1081. [Corticosurrenaloma: an exceptional cause of primary hyperaldosteronism].
作者: Baha Zantour.;Ines Charrada.;Zohra Elati.;Fatma Larbi Ammari.;Fadia Boubaker.;Sondes Arfa.;Olfa Berriche.;Wafa Alaya.;Mohamed Habib Sfar.
来源: Pan Afr Med J. 2018年31卷60页
Corticosurrenaloma is a rare malignant tumor of the adrenal gland that often secretes corticosteroids, sex steroids and precursors. Aldosterone-producing corticosurrenaloma is very rare, accounting for 1 case/10million inhabitants. We report the case of a 38-year old man presenting with severe arterial hypertension associated with deep hypokalaemia (2.2 mmol/L). Exploration showed primary hyperaldosteronism (aldosterone = 2645 pmol/l, aldosterone/renin ratio = 327 pmol/MUI), with hypersecretion of glucocorticoids. Abdominopelvic CT scan revealed left poorly differentiated and heterogeneous adrenal mass measuring 9cm, infiltrating into the surrounding adipose tissue and the diaphragm, extending into the left renal vein, with regional adenopathy and hepatic nodule measuring 4cm. The patient underwent radical nephrectomy followed by right hepatectomy two months after resulting in remission. A year after the patient developed lung metastases. This study highlights that corticosurrenaloma should be suspected in patients with primary aldosteronism despite its rarity.
1084. [Effects of high-dose irradiation on vascularization: Physiopathology and clinical consequences].
A review of the literature has been conducted to better understand the effect of stereotactic radiotherapy on tumour vascularization and their consequences. High irradiation doses cause the death of endothelial cells by apoptosis through the ceramide pathway. Vascular consequences are multiple: increased permeability, decreased blood flow, increased hypoxia and tumoral necrosis. This necrosis causes an indirect death of the cancer cells in the days following the irradiation. The importance of indirect cell death in tumour control remains controversial, but it can explain the good clinical results of stereotactic radiotherapy. Stereotactic radiotherapy is a treatment of the tumour microenvironment. Tumour recurrence in this hypoxic environment is slower through disturbance of revascularization mechanisms but also more aggressive, with increased resistance to treatment and a higher risk of metastasis. In a longer time, vascular damage leads to chronic inflammation and a dysfunctional healing process resulting in vascular occlusion. This phenomenon is used in the treatment of arteriovenous malformations but is also responsible for damage to healthy tissues responsible for chronic complications. This is why high-dose irradiation can only be delivered using stereotactic radiotherapy, which reduces the irradiated volume.
1085. [Giant verrucous nevus in a 15-year old girl: about a case].
Verrucous nevus is a benign tumor showing a linear pattern on Blaschko lines. It is caused by mosaic mutations of the receptor FGFR3 gene. It manifests as an aesthetic disfigurement, but individuals even experience functional complications due to itchiness. Lesions may be localized or extended (giant). In our context, the scarcity of specialized centers in dermatology is a cause of diagnostic delay inducing the patient to undertake unsuitable therapies responsible for infectious or degenerative complications. We report the case of a 15-year old girl with a history of keratotic papules showing a linear pattern along the neck, the right upper limb, the flank since childhood. Histological examination confirmed the diagnosis of verrucous nevus.
1086. [Aggressive natural killer cell leukemia: study of a case occurred in Africa and literature review].
作者: Amine Benmoussa.;Khaoula Khalil.;Fatimazzahra Boufarissi.;Ilias Tazi.
来源: Pan Afr Med J. 2018年31卷28页
Aggressive natural killer cell leukemia (ANKL) is a disease entity within the spectrum of lymphoproliferative syndromes of NK cells. It is rare, preferentially affecting Asiatic people. It has been very rarely reported in the African population; hence the interest of our case. Our study involved a 19-year old female patient of Moroccan origin presenting with acute-onset bone marrow failure syndrome associated with tumor syndrome. The diagnosis of natural killer cell leukemia was retained based on microscopic and immunophenotypic study of the bone marrow. Patient's outcome was fatal; she died within 2 months of diagnosis due to septic shock. Patients with ANKL have a worse prognosis and the median survival time following diagnosis is, on average, two months. In the absence of a prospective study, no therapeutic guidelines have been developed.
1087. [Primary penile cancer: about 11 cases and literature review].
作者: Houyem Mansouri.;Ines Ben Safta.;Med Ali Ayadi.;Selma Gadria.;Tarek Ben Dhiab.;Khaled Rahal.
来源: Pan Afr Med J. 2018年31卷14页
Penile cancer is a rare pathology in Tunisia where circumcision is a common practice. Squamous cell carcinoma accounts for 95% of cases. Treatment is essentially based on surgery. We conducted a retrospective study in order to highlight the epidemiological, clinical, therapeutic features as well as the evolutionary characteristics of 11 cases with primary penile cancer.
1088. [Extra-nodal NK/T-cell lymphoma, nasal type presenting as orbital cellulitis].
Extra-nodal NK/T-cell lymphoma, nasal type, is a rare form of malignant non-Hodgkin's lymphomas. It poses a diagnostic problem due to nonspecific symptoms. We report a case of NK/T-cell lymphoma simulating orbital cellulitis. The study involved a 46-year old female patient followed up for Behçet's disease. The patient was admitted with orbital cellulitis. CT scan of the midface showed pansinusitis with orbital infiltration.The patient had no improvement under antibiotic therapy. Surgical treatment with biopsy was performed. Histological examination showed inflammatory vasculitis and the patient underwent corticosteroid therapy with clinical improvement. The patient had recurrence requiring reoperation with ethmoid biopsies whose histological examination showed NK/T-cell lymphoma, nasal type. Treatment was based on radiochemotherapy and the patient died after two months. NK/T-cell lymphomas are aggressive, they primarily affect the nasal cavities and sinus. They cause angiodestruction and necrosis, resulting in unspecific symptoms. Biopsies are often negative, posing a problem of differential diagnosis. Treatment is based on radiotherapy and chemotherapy. Prognosis is reserved.
