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Intissar Haddiya,1 Hakim Hamzaoui,1 Zitouna Alhamany,2 Fatime-zohra Berkchi,1 Hakima Rhou,1 Loubna Benamar,1 Naima Ouzeddoun,1 Rabea Bayahia1 1Department of Nephrology-Dialysis-Renal Transplantation, Ibn Sina University Hospital, Rabat, Morocco; 2Department of Pathology, Rabat Children's Hospital, Ibn Sina University Hospital, Rabat, Morocco Background: Spontaneous rupture of the kidney is uncommon and is mainly caused by renal tumors. Only a few cases are caused by vasculitis. We report here the first case of spontaneous rupture of kidney resulting from mixed cryoglobulinemia. Case presentation: A 44-year-old man presented with sudden onset of fever, acute pulmonary edema, left flank abdominal pain unassociated with trauma, and rapidly progressive renal failure requiring dialysis. Computed tomography of the abdomen revealed a large perirenal hematoma of the left kidney. During conservative surgery, the patient underwent renal biopsy that showed renal vasculitis and membranoproliferative glomerulonephritis with intracapillary microthrombi. Tests were positive for mixed cryoglobulinemia caused by Sjögren's syndrome. The patient was better after immunosuppressive therapy, with the disappearance of clinical symptoms and the recovery of baseline renal function. Conclusion: We report on this case and discuss a possible link between spontaneous rupture of kidney and mixed cryoglobulinemia-associated Sjögren's syndrome. Keywords: mixed cryoglobulinemia, Sjögren's syndrome, spontaneous rupture of kidney, renal hematoma, Wünderlich syndrome

Background: Angiosarcoma of the breast is a rare tumor, which may be primary or secondary to breast surgery or irradiation. It is characterized by polymorphic and nonspecific clinical and radiological features. A pathologist plays a key role in positive and differential diagnosis and in establishing the prognosis: only a histological examination can confirm the diagnosis, and the histologic grade is the most important prognostic factor. In fact, angiosarcomas of the breast constitute a very heterogeneous group and they are classified into three grades based on the degree of differentiation. We will illustrate diagnostic challenges through this new case of primary angiosarcoma of the breast. Microscopic findings were initially interpreted as a benign vascular tumor. We will also discuss the relevant medical literature., Case Presentation: A 56-year-old Arabian woman presented with a palpable right breast mass that had been enlarging for 2 months, measuring 5 cm, without axillary lymphadenopathy. She had no personal or family history of breast surgery or breast irradiation. A mammography showed no evidence of spiculation. No suspicious calcifications were seen. A needle core biopsy was performed. Microscopic findings were initially interpreted as a benign vascular tumor. However, as the mass measured 5 cm, the diagnosis of angiosarcoma was more appropriate, and mastectomy without axillary dissection was performed. Microscopic examination found mild to moderately scattered pleomorphic cells, and scattered mitotic figures. It also showed papillary formations, solid foci of spindle cells, and hemorrhagic necrosis. The margins of the tumor were infiltrative. The diagnosis of primary intermediately differentiated angiosarcoma of the breast (grade II) was made. No distant metastases were found. Our patient was lost to follow-up and further treatment after mastectomy until she developed local tumor progression 4 months later., Conclusions: Through this case report, we emphasize the importance of clinicopathological confrontation in angiosarcoma of the breast.

Background: Peripheral primitive neuroectodermal tumors are extremely rare tumors in the spine; only 18 cases of extra-dural peripheral primitive neuroectodermal tumor cervical region have been reported. The aim of this report is to highlight the challenges in diagnosis and management of this condition., Case Presentation: We present a case of 5-year-old Moroccan boy, who presented with torticollis for 1 month. Computed tomography scan and Magnetic resonance imaging of the cervical spine revealed an extradural, dumbbell-shaped mass with extra-spinal extension at the left C1-C6 level. Multiple biopsy specimens were obtained. Histological examination revealed a highly cellular neoplasm composed of diffuse sheets of tumor cells having monomorphic, round to oval, finely vesicular nuclei. Immunohistochemical findings confirmed the diagnosis of intraspinal peripheral primitive neuroectodermal tumor., Conclusion: After this illustrative case, we review the literature on clinicopathological and therapeutic aspects. In practice, it is important to consider the diagnosis of peripheral primitive neuroectodermal tumor in children and adolescents with an apparent soft-tissue mass located in the spine.

Objective: Study of histological and clinical correlations of 30 cases of renal amyloidosis AA diagnosed between November 2010 and December 2012., Results: The main causes associated with amyloidosis AA were represented by chronic infectious diseases (60%). Nephrotic syndrome and renal failure were observed in 94% and 73% respectively. The distribution of amyloid deposits: 90% of patients had a glomerular form and 10% had a vascular form. Inflammatory reaction associated with AA renal amyloidosis was present in 50% of cases. This inflammation was observed near amyloid deposits associated with a deposition of immunoglobulin chains and/or complement factors., Conclusion: Our study confirms the predominance of AA amyloidosis complicating chronic infectious diseases, especially tuberculosis. Our data point out a relationship between the morphology of renal AA amyloidosis, its clinical presentation and prognosis., (Copyright © 2015 Association Société de néphrologie. Published by Elsevier SAS. All rights reserved.)

Kaposi's sarcoma (KS) is widely reported to develop after renal transplantation and is induced by activation of a latent human herpes virus 8. We report the clinical features and outcome of a 50-year-old woman who presented with KS 18 weeks after starting immunosuppressive therapy for vasculitis. She had positive-titer IgG antibody to human herpes virus 8. Cyclophosphamide pulses were interrupted, and prednisone was decreased gradually to 10 mg/day. Skin lesions showed important regression with stabilization of the general state and renal function. Eight months later, the patient presented with a diffuse cutaneous KS that required the discontinuation of steroids. Within 1 month, her general status and renal function deteriorated, and she died with a disseminated intravascular coagulation syndrome.

Sclerodermic renal crisis is defined as a severe arterial hypertension or rapidly progressive glomerulonephritis in a sclerodermic patient, associated with anuria due to no other cause but systemic sclerodermia. It constitutes a rare and dangerous complication. The renal prognosis can be effectively improved by converting enzyme inhibitors, allowing a better arterial hypertension control. However, the associated mortality remains high, a follow up must be instaured in order to prevent the apparition of renal lesions in all sclerodermic patients. We report a case of rapidly progressive renal failure with hypertension in a patient with no previous problems, which preceded the development of scleroderma diffuse cutaneous form. The biopsy showed a microangiopathy in the context of scleroderma renal crisis. The evolution was marked by the presence of chronic renal insufficiency which necessitated the dialysing start., (Copyright © 2010 Association Société de néphrologie. Published by Elsevier SAS. All rights reserved.)

We present two cases of renal lymphoma revealed by acute renal failure (ARF), which remains a rare clinical entity. Case 1 was a 29-year-old man with an ARF. The diagnosis was a primitive kidney immunophenotype B lymphoma. The patient died after three courses of chemotherapy due to rapid spread lymphoma. The second case was a high-grade renal lymphomatous infiltration, with an unusual computer tomography image of two large kidneys compressing the stomach. Death happened early before initiating therapy. In both cases the diagnosis has been established by renal pathology. Early diagnosis is a key component of therapeutic success, however, the rapid spread of lymphoma worsened renal and vital prognosis., (Copyright © 2010 Association Société de néphrologie. Published by Elsevier SAS. All rights reserved.)