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PubMed1995

Two cases of duodenal gangliocytic paraganglioma: immunocytochemical characteristics.

Two cases of duodenal gangliocytic paraganglioma were studied by means of immunocytochemical methods using 41 kinds of antibodies. The tumors consisted of three histological types; carcinoid, ganglioneuroma and paraganglioma. Tumors of both cases exhibited immunoreactivity to at least one or as many as three of the following: calcitonin, calcitonin-gene related peptide, endocrine granule constituent, Leu7, neuropeptide Y and basic fibroblast growth factor. In addition, these tumors were also immunopositive for neuron specific enolase, S-100 protein, neurofilament protein, pancreatic polypeptide, chromogranin A, somatostatin, leuenkephalin, substance P and vasoactive intestinal peptide, as has been described in previous reports. In one case, tumor cells were immunopositive for adrenocorticotropin, bombesin, gastrin releasing peptide, myelin basic protein, neuroendocrine marker and tyrosine hydroxylase. Moreover, paraganglioma cells of tumors showed both argyrophilia and argentaffinity. These results suggest that duodenal gangliocytic paraganglioma may originate from embryonic neuroinsular complex.

Duodenal Neoplasms↗

PubMed1996

[Combined embolization and surgery of paragangliomas of the glomus caroticum. Report of a case and review of literature].

Carotid body paragangliomas are rare, and are generally benign tumours that grow slowly. Extirpation of carotid body paragangliomas is well documented as the best curative treatment, but can be accompanied by complications such as peroperative bleeding, stroke and injury to cranial nerves. Accurate preoperative diagnosis and evaluation are important, and preoperative carotide angiography is essential to confirm the diagnosis. In this case report, a patient with large carotid body paraganglioma underwent preoperative selective embolization of the major afferent arteries. The vascularity of the paraganglioma was reduced substantially, and complete extirpation was accomplished without complications. Blood loss was negligible and the postoperative course was uneventful. The patient had no cranial nerve or cerebral dysfunction after operation. In cases with angiographically distinct feeding arteries, in addition to satisfactory cerebral perfusion, preoperative selective embolization can be an important supplementary treatment.

Adult↗

PubMed1996

[Hypertension secondary to multiple malignant paragangliomas].

We describe a case of severe hypertension, due to multiple malignant norepinephrine secreting paragangliomas. Neuroendocrine system tumours are responsible for 0.1-0.5% cases of hypertension. Their extra-adrenal localisation is about 10%. Only 20% of paragangliomas are catecholamine secreting, and causing a syndrome similar to that of pheochromocytoma. In our patient the diagnosis of retroperitoneal multiple paragangliomas was made two years after the beginning of the typical symptoms. At surgery 6 paragangliomas were removed; some vascular and muscular structures (inferior vena cava, ileopsoas) were infiltrated. A radiotherapy treatment was conducted for a total dose of 55.8 Gy. Immediately after the operation blood pressure decreased, but the administration of alpha and beta blockers was required to reach normotension. This treatment was confirmed at six months follow-up, in consideration of the persistently high norepinephrine plasma levels.

Adult↗

PubMed1996

Diagnostic imaging in patients with paragangliomas. Computed tomography, magnetic resonance and MIBG scintigraphy comparison.

OBJECTIVE: We compared iodine-131 metaiodobenzylguanidine (MIBG), computed tomography (CT) and magnetic resonance (MR) imaging studies in 40 patients with suspected or proven paragangliomas. PATIENTS: 21 patients were studied during the initial evaluation, while 19 were evaluated after surgery for paragangliomas. Furthermore, in 18 patients with benign (n = 10) or malignant (n = 8) tumors, MIBG uptake, MR signal intensity ratios (SIR) and CT parameters were analyzed. RESULTS: In patients studied during the initial evaluation, CT and MR had a significant (p < 0.05) higher diagnostic sensitivity (100% for both) compared to MIBG (82%); conversely, MIBG showed a higher specificity (100%) compared to CT and MR (50% for both); the accuracy of MIBG (86%), CT and MR (91% for both) were similar. In patients evaluated after surgery, MIBG and MR had slightly better sensitivity (85% for both) compared to CT (77%). MIBG showed again better specificity (100%) than CT and MR (83% for both); the accuracy of MIBG, MR and CT were respectively 90%, 84% and 79%. While no significant differences were observed in MR SIR and CT findings between benign and malignant tumors, MIBG uptake was significantly higher in malignant compared to benign lesions (p < 0.03). CONCLUSIONS: Our results suggest that CT and MR are particularly useful in the initial evaluation of patients with suspected paragangliomas. MIBG should be recommended during the post-surgical follow-up of such patients since recurrent, malignant or extra-adrenal disease frequently occur. Finally, while MR SIR and CT features are not able to distinguish malignant paragangliomas from benign tumors, MIBG uptake is higher in malignant lesions compared to benign tumors providing a diagnostic criterion to differentiate these lesions.

