Mesenteric Paraganglioma Research Articles

Mesenteric paraganglioma with a late-onset metastases accelerated after delayed surgery

Searchable abstracts of presentations at key conferences in endocrinology ISSN 1470-3947 (print) | ISSN 1479-6848 (online)

GIANT PULMONARY CHONDROMAS AS A COMPONENT OF CARNEY’S TRIAD

Carney’s triad is a rare syndrome, comprising of the GIST of the stomach, functioning extra-adrenal paraganglioma and pulmonary chondroma. We present a case of a young woman diagnosed with gastric GIST, mesenteric paraganglioma and two giant chondromas of the left lung. Due to the rapid growth of pulmonary neoplasms and their potential malignant behaviour, the left pneumonectomy was performed.

Mesenteric Paraganglioma-A Cyst in The Mist

Extraadrenal paraganglioma is rarely found in the mesentery. It accounts only for 5-10% of cases and may present incidentally as abdominal mass. We report a case of 30 year old male presented as mass per abdomen. A differential diagnosis of neurogenic tumor or cystic mesothelioma was considered based on CT-abdomen. Histopathological examination showed features of mesenteric paraganglioma and confirmed on immunohistochemistry. This case report highlighted several important key issues regarding diagnosis of paraganglioma, including malignant or metastatic potential, recurrence rate, pre and postoperative imaging study and lifelong follow-up of the patient.

Paraganglioma presenting as a mesenteric cystic mass: A case report.

The present study aims to raise awareness about the necessity of a thorough differential diagnosis process when diagnosing paraganglioma as it is an extremely rare entity with a high malignant potential. A 64-year-old female patient was admitted to the hospital with symptoms of bowel obstruction and a palpable abdominal mass. Abdominal and pelvic computed tomography revealed a cystic heterogeneously enhanced mass. The tumor was surgically removed and then microscopically analysed. Microscopically, the mass was composed of nests of small polygonal and round cells with central vesicular nuclei, consistent with the diagnosis of mesenteric paraganglioma which was later confirmed by immunohistochemistry. The critical markers for paragangliomas, namely chromogranin A and S100, should be used as the first step in diagnosis, followed by other valuable immunohistochemical markers. A long-term follow-up is extremely important following the diagnosis of paraganglioma as all these tumors have a malignant potential.

A rare mesentric tumor “paraganglioma” – a case report

Paraganglioma are rare type of neuroendocrine tumors that arise from embryonal neural crest. Paraganglioma of adrenal medulla is known as Pheochromocytoma. Extra adrenal localization has incidence of 2-8 per million people per year, which can extend from upper cervical region to pelvis related to autonomic nervous system. Even rarer is occurrence of paragangliomas outside the usual distribution of paraganglionic tissue. Mesenteric paragangliomas are extremely rare. The present case was of a non-functional extra adrenal paraganglioma occurring in a 43-year-old male presented with complain of pain in abdomen and mild discomfort. Abdominal CECT revealed well defined enhancing solid mass with central necrosis in right side of mesentry. Explorative laparotomy and surgical excision were done, on histology typical “zellballen” pattern was found. Paragangliomas are rare neuroendocrine tumor known to store and secrete catecholamines and hence are known as functional paragangliomas and may present with symptoms like headache, sweating, palpitations and hypertension. On other hand, they may remain silent and non-functional and present with vague symptoms like pain abdomen due to episodic release of catecholamines. Surgical excision is treatment of choice. Long term follow up is mandatory as tumor is prone to recur and metastasize.

El paraganglioma como tumor mesentérico infrecuente

Paragangliomas are rare neuroendocrine tumors that arise in the autonomic nervous system. Although these tumors are considered benign, they must be removed by surgery due to their potential malig- nant transformation. Mesenteric paragangliomas are extremely rare.

An unusual presentation of Mesenteric Paraganglioma

Introduction: Paragangliomas are rare tumors derived from neural crest cells, which are mostly localized in the adrenal medulla. Only 5–10% occur in extra-adrenal localizations. Given the potential of the paraganglioma to secrete catecholamines, some patients present constitutional symptoms such as headaches, tremors and hypertension. However, most patients are asymptomatic. Preoperative diagnosis of paraganglioma in asymptomatic patients is difficult but should be included in the differential diagnosis of solid mesenteric tumors. The gold standard therapy is complete resection, with the need for a long-term follow-up after surgery. Case Report: Our case study takes a look at a 32-year-old nulliparous woman observed in the emergency room with a severe low abdominal pain. The ultrasonography and computerized tomography revealed a large pelvic tumor with apparent origin in the left ovary. During an exploratory laparotomy, an encapsulated, well vascularized mass was found in the mesentery of the ileum, behind the uterus, conditioning the twisting and consequent bottlenecks of 30 cm of the small intestine. An enterectomy was performed and the final histopathology revealed a 6 cm paraganglioma. Conclusion: This case is unique due to its unusual location and clinical presentation. Only 20 cases of mesenteric paraganglioma are described and this is the first found in the context of an emergency.

