A Rare Case of Congenital Nephrogenic Diabetes Insipidus Associated with Aquaporin 2 Gene Mutation and Subsequent Acute Lymphoblastic Leukemia: Impact of Steroids on Kidney Function.

BACKGROUND Nephrogenic diabetes insipidus (NDI) is a rare renal disorder that can be congenital, and is caused by mutations in either aquaporin 2 or arginine vasopressin receptor 2, or it can be secondary to kidney disease or electrolyte imbalance. The clinical signs of NDI include polyuria, compensatory polydipsia, hypernatremic dehydration, and growth retardation without prompt treatment. In this report, we present the case of a patient with congenital NDI who was later diagnosed with acute lymphoblastic leukemia (ALL). With dexamethasone treatment, he had uncontrolled polyuria and polydipsia. Our aim was to concentrate on the impact of steroids on the kidneys. CASE REPORT Our patient presented at the age of 9 months with signs of severe dehydration that were associated with polyuria. His laboratory examinations revealed hypernatremia and decreased urine osmolality. He was diagnosed with NDI and his exome sequence revealed a homozygous mutation at the nucleotide position AQP2 NM_000486.6: c.374C>T (p.Thr125Met). He was treated with hydrochlorothiazide and amiloride. Then, at age 19 months, he presented with gastroenteritis and a complete blood count (CBC) showed high white blood cell count and blast cells. He was diagnosed with (ALL) and began receiving chemotherapy, during which again developed polydipsia and polyuria, which could not be controlled with an increased dosage of hydrochlorothiazide. CONCLUSIONS We report a rare case of NDI caused by a missense mutation in the aquaporin 2 gene. One year later, the child developed ALL, and treatment with dexamethasone led to an uncompensated state of polydipsia and polyuria.

Mature cystic teratoma of the ovary with malignant transformation of tall cell subtype of papillary thyroid carcinoma.

Malignancies rarely occur in somatic parts of mature cystic teratoma of the ovary. Squamous cell carcinoma is the most common form of cancer that can develop in mature cystic teratoma. Other less frequent malignancies include melanoma, sarcoma, carcinoid, and germ cell neoplasms. Only three cases have been reported as papillary thyroid carcinoma arising in struma ovarii. We present a unique case of a 31-year-old female patient who presented with a left ovarian cyst and underwent conservative surgical management in the form of cystectomy. Histopathological examination confirmed the diagnosis of a tall cell subtype of papillary thyroid carcinoma arising from a small focus of thyroid tissue in a mature cystic teratoma of the ovary. The patient was followed up for 60 months with an uneventful clinical course. For a better understanding of such rare cancers, collaborative retrospective studies on large databases with other medical centers are required.

Impact of COVID-19 Exclusive Allocation Strategy on Quality of Healthcare: A Study From Jordan, 2020.

The COVID-19 pandemic has brought uncertainty to everyday medical practice. Deciding how to ration limited healthcare resources is difficult and requires the involvement of higher authorities in each country. In this article we focus on the Jordanian strategy of allocating tertiary healthcare centers exclusively for COVID-19 patients and postponing all other treatments and healthcare provision. We collected secondary data on admissions, occupancy of hospital beds, and length of stay at emergency departments and outpatient clinics, as well as surgeries conducted, between March and May 2020 at King Abdullah University Hospital in Irbid, Jordan. We also conducted a literature review to explore health resource utilization and allocation in terms of health service quality. Our findings showed a major decrease in the demand for health services at the hospital including admissions, emergency department visits, outpatient clinic visits, surgeries, and radiology during the study period. These findings indicate the enormous impact of the pandemic on the largest segment of patients in Jordan-those who depend on government health insurance-to manage their routine healthcare needs, which may affect the health status of patients. Authorities should address the COVID-19 pandemic holistically by prioritizing both COVID-19 cases and non-COVID-19 cases and should draft a framework for managing future pandemics. Moreover, planning a strategy to accommodate the number of people waiting for elective surgeries and routine healthcare should be in place to minimize the burden of this pandemic.

Soft Tissue Sarcomas: A 16-Year Experience of a Tertiary Referral Hospital in North Jordan.