3-Iodobenzylguanidine↗

PubMed1998

Nonsporadic cases and unusual morphological features in pheochromocytoma and paraganglioma.

BACKGROUND: Pheochromocytomas and paragangliomas are well-defined entities. Some of their nonsporadic associations and unusual morphological appearances are not universally appreciated. DESIGN: We reevaluated all adrenal pheochromocytomas and extra-adrenal paragangliomas seen at our institution in the period from 1980 through 1995 for their nonsporadic presentation or unusual morphological features and for the presence of sustentacular cells. RESULTS: Among 71 pheochromocytomas and paragangliomas in 60 patients, there were seven nonsporadic cases (11.5%), including three familial cases, two cases of multiple endocrine neoplasia 2a syndrome, one case associated with neurofibromatosis type 1, and one case of incomplete Carney's triad. In addition, we saw two cases of pheochromocytoma associated with metachronous malignant melanoma and one case of multicentric paraganglioma in a patient with Maffucci's syndrome. Unusual morphological features were as follows: some degree of coexisting cortical hyperplasia (eight cases), vacuolar degeneration of tumor cells (three cases), composite pheochromocytoma (one case), presence of pheochromoblasts and calcospherites (one case), melanin pigmentation (two cases), and insular growth pattern (four cases); the latter was associated with malignant behavior in two cases. Sustentacular cells varied in number but were consistently numerous in all nonsporadic cases. CONCLUSIONS: Nonsporadic cases of pheochromocytoma comprise around 10% of all cases in this as in other series. Some other, less well-recognized associations, eg, with malignant melanoma, may also belong to this group. Unusual morphological features occur in a substantial number of cases and may cause diagnostic problems.

Adolescent↗

PubMed1990

Carotid body paragangliomas. A clinicopathologic and DNA analysis of 13 tumors.

The clinical and pathological features of 13 carotid body paragangliomas from 12 patients were examined and correlated with the DNA ploidy pattern as determined by image analysis. These tumors occurred in 7 women and 5 men aged 19 to 62 years (average, 42 years). All presented with a slowly enlarging, usually asymptomatic mass of 2 weeks' to 25 years' duration. Two patients were related and had a family history of paragangliomas. The tumors ranged from 2 to 6 cm. All contained scattered chief cells with pleomorphic nuclei, two exhibited mitoses, and three showed perineural and three vascular invasion. Follow-up was available in all 12 patients and ranged from 15 months to 28 years (average, 7.3 years). None of the tumors recurred locally, but one did metastasize to a single cervical lymph node that was apparent at the time of diagnosis. Of 13 carotid body paragangliomas examined for DNA, 4 were diploid, 3 diploid-tetraploid, 3 tetraploid, 2 aneuploid, and 1 polyploid. The only malignant tumor was polyploid. From these observations, we conclude that abnormalities in DNA content of carotid body paragangliomas are common and that tumor ploidy cannot be used to assess malignant potential. We also found no apparent relationship among nuclear pleomorphism, mitotic activity, perineural invasion, or vascular invasion and clinical behavior. Perineural and vascular invasion, however, were observed only in tumors with abnormal DNA histograms.

Adult↗

PubMed2000

Diagnostic challenges in the fine-needle aspiration diagnosis of carotid body paragangliomas: report of two cases.

