[Tuberculous vesicovaginal fistula complicated by bladder stones: about an exceptional case]. / Calculs vésicaux multiples compliquant une fistule vésico-vaginale d'origine tuberculeuse: à propos d'un cas exceptionnel.

Vesicovaginal fistula (VVF) continues to be a major public health problem in developing countries. Given the particular association of VVF with stones, the question that arises is whether the fistula is primary or secondary to bladder stone and then whether to use single-stage or two-stage treatment. But what is special about this study is that these rare clinical features are due to tuberculosis. We here report the case of a 62-year old female patient with a history of treated tuberculous spondylodiscitis. The patient had been declared cured 4 years before. For the previous 2 years she had been suffering from continuous urinary incontinence. Clinical examination showed almost complete vaginal synechia. Complementary scannography and cystography showed the presence of 3 stones on the way to the VVF. The largest stone measured 6cm along its longer axis with passage of contrast material into the uterovaginal cavity through the fistula. The patient was admitted to the operating room where she underwent cystolithotomy with ablation of the stones that were on the way to the fistula, biopsy of the fistulous tract and single-stage closure of the VVF in two layers without interposition of the autologous tissue. Anatomopathological results confirmed the presence of active tuberculosis on the way to the fistula, requiring resumption of antibacillary treatment for 9 months. During the follow-up visit at 3, 6 and 9 months the patient showed good clinical status with absence of urinary incontinence. VVF secondary to tuberculosis associated with urinary stones appears very little in literature. Our case demonstrates the feasibility of antibacillar medical treatment associated with single-stage surgical treatment with very satisfactory results despite the history of our patient and the duration of his disease.

[Outcome of locally advanced Darier and Ferrand dermatofibrosarcoma: about a case and literature review]. / Une réponse complète d'un dermatofibrosarcome de Darrier et Ferrand localement avancé: à propos d'un cas et revue de la littérature.

Darier and Ferrand dermatofibrosarcoma (DFS) is a rare skin tumor characterized by its local aggressiveness and a high risk of recurrences. Prognosis depends essentially on the quality of treatment. Complete response in Darier and Ferrand dermatofibrosarcoma with healthy margins, initially judged inoperable after 1 year of neoadjuvant imatinib-based treatment, has been rarely described in literature. We here report the case of a young female patient treated in the Department of Medical Oncology in FES for locally advanced DFS with complete response, in order to assess the role of imatinib and of targeted therapies in the treatment of DFS.

[Renal amyloidoses in nephrology]. / Les amyloses rénales en néphrologie.

The purpose of this study was to investigate the epidemiological, evolutionary and clinical features of the renal amyloidosis and to identify poor prognostic factors. We conducted a retrospective study focusing on all patients hospitalized for renal amyloidosis between January 2013 and December 2014. The diagnosis was confirmed by renal puncture-biopsy or by biopsy of minor salivary glands. We collected data from 25 patients, 17 men and eight women, with an average age of 47.2 ± 18 years. Hospitalization rate and prevalence were 2.4% and 12.5 cases/year respectively. On admission, nephrotic syndrome was detected in 100% of cases and renal failure in 68% of cases. Proteinuria was ≥6g/24h in 60% of cases. Digestive symptoms (n=14), cardiac symptoms (n=10) and arterial hypotension (n=11) were the other manifestations. Infectious and inflammatory diseases were the main causes found (60%). Tuberculosis alone accounted for 20%. After a mean follow-up period of 219.5 days, chronic renal failure was found in 16 cases (64%), including 11 cases with end-stage disease (44%). Six patients died. Renal insufficiency at the time of diagnosis, the worsening of renal function and readmission were associated with a risk for chronic terminal renal failure (p: 0.03-0.04). Cardiac damage, the readmission and proteinuria ≥6g/24h were factors associated with the risk of mortality (p< 0.03). Renal failure, cardiac damage, proteinuria ≥6g/24h and readmission were the main factors for poor prognosis in this cohort.

A rare pure embryonal rhabdomyosarcoma of the urinary bladder in an adult successfully managed with neoadjuvant chemotherapy and surgery: a case report.

