invaded the B8 bronchus and hilar lymph nodes with microscopic metastasis to the mediastinal nodes. The tumor showed histologic, immunohistochemical, and ultrastructural features of paraganglioma: argyrophilic cells arranged in a nesting (Zellballen) or anastomosing trabecular pattern within an arcuate vascular network. Neoplastic chief cells positive for neuroendocrine markers (CD56, synaptophysin, chromogranin A) were surrounded by sustentacular cells positive for S-100 protein. Neurofilament protein was positively stained, but cytokeratins were totally negative. On electron microscopy, chief cells possessed abundant dense core granules with an eccentric halo ("norepinephrine-type" granules). The patient's blood pressure began to decline soon after the resection, and her serum norepinephrine promptly returned to almost normal. On the basis of our experience, our case is a bona fide primary pulmonary paraganglioma, a tumor heretofore subject to considerable skepticism.

Topics: CD56 Antigen; Chromogranin A; Chromogranins; Cytoplasmic Granules; Female; Humans; Hypertension; Keratins; Lung Neoplasms; Lymphatic Metastasis; Middle Aged; Neurofilament Proteins; Norepinephrine; Paraganglioma; S100 Proteins; Synaptophysin

A large primary retroperitoneal sublumbar neoplasm was identified in an 11-year-old Holstein cow, with metastases to the lungs, kidneys and lymph nodes. The tumour cells proliferated in a characteristic endocrine pattern, were argyrophilic and positive for neuron-specific enolase, and had membrane-bounded intracytoplasmic granules. In addition, the cells were occasionally positive for cytokeratin and had desmosome-like intercellular junctions. The primary tumour mass was diagnosed as a malignant paraganglial tumour of the aortico-sympathetic ganglion (organ of Zuckerkandl), and was considered to contain primitive cells with epithelial differentiation.

Topics: Animals; Biomarkers, Tumor; Cattle; Cattle Diseases; Cytoplasmic Granules; Desmosomes; Female; Ganglia, Sympathetic; Immunoenzyme Techniques; Keratins; Kidney Neoplasms; Lung Neoplasms; Lymph Nodes; Paraganglioma; Phosphopyruvate Hydratase; Retroperitoneal Neoplasms; Silver Staining

Topics: Biopsy, Needle; E2F6 Transcription Factor; Endothelium, Vascular; Female; Humans; Hyperplasia; Immunohistochemistry; Keratins; Middle Aged; Paraganglioma; Platelet Endothelial Cell Adhesion Molecule-1; Repressor Proteins; Thyroglobulin; Thyroid Neoplasms; Transcription Factors

The authors report a case of gangliocytic paraganglioma of the lung, which has not yet been described in a pulmonary neoplasm. A 75-year-old man underwent right middle and lower lobe lobectomy. A slightly yellowish mass was located at the bifurcation between the lower and middle lobe bronchus, protruding into the truncus intermedius. The neoplastic cells were composed of three cellular elements: uniform endocrine cells in a Zellballen arrangement, large ganglion-like cells within the nests of endocrine cells, and spindle-shaped cells arranged in streams to surround the nests. Each component exhibited the characteristic immunohistochemical properties, which were similar to those of the corresponding neuroendocrine neoplasms: Endocrine cells were positive for CAM 5.2, chromogranin A, and synaptophysin, like carcinoid tumor; ganglion-like cells were positive only for neurofilament, like ganglioneuroma; and spindle-shaped cells were positive for neurofilament and S-100 protein, like paraganglioma. These results agreed with those in gangliocytic paraganglioma of the duodenum. Pulmonary gangliocytic paraganglioma is similar to that in the duodenum, and is a hamartomatous proliferation of epithelial endocrine and neuronal cells of the bronchus.

Topics: Aged; Biomarkers; Biomarkers, Tumor; Chromogranin A; Chromogranins; Humans; Immunoenzyme Techniques; Keratins; Lung Neoplasms; Male; Neoplasm Proteins; Neurofilament Proteins; Paraganglioma; Radiography, Thoracic; S100 Proteins; Synaptophysin; Tomography, X-Ray Computed

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.

