“…The average age of the patients presenting with an AMH was 47 years and in those with AMH plus PCC 44 years, which was 8 and 11 years earlier than in the AMH-devoid PCC control group. Also, other AMH cohorts have had similar age at presentation [5, 9, 11], which seems lower than the age of presentation in sporadic PCC [1]. The probable reason is that AMHs are more frequently associated with hereditary conditions, mainly MEN2 [3], which was also shown in our study.…”
Section: Discussionsupporting
confidence: 69%
“…Similar to patients with PCC, laparoscopic surgery was the preferred method for adrenalectomy in patients with AMH, with similar numbers requiring conversion to open surgery and having complications in addition to similar length of hospital stay postadrenalectomy. Both the case series of 7 patients with AMH by Marín et al [9] and of 12 patients with AMH by Yang et al [11] appeared to have comparable peri- and postoperative management as our cohort; however, surgical complications were not detailed. Thus, peri- and postoperative outcomes seem good in AMH, but if suspected preoperatively, AMH should have similar pre- and postoperative management as PCC including a laparoscopic approach.…”
Section: Discussionmentioning
confidence: 51%
“…In our 6- to 12-month follow-up data of hypertension, the majority were normotensive, suggesting that for patients with both AMH and PCC, adrenalectomy will cure hypertension in most patients nowadays, at least short term. Others have also shown increased blood pressure at presentation in patients with AMH [9, 11], with almost normalized blood pressure during follow-up [11].…”
Section: Discussionmentioning
confidence: 99%
“…Glycemic disturbances are common at PCC presentation [1, 16, 21, 26, 27], with dramatic improvements in insulin sensitivity by adrenalectomy [16, 26, 27]. Occasional case reports have described diabetes in patients with AMH [9, 10], but with unclear improvements in glycemic control postadrenalectomy. We had one case of diet-controlled diabetes in our AMH cohort, and surgery cured the condition.…”
Section: Discussionmentioning
confidence: 99%
“…There is limited literature about AMH with only case reports or small case series, and sporadic AMH has only occasionally been published [2, 3, 5–11]. AMH has been claimed to mimic PCC clinically with symptoms and signs of catecholamine excess [9–11], but no formal comparisons have been made.…”
Context
Information about adrenal medullary hyperplasia (AMH) is scarce.
Objective
To study a large cohort of AMHs.
Design, Setting, and Participants
Nineteen AMH cases were compared with 95 pheochromocytomas (PCCs) without AMH. AMH without (n = 7) and with PCC (n = 12) were analyzed separately.
Results
Of 936 adrenalectomies, 2.1% had AMH. Mean age was 47.2 ± 15.1 years. Only two (11%) AMHs had no concurrent PCC or adrenocortical adenoma. In AMHs, a genetic syndrome was present in 58% vs 4% in PCCs (P < 0.001). The noradrenaline/metanephrine levels were lower in AMHs, whereas suppression of dexamethasone was less than in PCCs. Cushing syndrome was found in 11% of AMHs. More AMHs were found during screening and less as incidentalomas. PCC symptoms were less prevalent in AMHs. Surgical management was similar; however, fewer of the AMHs were pretreated with alpha-blockers. Adrenalectomy improved blood pressure slightly less in AMHs. The disappearance of glycemic disturbances was similar to the PPCs. During a period of 11.2 ± 9.4 years, a new PCC developed in 32% of patients with AMH, 11% died, but no PCC metastasis occurred (PCCs: 4%, P < 0.001; 14% and 5%). AMHs without PCC had milder symptoms but more often Cushing disease than patients with PCC, whereas AMH with PCC more often displayed a familiar syndrome with more PCC recurrences.
Conclusion
A total of 2.1% of all adrenalectomies displayed AMH. AMH seemed to be a PCC precursor. The symptoms and signs were milder than PCCs. AMHs were mainly found due to screening. Outcomes seemed favorable, but new PCCs developed in many during follow-up.
