Correlation between the size of pheochromocytoma and the level of metanephrines.

Objective. Pheochromocytomas (PHEO) and paraganglioma (PGLs) are rare neuroendocrine catecholamine-producing tumors that arise from the chromaffin cells of either the adrenal medulla or extra-adrenal paraganglionic tissues. Despite the recent advances in imaging technologies, biochemical evidence of excessive catecholamine production by the tumor is considered the most important test for the diagnosis of these tumors. The aim of the present study is to investigate the role of the catecholamine metabolites (normetanephrine and metanephrine) levels in the diagnosis of PHEO/PGLs and to evaluate if their levels correlate with the size of these tumors. Patients and Methods. Twenty-five patients were included in the study during the time period of 10 years. Their data were compared with another set of 25 patients to obtain the sensitivity and specificity of metanephrine and normetanephrine in the diagnosis of PHEO/PGLs. The tumor size was reviewed in every patient to obtain the correlation coefficient between the tumor sizes and the plasma/24-hour urinary metanephrine levels. Results. The sensitivity and specificity rates for plasma metanephrine were 80-92% and 92-96%, respectively; while for 24-hour urinary metanephrine were 80-90% and 95-100%, respectively. We found a strong positive relationship between the tumor size and the plasma levels of normetanephrine (r=0.518, p<0.01), and metanephrine (r=0.577, p<0.01). While the relation with the 24-hour urinary concentrations of normetanephrine (r=0.384, p=0.01) and 24-h urinary meta-nephrine (r=0.138, p<0.01) was low. Conclusion. The determination of plasma and 24-hour urinary levels of metanephrines is a reliable test for the diagnosis of PHEO, as they are continuously produced by the tumor cells in contrast to catecholamines.

Breast lymphoma: General review.

BACKGROUND: Lymphoma of the breast can be classified as either primary breast lymphoma (PBL) or secondary to systemic lymphoma (SBL). PBL is a rare disease with Diffuse Large B cell Lymphomas (DLBCL) being the most common subtype. OBJECTIVES: In the current study, we represented eleven cases diagnosed with breast lymphoma in our trust; two of them had PBL and nine had SBL. We focused mainly on the clinical presentation, diagnosis, management and outcomes. METHODS: We did this retrospective review for all breast lymphoma patients who were diagnosed in our trust during the periods from 2011-2022. Patients' data were obtained from the hospital recording system. We followed up these patients thus far to identify the outcome of treatment in each patient. RESULTS: Eleven patients were included in our review. All patients were females. Average age of diagnosis was 66.1 ± 13 years of age. Eight patients were diagnosed with DLBCL, two patients were diagnosed with follicular lymphomas, and the last one had lymphoplasmacytic lymphoma. Chemotherapy +∕- radiotherapy was the standard treatment regimen in all patients. Four patients passed away within one year of chemotherapy, five patients achieved complete remission, one patient had two relapses and is still under treatment, while the last patient was diagnosed recently and still awaiting treatment. CONCLUSION: Primary breast lymphoma is an aggressive disease. The treatment for PBL is mainly systemic with chemoradiotherapy. The role of surgery is now limited to the diagnosis of the disease. Early diagnosis and proper treatment are crucial for the management of such cases.

Pheochromocytoma associated with a succinate dehydrogenase subunit B mutation: A minireview and a case report.

Objective. Pheochromocytomas and paragangliomas are rare neuroendocrine tumors that arise from the chromaffin cells of the adrenal medulla or extra-adrenal tissues. These tumors are characterized by an excessive secretion of catecholamines, which are responsible for the clinical manifestation of the disease. Although most of these tumors are sporadic, underlying genetic abnormalities may be present in up to 24% of the cases. A succinate dehydrogenase subunit B (SDHB) mutation represents one of the rare presentations of the disease. In this study, we represent a rare case of pheochromocytoma associated with SDHB mutation. Methods. We performed a retrospective review of our case in addition to reviewing the available literature on the same topic. Results. A 17-year-old patient presented with sustained hypertension. Clinical, laboratory, and radiological evaluations confirmed the diagnosis of catecholamine-secreting tumor. Laparoscopic adrenalectomy was performed. Histopathological and genetic testing confirmed a pheochromocytoma associated with SDHB mutation. No recurrence was detected on two-years of follow up. Conclusion. Pheochromocytoma associated with SDHB mutation is a rare presentation. Genetic testing for suspecting cases is essential to help to establish the appropriate follow-up plan.

Correlation between preoperative calcium and parathormone levels with parathyroid gland volume.