Topics: Adult; Biopsy; Chromogranins; Female; Humans; Immunoenzyme Techniques; Immunohistochemistry; Keratins; Nasopharyngeal Neoplasms; Paraganglioma; S100 Proteins; Synaptophysin; Tissue Embedding; Vasoactive Intestinal Peptide

Paraganglioma of the urinary bladder is rarely encountered and its biologic behavior is uncertain. The authors sought to determine the prognostic factors that would predict patient outcome.. The Mayo Clinic experience over 53 years with paraganglioma of the bladder was reviewed. All histologic slides from 16 patients were reviewed by the authors. Eight cases were examined immunohistochemically with cytokeratin (AE1/3, cytokeratin 7, and cytokeratin 20), vimentin, S-100 protein, neuroendocrine markers (chromogranin, synaptophysin, and neuron specific enolase), p53 protein, and MIB-1. DNA ploidy was determined by digital image analysis in formalin fixed, paraffin embedded tissue. The mean follow-up was 6.3 years (range, 0.4-16.4 years).. Paraganglioma usually occurred in young adult women (mean age, 45 years; range, 16-74 years). The male-to-female ratio was 1 to 3. The common symptoms and signs were hypertension and hematuria. The tumors were usually located intramurally in the lateral and posterior wall of the bladder and were multifocal in 3 cases (18%). Seven patients were treated by transurethral resection, eight by partial cystectomy, and one by radical cystectomy. T classification was T1 (1 patient), T2 (9 patients), T3 (2 patients), and T4b (4 patients). At the time of diagnosis, one patient had distant metastasis and one had regional lymph node metastasis. One patient developed metastasis 1 year after diagnosis and died of the disease 1.5 years later. None of the patients with T1 or T2 tumors had recurrence or tumor progression. All tumors were aneuploid. The mean MIB-1 labeling index was 1.5% (range, 0.03-7.0%). The tumor cells displayed immunoreactivity for S-100 protein and neuroendocrine markers and were negative for p53 (except 1 case) and cytokeratin.. Paraganglioma of the urinary bladder occurs mostly in young adult women. Patients with tumor of advanced classification (>/=T3) are at risk of recurrence, metastasis, and dying of the disease, whereas patients in this study with T1 or T2 disease had favorable outcomes after complete tumor resection.

Topics: Adolescent; Adult; Aged; Biomarkers, Tumor; DNA, Neoplasm; Female; Humans; Immunohistochemistry; Keratins; Ki-67 Antigen; Male; Middle Aged; Nerve Tissue Proteins; Paraganglioma; Pheochromocytoma; Ploidies; Prognosis; Retrospective Studies; S100 Proteins; Tumor Suppressor Protein p53; Urinary Bladder Neoplasms; Vimentin

Topics: Carcinoid Tumor; Cauda Equina; Diagnosis, Differential; Humans; Keratins; Paraganglioma; Peripheral Nervous System Neoplasms; Sacrum

Although rare, renal cell carcinoma (RCC) can metastasize to the bladder. When this occurs, it might complicate diagnosis. Morphologically, RCC can be confused with transitional cell carcinomas (TCCs), especially those exhibiting clear cell features, and also with other bladder tumors, such as paragangliomas and metastatic melanomas. We report seven cases of RCC metastatic to the bladder that occurred in 6 men and 1 woman who were 35 to 69 years old. The most common presenting symptom was the reappearance of hematuria, which developed from 2 to 131 months (mean, 41.3 mo) after the removal of the primary RCC. In all of the patients, the metastatic RCC involved multiple organs; no case had an isolated metastasis to the bladder. The prognosis was poor, and five patients died of disease between 4 and 24 months (mean, 12.8 mo) after diagnosis of the metastasis to the bladder. The remaining two patients were lost to follow-up. All of the tumors were conventional clear or "granular" cell RCCs, with nuclear grades of 2 or 3. In five patients, metastases were confined to the lamina propria, but in two patients, tumors involved the muscularis propria as well. A comparative immunohistochemical study showed that metastatic RCCs were positive for CAM5.2, vimentin, and Leu-M1, and negative for cytokeratin 20, cytokeratin 7, 34betaE12, carcinoembryonic antigen, S-100 protein, HMB45, and chromogranin. Classic and clear cell TCCs were positive for all of the cytokeratins and carcinoembryonic antigen and negative for vimentin. Paragangliomas were positive for chromogranin and showed scattered positivity for the S-100 protein in the sustentacular cells. Metastatic melanomas were positive for S-100 protein and HMB45. The histologic appearance of RCC, particularly the delicate fibrovascular stroma with abundant sinusoidal vessels, is a feature that can be used to recognize the tumor. When there is difficulty diagnosing metastatic RCC, TCC, or other tumors in the bladder, the immunohistochemical findings can assist in the differential diagnosis.

