Surgical approach to rare case of recurrent pheochromocytoma and bones metastatic paraganglioma. (original) (raw)
Related papers
Bone metastasis of malignant pheochromocytoma four years after adrenalectomy
Russian Open Medical Journal, 2016
Malignant adrenal pheochromocytoma is an uncommon entity with low 5-year survival rate. Early diagnosis and adrenalectomy often yields good clinical outcome. The aim of the present case study is to report a 64-year-old Brazilian man with bone metastases of malignant pheochromocytoma first diagnosed and treated by right adrenalectomy four years ago. Recent evidence emphasized the recommendation about the safest duration of follow up, which should be at least 10 years from the first operation or first diagnosis of adrenal pheochromocytoma.
Pheochromocytoma and paraganglioma: from treatment to follow-up
Şişli Etfal Hastanesi tıp bülteni, 2020
P heochromocytomas (PCC) and paragangliomas (PGL) are rare neuroendocrine tumors arising from chromaffin cells in the adrenal medulla, sympathetic or parasympathetic ganglia. [1, 2] Currently, the only curative treatment option of pheochromocytomas/paraganglioma (PPGL) is surgical resection. Surgery aims to eliminate both risks of hypersecretion and tumor growth. The consequences of hypersecretion should be carefully controlled with medical therapy before and during the surgery. The choice of surgical approach is determined based on multiple factors, including germline genetic test results, the size of the tumor, body mass index, surgeon's experience, and the like-Pheochromocytomas (PCC) and paragangliomas (PGL) are rare neuroendocrine tumors arising from chromaffin cells in the adrenal medulla, sympathetic or parasympathetic ganglia. Currently, the only curative treatment option of pheochromocytomas/paraganglioma (PPGL) is surgical resection. Surgery aims to eliminate both risks of hypersecretion and tumor growth. The consequences of hypersecretion should be carefully controlled with medical therapy before and during the surgery. Postoperative major complications are hypotension and rebound hypoglycemia, and patients should be followed closely for 24-48 hours. The choice of surgical approach is determined based on multiple factors, including germline genetic test results, the size of the tumor, body mass index, surgeon's experience, and the likelihood of malignancy. Primary tumor resection does not completely eliminate the risk of tumor persistence and recurrence. Therefore, all patients with PPGL who are surgically treated should be followed for at least 10 years for recurrent disease and new tumor formation. Although surgical resection is the only curative treatment for PPGLs, surgical treatment is palliative except for resectable locoregional metastases in metastatic disease or for isolated distant metastases. The purpose of palliative treatment is to reduce hormone secretion and prevent metastasis-related complications in a critical anatomical location Combined and alfa;-and beta-adrenergic blockade is usually applied in patients with PPGL preoperatively. Some patients may present with pheochromocytoma multisystem crisis, which is a life-threatening condition that can involve cardiovascular, pulmonary, neurological, gastrointestinal, renal, hepatic and metabolic systems. Pheochromocytoma crisis may be spontaneous or may present with the tumor manipulation, trauma, corticosteroids, beta-blockers, anesthetic drugs, and the stimulation of nonadrenal surgical stress. These patients should be considered as medical emergencies rather than surgical emergencies. In this review, it was aimed to evaluate the pre-, per and post-operative management, curative and palliative surgical management, and postoperative outcomes and follow-up of the patients with PPGLs.
Metachronous pheochromocytoma metastasis to the upper dorsal spine—6-year survival
The Spine Journal, 2008
BACKGROUND CONTEXT: Malignant pheochromocytoma is a rare neoplasm of chromaffin tissue. Very few cases of malignant adrenal pheochromocytoma metastatic to vertebrae exist. PURPOSE: To determine the prognosis of a patient with an excised adrenal pheochromocytoma and a single metachronous metastasis to the upper dorsal spine. STUDY DESIGN: Case report METHODS: The authors report a patient who underwent total excision of an adrenal pheochromocytoma of the left adrenal gland in 2000 who developed a single metastasis to the second dorsal vertebra in 2002 with no evidence of abdominal recurrence.