1091. [Primitive myxoid melanoma: An unusual histological aspect].
作者: C Capelle.;L Scherman.;A Gerard.;C Vicentini.;M Levavasseur.;O Carpentier.;L Mortier.
来源: Ann Dermatol Venereol. 2019年146卷5期372-376页
Myxoid melanoma is a rare variant of melanoma that must be recognised. Herein we describe a new metastatic case.
1093. [Transudative myelomatous pleurisy revealing pleural localization of primary plasma cell leukemia].
作者: Ouardia Bouayadi.;Ahmed Id M'barek.;Amina Lyagoubi.;Rachid Taghlaoui.;Adnane Aarab.;Hatim Kouismi.;Rachid Seddik.
来源: Ann Biol Clin (Paris). 2019年77卷2期187-190页
Primary plasma cell leukemia (PPCL) is an aggressive and rare variant of multiple myeloma (MM), with frequent extramedullary involvement, mainly liver and splenic lesions. Pleuropulmonary involvement has rarely been described in the literature. We report a case of primary plasma cell leukemia in a 46-year-old patient, whose first symptom was pleural effusion with mediastinal adenopathies simulating a pleural localization of a lymphomatous process. However, blood smear examination, electrophoresis as well as immunofixation of plasma proteins and immuno-histochemistry have helped to guide the diagnosis. Pleurisy is a rare mode of revelation of plasma cell leukemia and is a factor of poor prognosis.
1094. [B-cell non-Hodgkin lymphoma discovery after observation of a platelet satellitism around atypical lymphocytes].
作者: Anne Gatignol.;Marie Amirault.;Éric Legac.;Julien Decker.
来源: Ann Biol Clin (Paris). 2019年77卷2期191-195页
We report the case of an 83-year-old woman admitted to the accident and emergency unit for pneumopathy. The blood cell count on the DXH automaton (Coulter® ™ UniCel® DxH) found a normochromic and normocytic anemia, anormal platelet count and subnormal leukocyte formula. The only alarm raised by the automaton was the presence of a basocytosis. A control of the leucocyte count by flow cytometry and blood smear has been done. It revealed there was no basocytosis, but showed the presence of an atypical monomorphic lymphocytes B population (T/B < 1 ratio in flow cytometry), around which platelets aggregated. The lymphocytic immunophenotyping allowed us to highlight the presence of a CD5+ B clonal subpopulation.
1095. [The value of healthy skin biopsy in the diagnosis of intravascular B-cell lymphoma: A case report and systematic literature review].
作者: E Diaz.;Y Ditchi.;A Roux.;P Senet.;A Barbaud.;C Francès.;J Pacanowski.;F Chasset.
来源: Ann Dermatol Venereol. 2019年146卷4期297-302页
Intravascular large B-cell lymphoma (ivLBCL) is a rare blood dyscrasia that is difficult to diagnose. Healthy skin biopsies may prove useful in diagnosis of the condition. Herein we report a case of ivLBCL diagnosed using this type of examination, and we provide a literature review to determine the sensitivity of such testing.
1096. [Testicular germ cell tumors: Histopathological and molecular features].
In 2016, the WHO classification of testicular germ cell tumors was revised considering advances in the understanding of their tumorigenesis and molecular features. This restructuring led to a division into two major groups with, on one hand, prepubertal-type tumors, not derived from germ cell neoplasia in situ (GCNIS), and on the other hand, postpubertal-type tumors, GCNIS-derived, which occur in youg men (seminoma and non seminomatous germ cell tumors - embryonal carcinoma, yolk sac tumor, teratoma and choriocarcinoma essentially). The term germ cell neoplasia in situ is consensually accepted as a new terminology for the precursor lesion. In this new classification, the term "spermatocytic seminoma" is replaced by "spermatocytic tumor", reclassified among non-GCNIS-derived tumors. The purpose of this change of nomenclature is to reflect the usually non-aggressive behaviour of this tumor and to avoid any confusion with usual seminoma. The spectrum of trophoblastic tumors continues to expand with the description of new rare entities such as the cystic trophoblastic tumor, the placental site trophoblastic tumor and the epithelioid trophoblastic tumor. This review aims to provide a focus on testicular germ cell tumors highlighting the new immunohistochemical and molecular features responsible for the restructuring of classification. The TNM staging is presented according to the AJCC 8th edition 2017 update.
1097. [Treatment of patients with brain metastases from a melanoma].
作者: A Modesto.;C Chira.;J-C Sol.;V Lubrano.;S Boulinguez.;C Pagès.;V Sibaud.;C Gomez-Roca.;É Moyal.;N Meyer.
来源: Cancer Radiother. 2019年23卷2期147-150页
Whereas immune checkpoint inhibitors of serine/threonine protein kinase B-raf therapy dramatically changed metastatic outcomes of patients with melanoma, they remain at high risk of brain extension. Additional local treatment can be offered in this situation such as surgery and or stereotactic radiotherapy. In this review article, we describe the different options with published data and their optimal timing.
1098. [Management of squamous cell carcinoma of the conjunctiva in a young patient: Case report].
Squamous cell carcinoma is the most common malignant tumor of the bulbar conjunctiva. However, it often remains under-diagnosed and exposed to therapeutic delay. The aim of this work is to elucidate the diagnostic and therapeutic difficulties posed by this tumor and to appreciate its prognosis.
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