Objective. Hyperparathyroidism is a prevalent disease with parathyroid adenomas being the most common cause. Surgical excision remains the standard treatment for parathyroid adenoma. Successful preoperative localization of the parathyroid adenoma could facilitate the decision regarding the extent of surgical exploration. The aim of the current study was to assess the correlation between the preoperative values of parathyroid hormone and ionized calcium with the adenoma weight and volume in patient with primary hyperparathyroidism caused by single-gland adenoma. Patients and Methods. We did this retrospective review for all patients who were diagnosed with primary hyperparathyroidism due to a solitary parathyroid adenoma in our general surgery department over 4 years. SPSS software was used to get the correlation coefficient between the peak preoperative levels of calcium and parathyroid hormone with the parathyroid adenoma weight and volume. Results. Ninety-nine patients were included into the study. The average age at surgery was 62.65±12.00 years. The correlation coefficient between the adenoma volume and weight with the preoperative ionized calcium level was weakly positive (r=0.329, p<0.01) and (r=0.281, p=0.019), respectively, while the correlation with the preoperative parathyroid hormone level was stronger (r=0.708, p<0.01) and (r=0.650, p<0.01), respectively. Conclusions. The strong positive relationship between the preoperative parathyroid hormone and calcium levels with the parathyroid adenoma size and weight can help the surgeon to predict the volume of the involved gland and avoid an unnecessary dissection.

Thyroid Lymphoma: Case Series Review.

BACKGROUND: Primary thyroid lymphoma (PTL) is a rare malignancy accounting for about 5% of all thyroid malignancies and less than 2% of extra-nodal lymphomas. Diffuse B-cell lymphoma is the most common pathological subtype of PTL. PATIENTS AND METHODS: This is a retrospective review of all patients diagnosed with thyroid lymphoma in our hospital during the period from 2011 to 2021. All patients had clinical, radiological, and pathological evaluation. We have followed up these patients since diagnosis till now. RESULTS: Eight patients were included in our review. Four patients were males and four were females. Mean age at diagnosis was 63 ± 32 years of age. Diagnosis was confirmed only with tissue histology. Six patients had stage IIE disease and two had stage IVE disease. Four patients had disease related mortality. Of the other four patients, two had chemotherapy (RCHOP) and one had local radiotherapy; all achieved complete remission, and the last one had hemithyroidectomy to relieve airway compression and still under treatment. CONCLUSION: Primary thyroid lymphoma is rare malignancy of the thyroid gland. Advanced stage and age at the time of diagnosis, the presence of compression manifestations, large tumor mass, and presence of distant metastasis, all are poor prognostic criteria. Our knowledge of the disease pathology, diagnosis, and management is still limited and more research studies are required.

Diagnostic Accuracy of Ultrasonography in Axillary Staging in Breast Cancer Patients.

Background: Breast cancer is the most common malignancy affecting women all over the world and represents 7% of all cancer-related deaths in the UK. One of the most crucial elements in assessing a patient's prognosis and chance of survival with breast cancer is the condition of their axillary lymph nodes. Ultrasonography (US) is now used as a routine preoperative diagnostic tool for pretherapeutic axillary evaluation. The aim of the current study is to investigate the diagnostic accuracy of US in axillary staging in breast cancer patients. Methods: We carried out this retrospective study for all invasive breast cancer patients who had surgery in addition to preoperative axillary staging using US during the period from January 2020 to February 2021. The final histology results were compared with the preoperative US findings to ascertain the sensitivity, specificity, positive predictive value, and negative predictive value of AUS in axillary staging. Results: One hundred and twenty-eight patients were included in our study. The average age of diagnosis was 63.9 ± 12.3 years of age. We calculated sensitivity rate of 59.6%, specificity rate of 95.1%, positive predictive value of 87.5%, and negative predictive value of 80.2% with overall diagnostic accuracy of 82.2%. Conclusion: Despite the important role of preoperative US in axillary staging in breast cancer patients; it failed to detect metastatic diseases in 14.8% of our patients. These findings necessitate the routine histological evaluation of the axilla for more accurate staging of the disease.

Risk of malignancy in Thy3 thyroid nodules.

Objective. Thyroid cancer is the most common endocrine malignancy in humans. Ultrasound guided fine needle aspiration cytology (FNAC) is now considered the best diagnostic tool for the evaluation of any thyroid nodule. Thyroid cytology is graded from Thy1 to Thy5 with Thy3 being the most challenging in diagnosis. Our aim was to identify the risk of malignancy in Thy3 cytology in our centre. This risk should be explained to the patient before taking any decision. Methods. One hundred and one patients were included in our study. All patients had Thy3 cytology on preoperative ultrasound scan guided FNAC. All patients had diagnostic hemithyroidectomy. The results from the histology were compared with the cytology findings and the rates of malignancy were identified. Results. Of the 101 patients, 17 were males and 84 females. Average age for diagnosis was 52.4±15 years of age. Patients were classified into three groups; patient who had completely benign histology (n=70), patients who had incidental finding of micro-carcinoma after diagnostic hemithyroidectomy (n=10), and patients who had thyroid macro-carcinomas (n=21). Total rate of malignancy was 30.7% when combining both the malignant and the incidental groups and 20.8% when excluding the incidental group. Conclusion. Our rates of malignancy in Thy3 cytology are similar to the literature. These rates should be explained clearly to the patient during the preoperative counselling. Future advances in biomarkers technology may help to improve the preoperative diagnostic accuracy and reduce the rate of unnecessary thyroid surgery.

A Rare Case of Cavernous Haemangioma of the Adrenal Gland: A Case Report and Review of Literature.