Two cases of carotid body paragangliomas sampled by fine-needle aspiration (FNA) cytology prior to other medical diagnostic studies are presented. In the first case, the presence of an ipsilateral ulcerative lesion of the nasopharynx along with pronounced atypia of the specimen posed a challenge to the correct cytologic interpretation, which was initially sidetracked in favor of a metastatic epithelial lesion. In the second case, a tumor mass of unusually large size and extension which included the pharynx, coupled with a large amount of profusely hemorrhagic aspirate, presented a diagnostic problem, which was overcome by processing part of the specimen as a cell block, which by its histologic and immunochemical features provided a definitive pathologic diagnosis. In handling these two clinically complex cases of carotid paraganglioma, two learning principles became clear on how to reach a correct FNA diagnosis in such lesions: 1) The anatomic location of the lateral neck mass with its prolonged history, along with a hemorrhagic FNA specimen exhibiting at least some cytologic features reminiscent of endocrine neoplasm, are among the factors that help in arriving at a suggestive diagnosis of paraganglioma, when other clinical features tend to sidetrack from interpretation of the cytologic changes. 2) In the practice of FNA cytology, if the possibility of paraganglioma arises, processing part of the specimen as a cell block with accompanying histology and immunohistochemistry can provide a definitive diagnosis of such lesion.

Biomarkers, Tumor↗

PubMed1997

Paraganglioma-Like Adenomas of the Thyroid (PLAT): Incidental Lesions with Unusual Features in a Patient with Nodular Goiter.

We discovered two well-demarcated nodules incidentally in a thyroid removed because of a nodular goiter. Histologically, the nodules showed a pattern of paraganglioma or so-called paraganglioma-like adenoma of the thyroid (PLAT), with lobules of polygonal and oval cells in a vascular stroma, but the immunohistochemical markers typical of paraganglioma, including chromogranin, synaptophysin, Leu 7 and 5-100, and thyroglobulin, characteristic of PLAT, were negative in the tumor cells. C-cell markers calcitonin and somatostatin were also negative. Stain for neuron-specific enolase (NSE), however, showed a distinctive pattern of reactivity within cells at the periphery of the lobules, whereas all tumor cells stained positively for keratins. Stain for carcinoembryonic antigen showed a focal interstitial pattern that corresponded to small intercellular spaces filled by microvilli identified ultrastructurally. This pattern of immunohistochemical staining has not been previously described in paraganglioma or in PLAT, and may have implications about the origin and nature of these controversial entities.

Journal Article↗

PubMed2004

Octreotide scintigraphy in the detection of recurrent paragangliomas.

OBJECTIVE: Conventional follow-up of surgically and radiotherapy-treated paragangliomas is usually carried out with physical examinations and either magnetic resonance imaging and/or computed tomography. Up to now, octreotide has been reserved for primary diagnosis, for which it has been shown to be a reliable imaging modality. However, the postsurgical and postradiation changes seen on conventional imaging make the diagnosis of small recurrent lesions difficult. We sought to evaluate the role of octreotide scintigraphy in the detection of recurrent paragangliomas. STUDY DESIGN: We based this case series on 3 patients who were found to have recurrent paragangliomas after primary surgical resection. All patients underwent octreotide scintigraphy during routine follow-up. RESULTS: Three patients were found to have recurrent paragangliomas using octreotide scintigraphy. In one of the patients, conventional imaging (magnetic resonance imaging, computed tomography) failed to detect the recurrence. CONCLUSION: The octreotide study was able to detect the small recurrences because octreotide binds directly to receptors on the tumor and is not affected by postsurgical or postradiotherapy changes. The intrinsic resolution of most commercially available gamma cameras is about 1 cm, making it quite sensitive to detect small tumors. Although this patient did not have synchronous lesions, octreotide scintigraphy can also aide in the detection of such lesions.

Adult↗

PubMed1995

Studies for estimating the biologic behavior and prognosis of paragangliomas in the head and neck.