Background and Objectives: Sarcomas are rare malignant tumors of mesenchymal origin. Their low prevalence and histological heterogeneity make their diagnosis a challenging task. To the best of our knowledge, the epidemiology of soft tissue sarcomas (STSs) was not well studied in Jordan. This study thus aimed to determine STS epidemiologic trends at King Abdullah University Hospital (KAUH); a tertiary hospital that provides cancer healthcare for 70% of the population in Irbid Governorate, North Jordan. The findings of this study will provide a good reference point of the burden of STSs in Jordan and the Middle East region. Materials and Methods: All cases with confirmed STS diagnoses who attended KAUH from January 2003 until December 2018 were included in the initial analysis. Bone sarcomas, gastrointestinal stromal tumors and uterine sarcomas were not included in the study. Information collected from the pathology reports and electronic medical records was used to determine STS prevalence, incidence rate, age and gender distributions, histological types and anatomic location. Cases were reviewed by three pathologists with interest in soft tissue tumors. The findings were compared with literature. Results: In total, 157 STS cases were reported (1.9% of cancers diagnosed at KAUH during the 16-year study period). Crude annual incidence rate (IR) per 100,000 person-years ranged from 0.48 in 2015 to 1.83 in 2011 (average = 1.04). Age-standardized IR (ASR)(World WHO 2000-2025) was 1.37. Male:female ratio was 1.3:1. Median age was 39 years. Age ranged from <1 year to 90 years. Overall STS rates increased with age. The most common histological types were liposarcoma (19%), rhabdomyosarcoma (17%) and leiomyosarcoma (10%). The most common anatomic location was the extremity (40.1%), followed by the trunk (14.7%), then head and neck (10.8%). Conclusion: STSs are rare in North Jordan. A slight increase in their incidence was identified during the study period similar to global trends. The collection of relevant data on established risk factors along with a broader scale evaluation of the epidemiology of STS in the Middle East region is recommended to better evaluate disease burden and trends.

Primary Clear Cell Sarcoma of the Lung: a Case Report.

BACKGROUND: Clear Cell Sarcoma of Soft Tissue (CCSST), or melanoma of the soft part, is a rare, aggressive tumor that originates in the aponeurosis and fasciae of the distal parts of the extremities. Reports from other sites of the body are rare. OBJECTIVE: We are reporting an extremely rare tumor that presented as a central left-sided lung mass and found to be clear cell sarcoma of soft tissue. METHODS: We report a 24-year-old male patient presented with recurrent attacks of left-sided chest pain associated with cough and dyspnea. RESULTS: Imaging showed a central left-sided 8*5.5*5 cm lung mass. The age of the patient and the radiological characteristics of the lesion were suggestive of a benign pathology. After histopathological assessment of the lesion, suspicion of the malignant process was raised, mainly melanoma of soft part and PEComa. The patient underwent left-sided pneumonectomy. The postoperative histological examination, immunohistochemical findings including positive staining for S-100, HMB-45, and Melan-A, and positive FISH study for EWSR1 gene rearrangements supported the diagnosis of CCSST originating primarily in the major fissure of left the lung. CONCLUSION: The rarity of CCSST in general and tumors originating in the lung primarily raise the challenges in hypothesizing a differential diagnosis, choosing proper investigations and treatment methods. The histological examination, immunohistochemical, and cytogenetics of the tumor are mandatory to reach the final diagnosis.

Implications of a background of Hashimoto's thyroiditis on the current conservative surgical trend towards papillary thyroid carcinoma.

The purpose is to delineate the clinico-pathologic features of papillary thyroid carcinoma (PTC) occurring in a background of Hashimoto's thyroiditis (HT). A retrospective analysis of consecutive PTC patients who underwent surgery at an academic center between Jan. 2010 and Jan. 2020 was performed. The primary end point was to assess whether a background of HT implied a higher likelihood for postoperatively determined high-risk histopathologic features. Accordingly, its implication on initial surgical planning. Tumor high-risk features included aggressive histologic variants, lymphovascular invasion, extrathyroidal extension, multifocality, and positive nodal metastasis. To achieve the primary outcome of interest, a two group-designation was followed: group A (none-HT-group) and group B (HT-group). The two groups were also compared regarding postoperative complications as a secondary outcome of interest. In the specified period, 331 patients were found to have a histologically proven diagnosis of PTC. Group A accounted for 80% (265/331) of the study cohort, while group B accounted for the remainder 20%. PTC was significantly more prevalent in the absence of HT (p < 0.0001). Both groups were comparable in mean-patient-age. However, Group B demonstrated a considerably higher male:female ratio (1:10 vs. 1:3; p = 0.01). All postoperatively determined high-risk histopathologic features were comparable in both groups (p > 0.05). Nevertheless, transient hypoparathyroidism was considerably higher in group B (12% vs. 23%; p = 0.02). A background of HT does not seem to reflect a more aggressive cancerous biologic behavior. Therefore, it should not preclude the conservative surgical strategy adopted by the most recent clinical practice guidelines.