BACKGROUND: Rhabdomyosarcoma of the urinary bladder in adults is an extremely rare malignant neoplasm that develops from the bladder wall. CASE PRESENTATION: We report our experience of a rare case of rhabdomyosarcoma of the bladder in a 45-year-old Moroccan man who was successfully managed with neoadjuvant chemotherapy and surgical excision of the mass; he was disease free at 24-month follow up. CONCLUSIONS: To the best of our knowledge, this is the first reported case of a rare embryonal rhabdomyosarcoma of the bladder that was managed with neoadjuvant chemotherapy and surgery. This is why further studies using a large number of patients with a greater longitudinal follow up will be required.

Myxoma of the kidney - an unusual benign renal tumor: a case report.

BACKGROUND: Myxomas are rare benign soft tissue tumors. The kidney is an unusual location for this tumor. For this reason, less than 15 cases of renal myxoma have been reported in the English literature. There are no specific clinical and radiological features reported for this tumor that allow a preoperative diagnosis enabling a conservative treatment. CASE PRESENTATION: We report another case of renal myxoma found in a 50-year-old Moroccan woman who presented with a right dull flank pain. An abdominal computed tomography scan objectified a suspected malignant renal mass. Thus, radical nephrectomy was performed. Histopathology of the specimen revealed the typical appearance of a myxoma. CONCLUSIONS: The objective of this report is to add another case report of this rare benign renal tumor to the literature. This benign tumor with excellent prognosis has no specific preoperative features that could justify a conservative management; a radical approach remains the therapeutic option for now.

Subependymal giant cell astrocytoma (SEGA): a case report and review of the literature.

BACKGROUND: Subependymal giant cell astrocytoma is a rare tumor that occurs in the wall of the lateral ventricle and foramen of Monro and, rarely, in the third ventricle. It is one of the intracranial lesions found in tuberous sclerosis complex (which include subependymal nodules, cortical tubers, retinal astrocytoma and subependymal giant cell astrocytoma), but cases without such lesions have also been reported in the literature. It was described for the first time in 1908 by Vogt as part of the typical triad of tuberous sclerosis complex. At the 2012 Washington Consensus Conference, it was decided by the invited expert panel to document the definition of subependymal giant cell astrocytoma as a lesion at the caudothalamic groove with either a size of more than 1 cm in any direction or a subependymal lesion at any location that has shown serial growth on consecutive imaging regardless of size. Most subependymal giant cell astrocytomas will show avid enhancement after contrast administration; however, a growing subependymal lesion even in the absence of enhancement should be considered a subependymal giant cell astrocytoma. CASE PRESENTATION: We report a case of subependymal giant cell astrocytoma in a 10-year-old white girl, who had no clinical symptoms of tuberous sclerosis. A computed tomography scan revealed a voluminous mass in her perilateral ventricle. An extemporaneous examination was in favor of a benign ganglioglioma tumor. After fixation in 10 % neutral-buffered formalin, embedding in paraffin and staining with hematoxylin, eosin and safran, the definitive diagnosis was subependymal giant cell astrocytoma. CONCLUSIONS: Subependymal giant cell astrocytoma is a rare tumor of the central nervous system whose diagnosis is based on clinical, radiological, histological and immunohistochemical arguments. For its rarity, we must consider this diagnosis when faced with a mass near the foramen of Monro in the pediatric population even if there are no other features of tuberous sclerosis complex.

[The role of dermoscopy in the diagnosis of basosquamous carcinoma]. / L'apport de la dermoscopie dans le carcinome basosquameux.

Basosquamous carcinoma (BSC) is a rare skin cancer which has areas of basal cell carcinoma (BCC) and squamous cell carcinoma (SCC) and a transition zone between them. However, dermoscopic features of BSC are not well described in the literature, except for two studies. The aim of this case study was to better identify and clarify the role of dermoscopy in the diagnosis of BSC, although histological confirmation is necessary.

Vaginal metastasis of pancreatic cancer.