Topics: Adult; Aged; Antigens, Neoplasm; Carcinoembryonic Antigen; Carcinoma, Renal Cell; Carcinoma, Transitional Cell; Chromogranins; Diagnosis, Differential; Female; Humans; Immunohistochemistry; Intermediate Filament Proteins; Keratin-20; Keratin-7; Keratins; Kidney Neoplasms; Lewis X Antigen; Male; Melanoma; Melanoma-Specific Antigens; Middle Aged; Neoplasm Proteins; Paraganglioma; S100 Proteins; Urinary Bladder Neoplasms; Vimentin

This report describes a case of atypical carcinoid of the larynx with metastatic tumor to cervical lymph nodes showing features similar to paraganglioma. Immunohistochemically, both the primary and metastatic tumor were positive for epithelial membrane antigen, cytokeratin, neuron-specific enolase, chromogranin, synaptophysin, calcitonin and carcinoembryonic antigen. The sustentacular cells in pseudo-cell ball ("Zellballen") formations were shown by a positive S-100 marker. Electron microscopic analysis revealed numerous neuroendocrine granules measuring 150-250 nm in diameter. Clinical features, histological appearance, histochemical, immunohistochemical and ultrastructural findings, as well as differential diagnosis are discussed.

Topics: Aged; Calcitonin; Carcinoembryonic Antigen; Carcinoid Tumor; Chromogranins; Cytoplasmic Granules; Diagnosis, Differential; Humans; Immunohistochemistry; Keratins; Laryngeal Neoplasms; Lymphatic Metastasis; Male; Microscopy, Electron; Mucin-1; Neck; Paraganglioma; Phosphopyruvate Hydratase; S100 Proteins; Synaptophysin

A 21 cm retroperitoneal cystic mass was excised from a 71 year old woman. The cyst was filled with a hemorrhagic fluid and contained a 5 cm parietal hemorrhagic nodule. On histology, the nodule was composed of a uniform population of round cells arranged in trabeculae and nests. The neoplastic cells were immunoreactive to cytokeratin, EMA, NSE, chromogranin A, pancreatic polypeptide (PP) and Gastrin (G). Ultrastructural observation of neurosecretory granules confirmed the neuroendocrine nature of the tumor. No other lesions were detected and a diagnosis of primary epithelial neuroendocrine tumor was rendered. The histogenesis of the tumor including the possibility of a paraganglionic origin is discussed.

Topics: Aged; Biomarkers, Tumor; Calcitonin; Carcinoma, Neuroendocrine; Diagnosis, Differential; Epithelium; Female; Gastrins; Humans; Keratins; Neoplasm Proteins; Pancreatic Polypeptide; Paraganglioma; Retroperitoneal Neoplasms; S100 Proteins; Teratoma

A 36-year-old Russian patient had been exposed to severe nuclear radiation during the Tschernobyl accident and developed Cushing's syndrome in September 1991. After bilateral adrenalectomy a small centrally localized lung tumor in the left segment S6 was diagnosed, and the syndrome was correlated to primary lung cancer. Thoracotomy and resection of the segments S6 revealed a primary ACTH-producing lung tumor of the cell type of a paraganglioma. The patient lost his symptoms after curative tumor resection. The case is discussed under the aspects of tumor etiology, its clinical course and immunohistochemical findings.

