scholarly journals Recurrent benign adrenal pheochromocytomas associated with hemihypertrophy

Author(s):  
Maria Pikilidou ◽  
Maria Yavropoulou ◽  
Marios Katsounaros

Summary We report a case of a female with hemihypertrophy, who developed five recurrences of pheochromocytomas until the age of 35. Timely follow-up of the patient's blood pressure assisted in early diagnosis and treatment of recurrent tumors. Learning points Recurrent benign pheochromocytomas should raise suspicion of a genetic syndrome. A pheochromocytoma at a young age has a high propensity to recur and strict follow-up is mandatory.

Author(s):  
Kelvin Miu

Laryngeal cancer is a common head and neck cancer and typically presents with voice hoarseness in patients older than 60 years. Early recognition of signs and symptoms of laryngeal cancer can lead to early diagnosis and treatment, therefore improving patient outcomes. This article aims to provide an overview of the anatomy of the larynx, presentation and management of laryngeal cancer, and common follow-up problems.


Dental Update ◽  
2020 ◽  
Vol 47 (1) ◽  
pp. 71-74
Author(s):  
Jawaad Ahmed Asif ◽  
Paras Ahmad ◽  
Tahir Yusuf Noorani

Sialolithiasis is considered as one of the most frequently encountered diseases of the salivary glands. The most susceptible site is the submandibular gland and its duct. However, megaliths have been sparsely reported in the literature. This article portrays management of a sialolith and a megalith in a 26-year-old and a 59-year-old male patient, respectively. The sialolith in the first case case was 4 mm long, whereas the second case demonstrated a megalith measuring 46 mm at its greatest size. Follow-up revealed normal functioning and a painless gland in the first case, while the second case showed no eventful complications. It is interesting to know that both patients remained relatively pain-free, despite having such longstanding sialolith/megaliths. After removal of the small sialolith, the gland regained its normal functioning swiftly, whereas in the case of the megalith, the gland removal was mandatory because such a longstanding megalith led to irreversible functional injury to the gland. CPD/Clinical Relevance: A giant sialolith can be easily misdiagnosed as a submandibular infection or neoplasm, especially when the patient presents with a longstanding pain-free swelling. Hence, early and appropriate referral and investigation is necessary for early diagnosis and treatment.


2019 ◽  
Vol 3 (8) ◽  
pp. 1518-1530 ◽  
Author(s):  
Henrik Falhammar ◽  
Adam Stenman ◽  
Jan Calissendorff ◽  
Carl Christofer Juhlin

Abstract 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.


Author(s):  
N Atapattu ◽  
K A C P Imalke ◽  
M Madarasinghe ◽  
A Lamahewage ◽  
K S H de Silva

Summary Children rarely present with phaeochromocytoma. Their presentation differs from that of adults. The classic triad of sweating, headache and palpitation may not always present in children with phaeochromocytoma. In this study, we present a 6-year-old girl who came to us with polyuria and polydipsia for evaluation of suspected diabetes insipidus. She gave a clear history of increased sweating in the recent past. On clinical examination, she was noted to have high blood pressure. Subsequent investigations revealed a phaeochromocytoma. Her polyuria and hypertension resolved immediately after the surgery. We did not have the facilities to arrange for genetic tests; however, the patient and the family members are under follow-up for other associated conditions. Learning points Polyuria and polydipsia are rare symptoms of phaeochromocytoma. Complete physical examination prevented unnecessary investigations for polyuria and led to a correct diagnosis. Classic features are not always necessary for diagnostic evaluation of rare diseases.


2021 ◽  
Vol 12 ◽  
Author(s):  
Shi Tang ◽  
Min Yang ◽  
Dan Zhang ◽  
Ya-jie Tong ◽  
Ying Xin

AimTo analyze the clinical characteristics of Hashimoto’s thyroiditis (HT) in children below 3 years of age in order to improve the understanding of the disease, avoid misdiagnosis, and achieve early diagnosis and treatment.MethodsThe study retrospectively analyzed the clinical data of 19 patients diagnosed with HT in the first three years of life.ResultsThe patients (12 female, 7 male) had an average age of 26.1 ± 8.2 months (range 10–36 months). At presentation, one patient had euthyroidism, ten had hypothyroidism, seven had subclinical hypothyroidism, and one had hyperthyroidism. The most common reasons for doctor’s visits were thyroid enlargement (21.1%), global developmental delay (21.1%), and routine thyroid function tests in patients with type 1 diabetes (26.3%). Sixteen patients provided follow-up data, and the mean follow-up time was 23.31 ± 16.44 months (range 1–48 months). In the hypothyroidism group, one patient stopped levothyroxine (LT4) treatment after 2 months; the remaining patients had been treated with LT4 since their diagnosis. In the subclinical hypothyroidism group, one patient whose thyroid function returned to normal after 1 month of being diagnosed was not treated. The remaining patients received LT4 treatment at their diagnosis or during follow-up. The patient with hyperthyroidism was treated with methimazole after diagnosis, but treatment was discontinued 11 months later and LT4 was initiated 26 months after diagnosis. One in four patients with global developmental delay approached normal mental development after LT4 treatment. Four in six patients with short stature achieved height catch-up.ConclusionAt their initial HT diagnosis, most of the children showed hypothyroidism or subclinical hypothyroidism. Children with global developmental delay require continual screening, even if the thyroid function is normal after birth, to determine whether they have HT-induced hypothyroidism. Thyroxine replacement could partially relieve the clinical manifestations of hypothyroidism and early diagnosis and treatment are essential for improving patient prognosis.