Despite a large number of histopathologic and immunohistochemical studies, the biologic behavior and prognosis of paragangliomas (glomus tumors) of the head and neck still remain uncertain. In the present study 36 specimens from 32 patients who underwent surgery for a paraganglioma were examined. The examinations included routine histology, quantitative DNA analysis based on image cytometry, immunohistochemical detection of the proliferating cell nuclear antigen (PCNA) along with visualization of nucleolar organizer regions (AgNOR). According to LeCompte, the paragangliomas were histologically divided into three subcategories: 16 patients had a paragangliomatous tumor. 14 patients had an adenomatous tumor, and 6 patients had an angiomatous tumor. Quantitative DNA analysis revealed three categories of tumors with characteristical DNA pattern; DNA type I tumors were pure diploid, DNA type II tumors had stemlines at 2c and 4c and were therefore recognized as diploid-tetraploid. Aneuploid cells were not apparent in these two groups. DNA type III tumors had stemline ploidies exceeding 2c and 4c. Aneuploid cells were present in all of these tumors. The biologic behavior of these lesions therefore must be recognized as suspicious. DNA type III tumors and adenomatous tumors showed the highest values for the PCNA scores, indicating a higher proliferation rate and a more rapid growth pattern in these lesions. Twenty patients could be followed over a period of up to 110 months. Five of these patients developed a recurrent tumor. All of them had DNA type III tumors. The DNA indices showed significantly higher values in the recurrent tumor group. The 2c deviation index (DI) and the entropy value had the highest prognostic significance. No correlation to clinical follow-up was found for the AgNOR score. Based on these results, prognostic indices for paragangliomas were developed: patients with a tumor having a 2c DI exceeding 2.0, entropy value of more than 4.0. 5c exceeding rate more than 8.0, and a PCNA score more than 20.0% can be recognized as being at high-risk for developing recurrent disease.

Journal Article↗

PubMed2006

Resection of a recurrent paraganglioma via an endoscopic transnasal approach to the jugular fossa.

OBJECTIVE: To present a novel endoscopic, transnasal approach to a recurrent paraganglioma of the jugular fossa (glomus jugulare). STUDY DESIGN: Case report and review of the literature. SETTING: The study was carried out at the University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, U.S.A. PATIENT: A 64-year-old woman presented to the University of Pittsburgh Medical Center with a 9-month history of left otalgia, occasional vertigo, facial pain, and recurrent epistaxis. She had undergone two previous procedures over the past 35 years for a glomus tympanicum. Physical examination revealed a mass in the left nasopharynx originating from the eustachian tube. A computed tomographic scan revealed an expansive mass in the left jugular foramen extending into the posterior parapharyngeal space suggestive of a large jugular paraganglioma. INTERVENTION: The patient underwent preoperative embolization of the paraganglioma. An endoscopic, transnasal approach along the eustachian tube, the ascending parapharyngeal carotid artery, and into the jugular fossa was used to remove the tumor. Intraoperative image guidance assisted in the identification of key anatomic landmarks. RESULTS: Postoperative magnetic resonance imaging revealed a thin rim of enhancement at the posterior aspect of the jugular fossa consistent with residual tumor. The patient was discharged to home on postoperative Day 1 in stable condition. There have been no complications with 4 months of follow-up, and the left facial paralysis secondary to preoperative embolization has resolved. CONCLUSION: We report the successful subtotal resection of a recurrent paraganglioma via a novel endoscopic, transnasal, transclival, transpetrous approach with image guidance. This approach allowed the near-total resection of a recurrent glomus jugulare with minimal surgical morbidity. Technological advances and surgical experience with nasal endoscopy and image guidance allow minimally invasive surgical management of select extracranial lesions of the lateral cranial base.

Embolization, Therapeutic↗

PubMed1983

Malignant catecholamine-secreting carotid body paraganglioma.

The second known case of a malignant catecholamine-secreting (DA)-secreting carotid body paraganglioma is presented. Dopamine synthesis and secretion can be increased in malignant tumors derived from neural crest cells. Whether this is true, in addition, for extra-adrenal paragangliomas is not yet clear. Malignant paragangliomas of the carotid body and larynx, although rare, frequently have been accompanied by increased catecholamine secretion. Malignant catecholamine-secreting carotid body paragangliomas are best treated by composite resection (internal carotid artery and neck dissection), with special attention being given to measures preventing severe hypertension and arrhythmias in the perioperative period.

Adult↗

PubMedDate not supplied

A multidisciplinary approach to carotid paragangliomas.