Topics: Adrenocorticotropic Hormone; Adult; Cushing Syndrome; Humans; Keratins; Lung Neoplasms; Male; Neoplasms, Radiation-Induced; Paraganglioma; Phosphopyruvate Hydratase; Power Plants; Radioactive Hazard Release; Tumor Suppressor Protein p53; Ukraine; Vimentin

Topics: Adult; Cauda Equina; Female; Humans; Immunoenzyme Techniques; Immunophenotyping; Intermediate Filament Proteins; Keratins; Male; Middle Aged; Paraganglioma; Peripheral Nervous System Neoplasms

A case of vulvar paraganglioma is reported. Following presentation with vulvar pain, a 1-cm nodule was excised from the labium minus of a 58-year-old woman. Histologically, the tumor was composed of nests of round eosinophilic cells with moderately pleomorphic nuclei, beneath an intact squamous epithelium. Ultrastructural studies indicated two cell types within the neoplasm: chief cells with numerous small neurosecretory granules and peripheral slender sustentacular cells. A reticulin stain confirmed the "zellballen" nature of the neoplasm, and the neoplastic cells showed moderate argyrophilia on a Grimelius stain. The immunoperoxidase stains for chromogranin and neuron-specific enolase were strongly positive in the neoplastic chief cells. Immunostaining using anti-S-100 antibody confirmed the finding of sustentacular cells by identifying many slender cellular processes among the chief cells. These light and electron microscopic findings are diagnostic of paraganglioma, an entity not previously reported in the vulva to our knowledge.

Topics: Cell Nucleus; Cytoplasm; Cytoplasmic Granules; Female; Histocytochemistry; Humans; Immunoenzyme Techniques; Keratins; Microscopy, Electron; Middle Aged; Organelles; Paraganglioma; Staining and Labeling; Vulvar Neoplasms

Topics: Adenocarcinoma; Aged; Duodenal Neoplasms; Female; Humans; Immunohistochemistry; Keratins; Neoplasms, Multiple Primary; Pancreatic Neoplasms; Paraganglioma

Twenty-nine paragangliomas of the head and neck region including 20 glomus jugulare (GJ) and nine carotid body (CB) tumors were evaluated for the presence of neuroendocrine and intermediate filament antigens. Immunohistochemistry on formalin-fixed, paraffin-embedded tissue was used to identify: S-100 protein (S-100); neuron-specific enolase (NSE); chromogranin A (CHA); serotonin (SER); synaptophysin (SYN); cytokeratin (CK); neurofilament (NF); desmin (DES); vimentin (VIM); and glial fibrillary acidic protein (GFAP). S-100 protein staining of sustentacular cell nuclei and cytoplasm was found in all tumors and was present in chief cells in 4 of 20 GJ and 3 of 9 CB tumors. All tumors stained with at least three neuroendocrine markers (29 of 29 NSE, 28 of 29 SYN, 26 of 29 CHA, 25 of 29 SER). CK was detected in 2 GJ and 1 CB tumor using anticytokeratins AE 1/3 and CAM 5.2. Neurofilament protein could not be demonstrated in fixed material, and all tumors were negative for GFAP and desmin. Vimentin was inconsistently detected in chief and sustentacular cells. We conclude that, in formalin-fixed material, paragangliomas have S-100 protein staining of sustentacular cells with chief cells containing antigens associated with neuroendocrine differentiation. The presence of CK in some paragangliomas is consistent with recent tissue culture studies demonstrating immunoblot confirmation of CK in pheochromocytomas and represents a potential source of immunohistologic misinterpretation in diagnosis, unless a panel of markers is utilized.

Topics: Antibodies; Chromogranin A; Chromogranins; Desmin; Glial Fibrillary Acidic Protein; Head and Neck Neoplasms; Humans; Immunohistochemistry; Intermediate Filament Proteins; Keratins; Membrane Proteins; Neurofilament Proteins; Neurosecretory Systems; Paraganglioma; Phosphopyruvate Hydratase; S100 Proteins; Serotonin; Synaptophysin; Vimentin

A case of gangliocytic paraganglioma of the second portion of the duodenal loop is presented. The tumor was polypoid and, histologically, composed of mature ganglion cells, spindle cells and epithelial-like cells. Immunocytochemical examination demonstrated the presence of neurofilament 200 K and S-100 protein only in the first two types of cells; all the cells were positive for neuron-specific enolase. The reaction for cytokeratin was negative in all neoplastic components. According to morphologic and immunocytochemical findings, we suggest a hamartomatous nature of this entity.

Topics: Adult; Duodenal Neoplasms; Female; Ganglia; Histocytochemistry; Humans; Intermediate Filaments; Keratins; Paraganglioma