The Journal of Clinical Endocrinology & Metabolism, 2013
Context: Bone metastases (BM) can cause severe pain, spinal cord compression, pathological fractures, and/or hypercalcemia. These skeletal-related events (SREs) may cause immobilization, loss of independence, poor quality of life, and reduced survival. There is limited information on the clinical effects of BM and SREs in patients with malignant pheochromocytoma or sympathetic paraganglioma (PHEO/sPGL). Objectives: We studied the prevalence and clinical characteristics of BM and SREs in patients with PHEO/sPGL and investigated the risk factors for SRE development. Design: Using a large institutional database, we conducted a retrospective study of 128 patients with malignant PHEO/sPGL at The University of Texas MD Anderson Cancer Center from 1967 through 2011. Results: Of the patients, 91 (71%) had BM, and 57 of these (63%) developed metachronous BM at a median time of 3.4 years (range, 5 months to 23 years) after the primary tumor diagnosis. Metastatic disease was confined exclusively to the skeleton in 26 of 128 (20%) patients. Sufficient information to assess SRE occurrence was available for 67 patients, and 48 of 67 (72%) patients had at least 1 SRE. The median overall survival for the 128 patients was 12 years for patients with only BM, 7.5 years for patients with nonosseous metastases, and 5 years for patients with both BM and nonosseous metastases (log rank test P value ϭ .005). We were unable to identify factors predictive of SRE development, but the occurrence of a first SRE was associated with the development of subsequent SREs in 48% of subjects. In responsive patients, the use of systemic therapy was associated with fewer SREs (P Ͻ .0001). Conclusions: BM and SREs are frequent in patients with malignant PHEO/sPGL. SREs often develop shortly after the diagnosis of BM; severe pain is the most frequent SRE. These patients should be followed long-term by a multidisciplinary team to promptly identify the need for medical or surgical intervention.
Treatment for Malignant Pheochromocytomas and Paragangliomas: 5 Years of Progress
Current oncology reports, 2017
The purpose of this manuscript is to review the progress in the field of therapeutics for malignant pheochromocytomas and sympathetic paraganglioma (MPPG) over the past 5 years. The manuscript will describe the clinical predictors of survivorship and their influence on the first TNM staging classification for pheochromocytomas and sympathetic paragangliomas, the treatment of hormonal complications, and the rationale that supports the resection of the primary tumor and metastases in patients with otherwise incurable disease. Therapeutic options for patients with bone metastasis to the spine will be presented. The manuscript will also review chemotherapy and propose a maintenance regimen with dacarbazine for patients initially treated with cyclophosphamide, vincristine, and dacarbazine. Finally, the manuscript will review preliminary results of several phase 2 clinical trials of novel radiopharmaceutical agents and tyrosine kinase inhibitors. MPPGs are very rare neuroendocrine tumors....
Pheochromocytoma and paraganglioma: from epidemiology to clinical findings
SiSli Etfal Hastanesi Tip Bulteni / The Medical Bulletin of Sisli Hospital, 2020
P heochromocytoma (PCC) and paraganglioma (PGL) are rare neuroendocrine tumors. Pheochromocytomas arise from chromaffin cells in the adrenal medulla, and PGLs arise from chromaffin cells in the ganglia of the autonomic nervous system. [1, 2] Paragangliomas originate from sympathetic or parasympathetic ganglia in the abdomen, thorax, and pelvis. Paragangliomas may also originate from parasympathetic ganglia at the base of the skull and along the glossopharyngeal nerve and vagus nerve in the neck. [2] The majority of PCCs and sympathetic PGLs are endocrine active tumors, and they cause clinical symptoms by secreting excess catecholamines (norepinephrine, epinephrine, Pheochromocytomas (PCC) and paragangliomas (PGL) are rare neuroendocrine tumors. Pheochromocytomas arise from chromaffin cells in the adrenal medulla, and PGLs arise from chromaffin cells in the ganglia of the autonomic nervous system. Paragangliomas originate from sympathetic or parasympathetic ganglia in the abdomen, thorax, and pelvis. The majority of PCC and sympathetic PGL are endocrine active tumors causing clinical symptoms by secreting excess catecholamines (norepinephrine, epinephrine, dopamine) and their metabolites. The incidence of PCC and PGL ranges between 2 and 8 per million, with a prevalence between 1:2500 and 1:6500. It peaks between the 3rd and 5th decades of life, and approximately 20% of cases are pediatric patients. The prevalence among patients with hypertension in outpatient clinic ranges between 0.1-0.6% in adults and between 2-4.5% in the pediatric age group. 10-49% of these tumors is detected incidentally in imaging techniques performed for other reasons. However, 4-8% of adrenal incidentalomas are PCCs. Of these neuroendocrine tumors, 80-85% are PCCs and 15-20% are PGLs. Up to 40% of patients with PCC and PGL has disease-specific germline mutations and the situation is hereditary. Of 60% of the remaining sporadic patients, at least 1/3 has a somatic mutation in predisposing genes. 8% of the sporadic cases, 20-75% of the hereditary cases, 5% of the bilateral, adrenal cases, and 33% of the extra-adrenal cases at first presentation are metastatic. Although PCCs and PGLs have scoring systems for histological evaluation of the primary tumor, it is not possible to diagnose whether the tumor is malignant since there is no histological system approved for the biological aggressiveness of this tumor group. Metastasis is defined as the presence of chromaffin tissue in non-chromaffin organs, such as lymph nodes, liver, lungs and bone. Although most of the PCC and PGL are benign, the metastatic disease may develop in 15-17%. Metastatic disease is reported between 2-25% in PCCs and 2.4-60% in PGLs. The TNM staging system of the American Joint Committee on Cancer (AJCC) was developed to predict the prognosis, based on the specific anatomical features of the primary tumor and the occurrence of metastasis.
