Adenoid cystic carcinoma is an uncommon malignant neoplasm of the salivary gland. Liver metastasis from salivary gland cancer is a rare situation. In this article, we report the case of a 29-year-old woman treated 5 years previously for adenoid cystic carcinoma of the parotid gland by surgery and radiotherapy, who presented for a large hypervascularized hepatic metastasis of 20 cm. After 3-cycle chemotherapy stability, hepatic surgery was successfully performed. The patient maintained disease-free period of 12 months after the surgical treatment. This rare case represents a therapeutic challenge for oncologists and surgeons. Through this case and a review of the literature, we try to better detail the management of this uncommon entity.
Introduction
Renal angiomyolipoma is considered a benign mesenchymal tumor composed of fat, smooth muscle, and blood vessels. It represents 1–3% of solid renal tumors. Despite this tumor’s benignity, it can be aggressive with a locoregional extension.
Case report
A 41-year-old north African caucasian woman consulted with chief complaints of right lower back pain with no hematuria and no urinal sign. Thoracic-abdominopelvic contrast-enhanced computed tomography showed a right inferior polar heterogeneous renal mass complicated with venous thrombus ascending to the right atrium level. The patient underwent radical nephrectomy under extracorporeal circulation and direct supervision of the fatty thrombus at the right atrium level. The postoperative period was uneventful. The final histologic examination was concordant with renal angiomyolipoma.
Conclusion
Renal angiomyolipoma is the most common benign kidney tumor. Despite its benignity, it can be associated with lethal complications such as hemorrhage, and it can also show signs of local extension mimicking malignant tumors. The cornerstone of the treatment remains surgery.
BackgroundInborn errors of immunity (IEI) predispose patients to various infectious and non-infectious complications. Thanks to the development and expanding use of flow cytometry and increased awareness, the diagnostic rate of IEI has markedly increased in Algeria the last decade.AimThis study aimed to describe a large cohort of Algerian patients with probable IEI and to determine their clinical characteristics and outcomes.MethodsWe collected and analyzed retrospectively the demographic data, clinical manifestations, immunologic, genetic data, and outcome of Algerian IEI patients - diagnosed in the department of medical immunology of Beni Messous university hospital center, Algiers, from 2008 to 2021.ResultsEight hundred and seven patients with IEI (482 males and 325 females) were enrolled, 9.7% of whom were adults. Consanguinity was reported in 50.3% of the cases and a positive family history in 32.34%. The medium age at disease onset was 8 months and at diagnosis was 36 months. The median delay in diagnosis was 16 months. Combined immunodeficiencies were the most frequent (33.8%), followed by antibody deficiencies (24.5%) and well-defined syndromes with immunodeficiency (24%). Among 287 patients tested for genetic disorders, 129 patients carried pathogenic mutations; 102 having biallelic variants mostly in a homozygous state (autosomal recessive disorders). The highest mortality rate was observed in patients with combined immunodeficiency (70.1%), especially in patients with severe combined immunodeficiency (SCID), Omenn syndrome, or Major Histocompatibility Complex (MHC) class II deficiency.ConclusionThe spectrum of IEI in Algeria is similar to that seen in most countries of the Middle East and North Africa (MENA) region, notably regarding the frequency of autosomal recessive and/or combined immunodeficiencies.
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