The surgical management of carotid paragangliomas can be problematic. A multidisciplinary approach was used to include vascular surgery, otolaryngology, and neuroradiology to treat these patients over 9 years. From January 1992 to July 2001, a multidisciplinary team evaluated patients with carotid paragangliomas. Analyzed patient data included age, gender, diagnostic evaluation, tumor size, preoperative tumor embolization, operative exposure, need for extracranial arterial sacrifice/reconstruction, postoperative morbidity including cranial nerve dysfunction, and long-term follow-up. Twenty-five carotid paragangliomas in 20 patients underwent multidisciplinary evaluation and management. Average age was 51 years (range, 28-83 years), and 52% were male. Diagnostic evaluation included computed tomography in 76%, magnetic resonance imaging/magnetic resonance angiography in 52%, catheter angiography in 60%, and duplex ultrasonography in 16%. An extended neck exposure was required in 11 cases (44%), mandibulotomy was used once (4%), and mandibular subluxation was never required. The external carotid artery (ECA) was sacrificed in 8 cases (32%). The carotid bifurcation was resected in 1 patient (4%) requiring interposition reconstruction of the internal carotid artery. Preoperative tumor embolization was performed for 13 tumors (52%). Operative blood loss for patients undergoing preoperative embolization (Group I) was comparable to the nonembolized group (group II): group I lost 365 +/-180 mL versus 360 +/- 101 mL for group II (P = .48). This occurred despite larger tumors (group I - 4.2 cm versus group II - 2.1 cm, P = .03) and a higher mean Shamblin class (group I - 2.5 versus group II - 1.45, P = .001) for group I. There were no perioperative mortalities. Transient cranial nerve dysfunction occurred in 13 CBTs (52%), 2 (8%) of which remained present after 4 months. Patients with carotid paragangliomas benefit from a multidisciplinary team approach. Neuroradiology has been used for selective preoperative embolization, which has decreased estimated blood loss during excision of larger complex tumors. A combined surgical team of otolaryngology and vascular surgery provides for exposure of the distal internal carotid artery as high as the skull base, limited permanent cranial nerve dysfunction, and selective early division and excision of the external carotid artery for complete tumor resection.

Adult↗

PubMed2005

Locally advanced duodenal gangliocytic paraganglioma treated with adjuvant radiation therapy: case report and review of the literature.

BACKGROUND: Gangliocytic paraganglioma are rare neoplasms that predominantly arise in periampulary region. Though considered benign the disease can spread to regional lymphatics. CASE PRESENTATION: A 49 year old woman presented with melena and was found to have a periampullary mass. Endoscopic evaluation and biopsy demonstrated a periampullary paraganglioma. The tumor was resected with pylorus-preserving pancreaticoduodenectomy and was found to represent a gangliocytic paraganglioma associated with nodal metastases. In a controversial decision, the patient was treated with adjuvant external beam radiation therapy. She is alive and well one year following resection. The authors have reviewed the current literature pertaining to this entity and have discussed the biologic behavior of the tumor as well as the rationale for treatment strategies employed. CONCLUSION: Paraganglioma is a rare tumor that typically resides in the gastrointestinal tract and demonstrates low malignant potential. Due to rarity of the disease there is no consensus on the adjuvant treatment even though nearly 5% of the lesions demonstrate the malignant potential.

Journal Article↗

PubMed1997

Intrapericardial paragangliomas (pheochromocytomas): imaging features.

OBJECTIVE: The imaging features of intrapericardial paragangliomas (pheochromocytomas) are described. MATERIALS AND METHODS: We conducted a retrospective study of the imaging features of all intrapericardial paragangliomas seen at our institution over the last 13 years. RESULTS: In this study, intrapericardial paragangliomas were typically located adjacent to or involved the left atrium. The diameter of the tumors ranged from 3 to 8 cm. Metaiodobenzylguanidine (MIBG) scintigraphy revealed 11 of 12 tumors (sensitivity = 92%). After MIBG scintigraphic location, dynamic contrast-enhanced CT revealed all 12 tumors. CONCLUSION: Intrapericardial paragangliomas are rare tumors, typically located adjacent to or involving the left atrium. For initial detection, regional location of these extraadrenal tumors, and detection of distant metastases, MIBG scintigraphy is recommended. Dynamic contrast-enhanced CT or MR imaging can then provide detailed anatomic delineation before surgical resection.