Vaginal metastasis from pancreatic cancer is an extreme case and often indicates a poor prognosis. We present a case of pancreatic carcinoma with metastasis to the vagina that was discovered by vaginal bleeding. To our knowledge, this is the third case in the world of a primary pancreatic adenocarcinoma discovered of symptoms from a vaginal metastasis.

Primary perivascular epithelioid cell tumor of the liver: new case report and literature review.

Perivascular epithelioid cell tumors (PEComas) encompass a group of rare mesenchymal neoplasms, which typically have a perivascular location with dual melanocytic and muscular differentiation. They are found in a variety of localizations, though lesions in the liver are exceedingly rare. Because of their rarity, the clinical, radiological and histological features of these tumors have yet to be established. This is why, it seems appropriate to report the observation of this rare hepatic tumor with a literary review including others published cases, assessing through it, clinicopathologic and radiologic features of all reported cases as well as their follow-up whenever possible. VIRTUAL SLIDES: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1967094999126169.

Two rare schwannomas of head and neck.

Twenty-five to 45 percent of all schwannomas occur in the head and neck. Most of them arise along the vestibular portion of the eighth cranial nerve (acoustic neurinoma). They rarely originate from the peripheral facial nerve or other nerves within the parotid gland. Less than 4% of schwannomas involve the nasal cavity and paranasal sinuses. They arise from the branches of the trigeminal nerve and autonomic nervous system.We report two cases of schwannomas arisng from intraparotid facial nerve and nasal cavity. The first case diagnosed in a 62-year-old-man presented with 2-year-history of painless mass of the parotid gland. The lesion was found to be cystic through the pre operative examinations and investigations. The histology of the specimen retained an intraparotid cystic schwannoma. The second case concerned a 75-year-old-man presented with episodes of nasal obstruction, rhinorrhea, anosmia and headache. Histological study of the specimen showed fusiform cells with strongly and diffusely immunostaining for S100 protein suggesting a schwannoma. VIRTUAL SLIDES: http://www.diagnosticpathology.diagnomx.eu/vs/1098335216112242.

Primary renal teratoma: a rare entity.

Teratomas are neoplasms that arise from pluripotent cells and can differentiate along one or more embryonic germ lines. Renal teratoma is an exceedingly rare condition. Teratomas commonly arise in the gonads, sacrococcygeal region, pineal gland, and retroperitoneum. They present mainly as an abdominal mass with few other symptoms. Majority of the tumors are benign, situated on the left side and para renal, occasional lesions are bilateral. If diagnosed early, they are amenable to curative excision.Renal teratomas are rare and most have been dismissed as cases of teratoid nephroblastomas or retroperitoneal teratomas secondarily invading the kidney. The differentiation between these two neoplasms in the kidney is often problematic.We present a case of intrarenal immature teratoma in a six-month-old baby girl. VIRTUAL SLIDES: The virtual slides for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1746249869599954.

Multifocal tuberculosis revealed by multiple cutaneous gummas in the immunocompetent.

Cutaneous tuberculosis is rare. Its occurrence in multifocal tuberculosis (MT) is uncommon and happens frequently in the context of immunosuppression. We report the case of MT with multiple cutaneous gummas and bone and lung involvement that occurred in an apparently immunocompetent patient.

Solitary fibrous tumor of the kidney: a case report and review of the literature.

A solitary fibrous tumor (SFT) is an unusual spindle cell neoplasm that usually occurs in the pleura but has recently been described in diverse extrapleural sites. Urogenital localization is rare, and only 19 cases of SFT of the kidney have been described. We report a case of a large SFT clinically thought to be renal cell carcinoma arising in the kidney of a 70-year-old man. The tumor was well circumscribed and composed of a mixture of spindle cells and dense collagenous bands, with areas of necrosis or cystic changes noted macroscopically and microscopically. Immunohistochemical studies revealed reactivity for CD34, CD99, and Bcl-2 protein, with no staining for keratin, S-100 protein, or muscle markers, confirming the diagnosis of SFT. This tumor is benign in up to 90% of cases. The immunohistochemical study is the key to diagnosis.