Cavernous haemangiomas, also known as cavernoma or cavernous angiomas, are clusters of vasculature malformations arising from the endothelial layer of blood vessels. They are commonly found in the central nervous systems, skin, or liver. Rarely, they can also affect adrenal glands, a phenomenon with only 66 cases since the first case was reported in the literature in 1955 and 2018. Adrenal cavernous haemangiomas are typically non-functioning and found incidentally on radiological imaging. Here, we present the case of a 79-year-old male who was referred by a district general hospital to our tertiary centre with an incidentaloma of the left adrenal gland which was first noted in 2014 measuring 6 cm. A repeat computed tomography in 2020 revealed the mass was 20.8 cm. In 2020, the growing mass was causing anaemia and abdominal discomfort due to displacement of the surrounding viscera. The initial radiological impression performed in another hospital of the indeterminate mass was highly suspicious of primary adrenal malignancy. The decision was made to operate prior to biopsy as biopsy was deemed high risk for dissemination of primary malignancy of the adrenal. The patient subsequently underwent a radical open left adrenalectomy in September 2020. The diagnosis of adrenal cavernous haemangioma was made on histopathological examination post-surgery. Published literature on this rare adrenal tumour between 2019 and 2021 is also reviewed in this paper.

Adrenal Lymphoma: Case Reports and Mini-review.

Introduction: Adrenal lymphoma is a rare condition which may occur in one of two forms; either as primary adrenal lymphoma (PAL), or secondary to a systemic lymphoma. Primary adrenal lymphoma is a very rare diagnosis and the most common histological pattern is diffuse large B-cell non­Hodgkin lymphoma. Objectives: In this study, we represent two examples of adrenal lymphoma, primary and secondary. In addition, we have included a mini-review of the literature regarding this rare presentation. Patients and Methods: We retrospectively reviewed all patients who were diagnosed with adrenal lymphoma in our hospital. We represent mainly the most two challenging cases where adrenal surgery was required to confirm the diagnosis. We have included a mini-review of the literature (PubMed data base: 1990 - 2020) on the clinical presentation and management of adrenal lymphoma cases. Results: Seventeen patients had adrenal lymphoma in our hospital; 16 of them had secondary involvement of the adrenal gland, while the last one had primary adrenal lymphoma. Patients with adrenal lymphoma mainly present with fever, lumbar pain, and/or symptoms of adrenal insufficiency. Primary adrenal lymphoma usually appears as heterogeneous complex large masses with low density on computerized tomography (CT) scan or magnetic resonance imaging (MRI); however, there is no pathognomonic features to diagnose PAL. The diagnosis is confirmed only with tissue biopsy. Chemotherapy is generally the standard treatment for lymphoma, while the role of surgery is limited. Conclusions: The prognosis of these rare cases is generally poor with only about a third of patients achieving partial or complete remission following treatment.

Lesson of the month 2: Catecholamine-induced cardiomyopathy - pitfalls in diagnosis and medical management.

Cardiomyopathy as the initial presentation of phaeochromocytoma (PCA) is uncommon. Diagnostic work-up and perioperative management may be challenging within this context. We report three cases of PCA presenting with cardiomyopathy to illustrate the pitfalls in diagnosis and management. None of the patients had typical adrenergic symptoms and all three were established on beta-blockers prior to diagnosis. Their fractionated plasma catecholamine levels were elevated and the diagnosis of PCA was confirmed with various imaging modalities and post adrenalectomy. Interpretation of fractionated catecholamine levels in the context of established cardiomyopathy is difficult as cardiac failure of any aetiology generates an adrenergic response. Hence screening all patients with idiopathic cardiomyopathy is likely to generate a high false-positive rate. However, a high index of suspicion should prompt further diagnostic work-up in patients with idiopathic cardiomyopathy for occult PCAs. Peer-reviewed guidelines are required to guide the investigation and management of suspected catecholamine-induced cardiomyopathy.

Neonatal renal vein thrombosis: grey-scale and Doppler ultrasonic features.

Renal vein thrombosis RVT is the most common non-catheter related venous thromboembolic events VTE in newborns and is responsible for approximately 10% of all VTE in newborns. Almost 80% of all RVT present within the first month and usually within the first week of life. Currently ultrasound is the radiographic test of choice because of its practicality, sensitivity and lack of adverse effects. The sonographic features vary according to the severity, the extent of the thrombus, the development of collateral circulation and the stage of renal vein thrombosis. Initial diagnostic features include, renal enlargement, echogenic medullary streaks that have a vascular or perivascular distribution, thrombus in the vein prominent echopoor medullary pyramids, subsequently loss of corticomedullary differentiation, reduced echogenicity around the affected pyramids and echogenic band at the extreme apex of the pyramid. Higher resistance index and absent, steady, or less pulsatile venous flow on the affected side compared with flow in the contralateral kidney are helpful Doppler signs. While sonography may be useful in revealing the venous and renal morphology, it is insufficiently reliable for assessment of the functional impact of these morphologic findings. There are no characteristic grey-scale or Doppler ultrasound prognostic features to predict outcome of neonatal RVT and long-term follow up of those children is required.