Perioperative Management of Pheochromocytomas and Sympathetic Paragangliomas
Journal of the Endocrine Society
Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors arising from chromaffin cells of the adrenal medulla or extra-adrenal paraganglia, respectively. PPGLs have the highest degree of heritability among endocrine tumors. Currently, ~40% of PPGL individuals have a genetic germline and there exist at least 12 different genetic syndromes related to these tumors. Metastatic PPGLs are defined by the presence of distant metastases at sites where chromaffin cells are physiologically absent. Approximately 10% of pheochromocytomas and ~40% of sympathetic paragangliomas are linked to metastases explaining why complete surgical resection is the first-choice treatment for all PPGL patients. The surgical approach is a high-risk procedure requiring perioperative management by a specialized multidisciplinary team in centers with broad expertise. In this review, we summarize and discuss the most relevant aspects of perioperative management in patients with pheochromocytomas an...
Malignant Pheochromocytoma with Widespread Bony and Pulmonary Metastases
2018
Pheochromocytoma is a rare benign tumor of the adrenal gland. A select few cases may be malignant, and metastatic cases are exceedingly rare. It often presents with symptoms of catecholamine excess, such as sweating, palpitations, headaches, and characteristic paroxysmal hypertension. Due to its diffuse symptoms, it is difficult to diagnose and is often diagnosed late. We describe the unique case of a 44-year-old female patient who presented with uncontrolled hypertension and vomiting, accompanied by lower back pain. She was diagnosed with malignant pheochromocytoma with multiple metastases to the lungs, vertebrae, scapulae, and skull. Because of the advanced state of her disease, the patient was started on treatment with the chemotherapeutic combination of cyclophosphamide, vincristine, and dacarbazine. However, she had a complicated hospital course and died because of aspiration pneumonia and sepsis.
Frontiers in Oncology, 2019
Background: Pheochromocytoma and paraganglioma (PHEO/PGL) are rare neuroendocrine tumors which may cause potentially life-threatening complications, with about a third of cases found to harbor specific gene mutations. Thus, early diagnosis, treatment, and meticulous monitoring are of utmost importance. Because of low incidence of succinate dehydrogenase complex subunit A (SDHA)-related metastatic PHEO/PGL, currently there exists insufficient clinical information, especially with regards to its diagnostic and treatment characteristics. Methods: Ten patients with SDHA-related metastatic PHEO/PGL were followed-up prospectively and/or retrospectively between January 2010-July 2018. They underwent biochemical tests (n = 10), 123 I-MIBG (n = 9) scintigraphy, and multiple whole-body positron emission tomography/computed tomography (PET/CT) scans with 68 Ga-DOTATATE (n = 10), 18 F-FDG (n = 10), and 18 F-FDOPA (n = 6). Results: Our findings suggest that these tumors can occur early and at extra-adrenal locations, behave aggressively, and have a tendency to develop metastatic disease within Jha et al. SDHA-Related Metastatic Pheochromocytoma/Paraganglioma a short period of time. None of our patients had a family history of PHEO/PGL, making them appear sporadic. Nine out of 10 patients showed abnormal PHEO/PGL-specific biochemical markers with predominantly noradrenergic and/or dopaminergic phenotype, suggesting their utility in diagnosing and monitoring the disease. Per patient detection rates of 68 Ga-DOTATATE (n = 10/10), 18 F-FDG (n = 10/10), 18 F-FDOPA (n = 5/6) PET/CT, and 123 I-MIBG (n = 7/9) scintigraphy were 100, 100, 83.33, and 77.77%, respectively. Five out of 7 123 I-MIBG positive patients had minimal 123 I-MIBG avidity or detected very few lesions compared to widespread metastatic disease on 18 F-FDG PET/CT, implying that diagnosis and treatment with 123/131 I-MIBG is not a good option. 68 Ga-DOTATATE PET/CT was found to be superior or equal to 18 F-FDG PET/CT in 7 out of 10 patients and hence, is recommended for evaluation and follow-up of these patients. All 7 out of 7 patients who received conventional therapies (chemotherapy, somatostatin analog therapy, radiation therapy, 131 I-MIBG, peptide receptor radionuclide therapy) in addition to surgery showed disease progression. Conclusion: In our cohort of patients, SDHA-related metastatic PHEO/PGL followed a disease-course similar to that of SDHB-related metastatic PHEO/PGL, showing highly aggressive behavior, similar imaging and biochemical phenotypes, and suboptimal response to conventional therapies. Therefore, we recommend careful surveillance of the affected patients and a search for effective therapies.