Incidental Mesenteric Paraganglioma: A Case Report and Literature Review.

Mesenteric paragangliomas are a rare entity; consequently, only 12 cases have been reported to date. Although considered benign and often found incidentally, they have the potential to metastasize and are an important diagnosis to consider for patients with a mesenteric tumor. We report the case of a 71-year-old woman who was found on magnetic resonance imaging to have an incidental, large, complex, intra-abdominal mass that had initially been misdiagnosed as ovarian in origin. She underwent an open resection of the tumor and adjacent small bowel with no perioperative complications; histology confirmed the mass to be a mesenteric paraganglioma. This case report highlights several important key issues regarding paraganglioma, including diagnosis, imaging, genetic testing, and surgical resection. The results of a literature review are also discussed.

Long-Term Sequelae and Late Effects In Childhood Acute Lymphoblastic Leukemia (ALL) Survivors: A Single AIEOP Center Experience

The advent of more intensive chemotherapy and the improvement of supportive cares have dramatically changed the natural history of childhood acute lymphoblastic leukemia (ALL), with current estimated 5-year overall survival of about 80%. The increased survival rate and the establishment of follow-up survey for long term survivors (LTS) have allowed the identification of late chemo-radiotherapy adverse effects on psychological and general health. We retrospectively evaluate the incidence and type of sequelae and / or late effects in a cohort of 301 childhood ALL LTS, followed in a single pediatric AIEOP center.From June 1986 to June 2013, 301 LTS (154 male and 147 female), aged <18 years at ALL diagnosis, were followed-up by a multidisciplinary team. The surveillance protocol is summarized in Figure 1. The timing of follow-up (FU) was modified, case by case, in relation to the appearance of adverse events or organ diseases. Survivors' results were compared with chronic medical and psychological conditions of siblings (n=89). [Display omitted] Mean FU time (time from the stop therapy to the last control) was 6 years (range 1.8-26.8 years). The majority of LTS were teenager or young adults : 35% ranged between 15 and 20 years; 19.7% was more than 21 years old and the 45.3% was less than 14 years old. During FU, 16 late recurrences (5.3%) were identified and 3 secondary malignancies (0.99%) such as one mesenteric paraganglioma and two Acute Myeloid Leukemia in second complete remission ALL LTS. 40 patients (13.3%) received cranial radiotherapy during treatment. 39 LTS (13%) reported at least one sequelae. The most frequent sequelae were neurological and orthopedic (6% and 3% respectively) as summarized in Figure 2 151 LTS (50.17%) presented at least one late effect as showed in Figure 3. [Display omitted] [Display omitted] In our experience at least one late effect occurred in 50.17% of LTS; these late complications affect negatively the quality of life of survivors. Endocrine-metabolic events are the most frequent late effect (34,5%). 13% of LTS have at least one sequelae mainly neurological and orthopedic. Prevention and/or early identification of complications during follow-up survey of LTS are crucial in order to decrease the long-term health risks associated with curative treatment for childhood ALL. Disclosures:No relevant conflicts of interest to declare.

Mesenteric paraganglioma: Report of a case

We report a rare case of paraganglioma that developed in the mesentery of terminal ileum. A 78-year-old woman complained of right-sided abdominal pain. Abdominal computed tomography revealed a solid heterogeneously enhanced mass in the right lower abdomen. The tumor was laparoscopically excised. The mesenteric tumor was well circumscribed, ovoid, and encapsulated and measured 3 cm × 1.5 cm × 1.5 cm. Histological examination showed a cellular neoplasm comprised of nests and groups of tumor cells separated by fibrovascular connective tissue, giving a characteristic nested Zellballen pattern. Immunohistochemically, the tumor cells were positive for chromogranin, synaptophysin, CD56, and vimentin and negative for cytokeratins, SMA, CD34, CD117/c-kit and S100. On the basis of histologic and immunohistochemical features, a diagnosis of mesenteric paraganglioma was made. The operative and postoperative courses were unremarkable, and the patient was discharged on postoperative day 7. She was doing well 1 year after the surgery with no signs of recurrence. Extra-adrenal paragangliomas most commonly develop adjacent to the aorta, particularly the area corresponding to the organ of Zuckerkandl. Mesenteric paraganglioma, as in our case, is extremely rare; only 11 cases have been reported in the literature. We herein discuss the clinical findings of these cases.