3-Iodobenzylguanidine↗

PubMed2004

[Malignant jugular paraganglioma: a case report].

Paragangliomas are usually benign tumors. Overall, less than 10% of all paragangliomas become malignant. Although the indicator of malignancy is regarded as detection of histopathological findings of tumor by some authors, most authors accept extension to regional lymph nodes or distant metastasis as the only reliable indicators of malignancy. A 66-year old woman was admitted to our hospital with hearing loss, vertigo, and hoarseness. CT examination revealed a mass in the left jugular foramen. The tumor was confirmed as malignant paraganglioma. Indicators of malignant paragangliomas are discussed in this article.

Aged↗

PubMed1997

[Gangliocytic paraganglioma. A case report and review of the literature].

Gangliocytic paraganglioma is an uncommon and usually benign lesion although some cases with metastasis to regional lymph nodes have been described. It is usually located in the descending duodenum submucosa and more frequently arises in male patients. It comes to clinical attention for discomfort, gastrointestinal bleeding, incidental finding and rarely for obstructive jaundice when it involves the papilla. Even if its histologic and immunocytochemical features have been thoroughly described, its histogenesis is still debated although hyperplastic and amartoma-choristoma theories are well considered. We report a case of duodenal gangliocytic paraganglioma in 46 years old chronic alcoholic man who underwent previous upper endoscopy for discomfort without diagnosing the lesion. This one was observed during a second upper endoscopy that was made for hemathemesis. But it was only after an episode of melena that a third upper endoscopy showed the paraganglioma that still kept hemorrhage stigmata and no other lesions. We describe this case of duodenal gangliocytic paraganglioma with review of the literature.

English Abstract↗

PubMed2005

Predictors and prevalence of paraganglioma syndrome associated with mutations of the SDHC gene.

CONTEXT: Paraganglioma syndrome includes inherited head and neck paragangliomas (HNPs) and adrenal or extra-adrenal pheochromocytomas and are classified according to the susceptibility genes SDHB, SDHC, and SDHD. In contrast with those with germline mutations of the SDHB and SDHD genes, clinical and genetic data on patients with mutations of SDHC are scarce. OBJECTIVE: To determine the prevalence and clinical characteristics of SDHC mutation carriers compared with patients with SDHB and SDHD mutations and with sporadic cases. DESIGN, SETTING, AND PATIENTS: Genetic screening for SDHC mutations in an international HNP registry of 121 unrelated index cases and in 371 sporadic cases from a pheochromocytoma registry, conducted January 1, 2001, until December 31, 2004. Identified index cases and affected relatives were clinically evaluated. MAIN OUTCOME MEASURES: Prevalence of and clinical findings for SDHC mutation-associated HNPs vs those with SDHB and SDHD mutations. RESULTS: The prevalence of SDHC carriers was 4% in HNP but 0% in pheochromocytoma index cases. None of the SDHC mutation carriers had signs of pheochromocytoma. We compared HNPs in 22 SDHC mutation carriers with the HNPs of SDHB (n = 15) and SDHD (n = 42) mutation carriers and with 90 patients with sporadic HNPs. Location, number of tumors, malignancy, and age were different: more carotid body tumors were found in SDHC (13/22 [59%]) than in sporadic HNPs (29/90 [32%], P = .03), as well as fewer instances of multiple tumors in SDHC (2/22) than in SDHD (24/42; P<.001), 0 malignant tumors in SDHC vs 6/15 in SDHB (P = .002), and younger age at diagnosis in SDHC than in sporadic HNPs (45 vs 52 years; P = .03). CONCLUSIONS: Patients with HNP, but not those with pheochromocytoma, harbor SDHC mutations in addition to those in SDHB and SDHD. In total, more than one quarter of HNP patients carry a mutation in 1 of these 3 genes. Head and neck paragangliomas associated with SDHC mutations are virtually exclusively benign and seldom multifocal. Analysis for germline mutations of SDHC is recommended in apparently sporadic HNP to identify risk of inheritance.

Adolescent↗