Author(s):  
Ekaterina Manuylova ◽  
Laura M Calvi ◽  
Catherine Hastings ◽  
G Edward Vates ◽  
Mahlon D Johnson ◽  
...  

Summary Co-secretion of growth hormone (GH) and prolactin (PRL) from a single pituitary adenoma is common. In fact, up to 25% of patients with acromegaly may have PRL co-secretion. The prevalence of acromegaly among patients with a newly diagnosed prolactinoma is unknown. Given the possibility of mixed GH and PRL co-secretion, the current recommendation is to obtain an insulin-like growth factor-1 (IGF-1) in patients with prolactinoma at the initial diagnosis. Long-term follow-up of IGF-1 is not routinely done. Here, we report two cases of well-controlled prolactinoma on dopamine agonists with the development of acromegaly 10–20 years after the initial diagnoses. In both patients, a mixed PRL/GH-cosecreting adenoma was confirmed on the pathology examination after transsphenoidal surgery (TSS). Therefore, periodic routine measurements of IGF-1 should be considered regardless of the duration and biochemical control of prolactinoma. Learning points: Acromegaly can develop in patients with well-controlled prolactinoma on dopamine agonists. The interval between prolactinoma and acromegaly diagnoses can be several decades. Periodic screening of patients with prolactinoma for growth hormone excess should be considered and can 
lead to an early diagnosis of acromegaly before the development of complications.


Author(s):  
Pedro Marques ◽  
Nicola Tufton ◽  
Satya Bhattacharya ◽  
Mark Caulfield ◽  
Scott A Akker

Summary Mineralocorticoid hypertension is most often caused by autonomous overproduction of aldosterone, but excess of other mineralocorticoid precursors can lead to a similar presentation. 11-Deoxycorticosterone (DOC) excess, which can occur in 11-β hydroxylase or 17-α hydroxylase deficiencies, in DOC-producing adrenocortical tumours or in patients taking 11-β hydroxylase inhibitors, may cause mineralocorticoid hypertension. We report a 35-year-old woman who in the third trimester of pregnancy was found to have a large adrenal mass on routine obstetric ultrasound. On referral to our unit, persistent hypertension and long-standing hypokalaemia was noted, despite good compliance with multiple antihypertensives. Ten years earlier, she had hypertension noted in pregnancy which had persisted after delivery. A MRI scan confirmed the presence of a 12 cm adrenal mass and biochemistry revealed high levels of DOC and low/normal renin, aldosterone and dehydroepiandrosterone, with normal catecholamine levels. The patient was treated with antihypertensives until obstetric delivery, following which she underwent an adrenalectomy. Histology confirmed a large adrenal cortical neoplasm of uncertain malignant potential. Postoperatively, blood pressure and serum potassium normalised, and the antihypertensive medication was stopped. Over 10 years of follow-up, she remains asymptomatic with normal DOC measurements. This case should alert clinicians to the possibility of a diagnosis of a DOC-producing adrenal tumours in patients with adrenal nodules and apparent mineralocorticoid hypertension in the presence of low or normal levels of aldosterone. The associated diagnostic and management challenges are discussed. Learning points: Hypermineralocorticoidism is characterised by hypertension, volume expansion and hypokalaemic alkalosis and is most commonly due to overproduction of aldosterone. However, excess of other mineralocorticoid products, such as DOC, lead to the same syndrome but with normal or low aldosterone levels. The differential diagnosis of resistant hypertension with low renin and low/normal aldosterone includes congenital adrenal hyperplasia, syndrome of apparent mineralocorticoid excess, Cushing’s syndrome, Liddle’s syndrome and 11-deoxycorticosterone-producing tumours. DOC is one intermediate product in the mineralocorticoid synthesis with weaker activity than aldosterone. However, marked DOC excess seen in 11-β hydroxylase or 17-α hydroxylase deficiencies in DOC-producing adrenocortical tumours or in patients taking 11-β hydroxylase inhibitors, may cause mineralocorticoid hypertension. Excessive production of DOC in adrenocortical tumours has been attributed to reduced activity of the enzymes 11-β hydroxylase and 17-α hydroxylase and increased activity of 21-α hydroxylase. The diagnosis of DOC-producing adrenal tumours is challenging because of its rarity and poor availability of DOC laboratory assays.


2016 ◽  
Vol 31 (1) ◽  
pp. 53-56 ◽  
Author(s):  
Justin Iohanne Siy Rabo ◽  
Allan B. Carpela ◽  
Eutrapio S. Guevara ◽  
Joel A. Romualdez

Objective:  To present a case of mandibular ameloblastoma with pulmonary metastasis after ten years and discuss the possible pathophysiology, diagnostic and therapeutic options. Methods: Study Design: Case Report Setting:           Tertiary Private Hospital Subject:          One Conclusion: Though benign, ameloblastoma has a high propensity for local invasion and may metastasize. It is difficult to predict metastasis, even with adequate treatment of the primary lesion. There is no standard protocol to prevent or detect metastatic ameloblastoma, but regular and close follow up may ensure early diagnosis. Keywords: ameloblastoma, metastatic ameloblastoma, lung metastasis, follicular type ameloblastoma, odontogenic tumor


PEDIATRICS ◽  
1950 ◽  
Vol 6 (2) ◽  
pp. 286-298
Author(s):  
G. E. MCCOY ◽  
M. L. BRIDGEMAN

A case of a pheochromocytoma in a 13 year old white male is presented. The preoperative and postoperative effects of benzodioxain, dibenamine®, sedation, cold and posture on blood pressure in such a patient are shown. An eight month follow-up study is reported. The fact is re-emphasized that hypertension caused by a solitary pheochromocytoma may persist for many weeks following its removal. It may also return to normal immediately and stay there.


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