A Rare Surgical Tumor of Mesenteric Paraganglioma Presenting As A Fibroid Uterus

Paragangliomas are rare tumors originating from the parenchymal cells of paraganglia. In adults the adrenal medulla is the largest paraganglion and paragangliomas most frequently occur in the adrenal medulla. We report a rare case of a solitary primary paraganglioma arising in the mesentery, found in a 38 yr old woman who presented with complaints of infertility dysmenorrhoea, mass per abdomen, abdominal distension since 6 months. Clinical examination and ultrasound abdomen suggested multiple fibroids. Exploratory laparotomy revealed a large mesenteric cyst The histopathology and immunohistochemistry confirmed the diagnosis of mesenteric paraganglioma. Key words :Paraganglioma, Fibroid

Mesenteric paraganglioma with gallbladder paraganglion nest.

We report on an original case of a 64 year old lady diagnosed with an extraadrenal mesenteric paraganglioma associated with an incidental histological finding of a gallbladder paraganglion. Paragangliomas are rare neuroendocrine, neural crest-derived tumours, found in diverse locations and display variable catecholamine secreting behaviour. Malignant potential can be difficult to predict on histology. We review the current literature on these multifarious tumours.

Malignant paraganglioma of the mesentery: a case report and review of literature

Paragangliomas represent only 10% of chromaffin tissue tumors and those arising from the mesentery seem to be a rare occurrence. We report a case of a 55 year old man in whom an abdominal mass was discovered fortuitously by ultrasonography during a routine health exam. He presented occasional heart palpitations and diaphoresis as well as a well-demarcated mass upon abdominal physical examination. CT scan revealed a solid polylobulated mass in the right lower quadrant. Exploration laparotomy revealed a voluminous multi-nodular tumoral mass, which contained hemorrhagic spots. Histopathological studies confirmed the presence of a paraganglioma. The excision of the mass as well as the surrounding intestine and mesentery also revealed two lymphatic metastases, the first among 14 documented cases to be described concerning mesenteric paragangliomas. One year follow up and CT scan revealed neither recurrence nor the presence of distant metastases.

A mesenteric paraganglioma

Mesenteric paraganglioma is a solitary primary paraganglioma that occurs in the mesentery. It is a rare and unusual tumor, especially in non-typical sites such as the inferior mesenteric artery. We incidentally diagnosed a pelvic mass in a 22-year-old woman, who had not experienced vomiting, headache or hypertension. She was found to have an immobile solid mass on the left side of her pelvis, adjacent to the upper segment of the uterus. CT scans revealed a well-defined solid and cystic ovoid mass adjacent to the sigmoid colon and the uterus. Tumor markers were within the normal range. Surgery was performed to completely remove the mass. An intra-operative frozen section biopsy showed a benign cystic tumor. Histopathological examination indicated that it was a paraganglioma of the mesentery. Repeat CT examination did not reveal any local recurrence or distant metastasis during the 13-month follow-up period.

Mesenteric Paraganglioma with Cystic Degeneration: Case Report

Extra-adrenal abdominal paragangliomas are rare. Most arise from the organs of Zuckerkandl, involve large concentrations of paraganglionic tissue, and are located in the para-aortic space along the sympathetic chain. Published reports have, however, described normal paraganglionic tissue at the root of the mesentery which serves as the superior limit of the organs of Zuckerkandl, and mesenteric paraganglioma is very rare. We report a case of paraganglioma with cystic degeneration arising from the mesentery.

Mesenteric paraganglioma: a case report and review of the literature.

Approximately 5% to 10% of paragangliomas occur in extra-adrenal sites, which can extend from the upper cervical region to the pelvis, parallel to the autonomic nervous system. This distribution corresponds to the embryologic development of the paraganglia from neural crest cells. Rarely, extra-adrenal paragangliomas can also occur aberrantly outside this distribution. We report such a case of extra-adrenal paraganglioma occurring in the anterior mesentery in a 76-year-old man. Two case reports exist in the literature describing extra-adrenal paragangliomas in the posterior mesentery. Normal paraganglionic tissue has been described at the roots of the superior and inferior mesenteric arteries, theoretically explaining the origin of the posterior mesenteric paragangliomas. Our case can best be attributed to the ventral migration of paraganglionic tissue through these vessels to reach the anterior mesentery, where they could potentially give rise to paragangliomas in this site.