“…The average age of the patients presenting with an AMH was 47 years and in those with AMH plus PCC 44 years, which was 8 and 11 years earlier than in the AMH-devoid PCC control group. Also, other AMH cohorts have had similar age at presentation [5, 9, 11], which seems lower than the age of presentation in sporadic PCC [1]. The probable reason is that AMHs are more frequently associated with hereditary conditions, mainly MEN2 [3], which was also shown in our study.…”
Section: Discussionsupporting
confidence: 69%
“…Similar to patients with PCC, laparoscopic surgery was the preferred method for adrenalectomy in patients with AMH, with similar numbers requiring conversion to open surgery and having complications in addition to similar length of hospital stay postadrenalectomy. Both the case series of 7 patients with AMH by Marín et al [9] and of 12 patients with AMH by Yang et al [11] appeared to have comparable peri- and postoperative management as our cohort; however, surgical complications were not detailed. Thus, peri- and postoperative outcomes seem good in AMH, but if suspected preoperatively, AMH should have similar pre- and postoperative management as PCC including a laparoscopic approach.…”
Section: Discussionmentioning
confidence: 51%
“…In our 6- to 12-month follow-up data of hypertension, the majority were normotensive, suggesting that for patients with both AMH and PCC, adrenalectomy will cure hypertension in most patients nowadays, at least short term. Others have also shown increased blood pressure at presentation in patients with AMH [9, 11], with almost normalized blood pressure during follow-up [11].…”
Section: Discussionmentioning
confidence: 99%
“…Glycemic disturbances are common at PCC presentation [1, 16, 21, 26, 27], with dramatic improvements in insulin sensitivity by adrenalectomy [16, 26, 27]. Occasional case reports have described diabetes in patients with AMH [9, 10], but with unclear improvements in glycemic control postadrenalectomy. We had one case of diet-controlled diabetes in our AMH cohort, and surgery cured the condition.…”
Section: Discussionmentioning
confidence: 99%
“…There is limited literature about AMH with only case reports or small case series, and sporadic AMH has only occasionally been published [2, 3, 5–11]. AMH has been claimed to mimic PCC clinically with symptoms and signs of catecholamine excess [9–11], but no formal comparisons have been made.…”
Context
Information about adrenal medullary hyperplasia (AMH) is scarce.
Objective
To study a large cohort of AMHs.
Design, Setting, and Participants
Nineteen AMH cases were compared with 95 pheochromocytomas (PCCs) without AMH. AMH without (n = 7) and with PCC (n = 12) were analyzed separately.
Results
Of 936 adrenalectomies, 2.1% had AMH. Mean age was 47.2 ± 15.1 years. Only two (11%) AMHs had no concurrent PCC or adrenocortical adenoma. In AMHs, a genetic syndrome was present in 58% vs 4% in PCCs (P < 0.001). The noradrenaline/metanephrine levels were lower in AMHs, whereas suppression of dexamethasone was less than in PCCs. Cushing syndrome was found in 11% of AMHs. More AMHs were found during screening and less as incidentalomas. PCC symptoms were less prevalent in AMHs. Surgical management was similar; however, fewer of the AMHs were pretreated with alpha-blockers. Adrenalectomy improved blood pressure slightly less in AMHs. The disappearance of glycemic disturbances was similar to the PPCs. During a period of 11.2 ± 9.4 years, a new PCC developed in 32% of patients with AMH, 11% died, but no PCC metastasis occurred (PCCs: 4%, P < 0.001; 14% and 5%). AMHs without PCC had milder symptoms but more often Cushing disease than patients with PCC, whereas AMH with PCC more often displayed a familiar syndrome with more PCC recurrences.
Conclusion
A total of 2.1% of all adrenalectomies displayed AMH. AMH seemed to be a PCC precursor. The symptoms and signs were milder than PCCs. AMHs were mainly found due to screening. Outcomes seemed favorable, but new PCCs developed in many during follow-up.
Objective. To dissect the characteristics of adrenal medullary hyperplasia (AMH) and share our experience of diagnosis and treatment of AMH. Methods. From 1999 to 2013, 12 cases of AMH have been pathologically diagnosed after operation in our hospital. The clinical characteristics, process of diagnosis, treatment, and prognosis during follow-up of all patients are summarized retrospectively. Results. Four cases were trended to be AMH and 6 cases were trended to be pheochromocytoma before operation; moreover, the other two patients were diagnosed accidentally. All patients, except for the patient with mucinous tubular and spindle cell carcinoma of left kidney by open surgery, experienced a smooth laparoscopic adrenalectomy, including 2 with radical nephrectomy, 10 of which experienced unilateral adrenalectomy, 1 was bilaterally partial adrenalectomy, and the remaining one was unilaterally complete removal and then 2/3 partially contralateral excision. After a medium follow-up of 6.5 years, it demonstrated a satisfactory outcome of 8 cured patients and 4 symptomatic improved patients. Conclusions. AMH presents a mimicking morphology and clinical manifestation with pheochromocytoma. Surgery could be the only effective choice for the treatment of AMH and showed a preferable prognosis after a quite long follow-up.
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