Primary epitheloid angiosarcoma of the pleura: an exceptional tumor location.

Primary angiosarcoma of the pleura is an extremely rare tumour arising from arterial or venous pulmonary vessels of various size. It is characterized by an aggressive course and a poor prognosis. The early diagnosis is challenging due to diverse clinical and radiological manifestations. We report a case of a 70 year old male with primary right pleural epitheloid angiosarcoma. The patient had a history of a two week's progressive dyspnea. CT-scan showed a prominent thikening of the right pleura associated with pleural effusion and atelectasis. CT-scan guided by biopsy was performed and histological examination showed a tumor proliferation consisting of sheets of polygonal and epitheloid cells showing rudimentary vascular differentiation. Immunohistochemically, tumor cells were strongly positive for CD31 and Factor VIII-related antigen, negative for CD34, weakly and focally positive for EMA and Cytokeratine. The overall pathological and immunohistochemical features of the pleural specimens supported the diagnosis of epitheloid angiosarcma. The patient died after a week of discharge by pulsless ventricular tachycardia arrest. In addition, we also present a brief litterature review on pleural angiosarcoma. Our experience with this case suggests that comprehensive and sufficient sample collection and meticulous histological examination aided with immunohistochemical stains, particulary the endothelial markers, are required for accurate diagnosis of this rare malignancy.

[Primary cerebral gliosarcoma: about two cases and review of the literature]. / Gliosarcome cérébral primitif: à propos de deux cas et revue de la littérature.

Gliosarcoma is a very rare brain tumor accounting for 1.8 -8% of all glial tumors. It has been classified by the World Health Organization as a variant of glioblastoma. It is a tumor with double glial and sarcomatous component. Patient's clinical picture is polymorphic, imaging data are evocative, diagnosis is based on histology. Treatment is always surgical. Prognosis is closely linked to the quality of resection. We here report two clinical cases with the aim of assessing the diagnostic, therapeutic and prognostic features of this rare entity.

An Unusual Cause of Intestinal Obstruction in a Young Adult Patient: Inflammatory Fibroid Polyp.

Inflammatory fibroid polyps are uncommon benign lesions that originate in the submucosa of the gastrointestinal tract. The stomach and the ileum are the most commonly affected sites. Although inflammatory fibroid polyp is one of the rare conditions leading to intestinal obstruction in adults, it should be considered as a possible diagnosis in obstructive tumors of the small bowel causing intussusceptions. We present one case of inflammatory fibroid polyp as a rare cause of intussusception in a young adult patient.

Renal damage induced by pemetrexed causing drug discontinuation: a case report and review of the literature.

BACKGROUND: Pemetrexed maintenance therapy holds tremendous potential in improving the survival of patients with advanced pulmonary adenocarcinoma. Major side effects include myelosuppression and cutaneous reactions. However, little data are available on pemetrexed nephrotoxicity. Our case describes clinically relevant renal events leading to treatment discontinuation in routine practice. CASE PRESENTATION: We report a case of a 69-year-old Moroccan man treated for metastatic non-small cell lung cancer. He was not on any other medications and he did not receive any nephrotoxic agents. He was exposed to intravenously administered contrast from thoracoabdominal computed tomography in the week of his last pemetrexed treatment. He developed kidney disease related to pemetrexed. He was submitted to renal biopsy, which showed acute tubular damage and interstitial fibrosis. His kidney function remained impaired, but stable, after discontinuation of pemetrexed therapy. He died 5 months later. CONCLUSIONS: Medical oncologists should be aware of renal adverse events for patients with advanced non-small cell lung cancer eligible for pemetrexed maintenance therapy. Suggestions for mitigating the risk for renal toxicities (dehydration, non-steroidal anti-inflammatory drugs and zoledronic acid, radiocontrast agents) during pemetrexed maintenance should be followed to enable early detection and management of this adverse event.

[Soft tissue melanoma: a clinical case]. / Mélanome des tissus mous: cas clinique.

Soft tissue melanoma was first described by Enzinger in 1965 under the name of clear cell sarcoma. In 1983, Chung and Enzinger renamed it soft tissue melanoma due to its immunohistochemical similarities with melanoma. We here report the case of a 22-year old young man with this rare type of melanoma, presenting with molluscoid lesion on his ankle without any clinical sign of malignancy. Histology examination confirmed the diagnosis of soft tissue melanoma.

Metachronous pancreatic metastases from renal cell carcinoma: is there a place of Active-Surveillance before deferred deliberately Molecular Target Agent?

BACKGROUND: Metastatic renal cell cancer is a heterogeneous disease due to its diverse morphological features, the prognostic categories based on clinical criteria. Sometimes indolent course without any significant symptoms can be differentiated before the introduction of novel targeted agents. This observation led to interest in a strategy of deferring systemic therapy in the era of effective systemic therapies. CASE PRESENTATION: We report of a 78-year-old Moroccan man with pancreatic metastasis from renal cell carcinoma which occurred 14 years from right nephrectomy. Indolent disease based on body computed tomography imaging with 4 years follow-up was recognized. Active surveillance with deferred antiangiogenic multikinase inhibitor at disease progression was proposed. Nowadays, the patient is under oncological follow-up, he is in a good state of health, and he is disease-free for 48 months from the diagnosis of the tumor and for 20 months from the start of the treatment with Sunitinib CONCLUSIONS: Active surveillance before target therapy may be a suitable approach to ensure long progression-free survival with minimal side-effects and better quality of life in asymptomatic, low-volume, metastatic disease. Further prospective studies with biomarker validation are required to define the patients most likely to benefit from this approach.

Upper urinary tract lipoma: A case report.

Upper urinary tract (UUT) benign tumours are rare. We present a case of UUT lipoma in a 41-year-old man with left flank pain. A computed tomographic urography scan revealed an irregular thickening of the left renal collecting system wall extending from the upper calices to the renal pelvis. The diagnosis of UUT was made and the patient underwent a nephroureterectomy with bladder cuff excision, as standard treatment. However, macroscopic and histological examination revealed a lipomatous tumour with no sign of malignancy. To our knowledge this is the first reported case of its kind of a UUT managed first with a minimally invasive approach.

[Pseudo-condylomatous neurodermatitis of the anal margin]. / Forme pseudo-condylomateuse d'une nevrodermite de la marge anale.

Cerebral venous thrombosis (CVT) is a rare origin of stroke, the clinical presentation and etiologies vary. The prognosis is shown to be better compared to arterial thrombosis. Magnetic Resonance Imaging (MRI) and MR Venograpgy (MRV) are currently important tools for the diagnostic. We studied 30 cases of CVT diagnosed in the department of neurology of the University Hospital of Fez (Morocco). Patients diagnosed with CVT signs between January 2003 and October 2007 were included in the study. Cerebral CT-scan was performed in 27 cases (90%) while the MRI examination was done in 18 patients (67%); and most patients (90%) received anticoagulant therapy. The mean age of our patients was of 29 years (age range between 18 days and 65 years). A female predominance was observed (70%). The clinical presentation of patients was dominated by: headache in 24 cases (80%), motor and sensory disability in 15 cases (50%), seizures in 10 cases (33%) , consciousness disorder in 10 cases (33%). CVT was associated to post-partum in 10 cases (33%), infectious origin in 8 cases (26%), Behcet disease in 2 cases (7%), pulmonary carcinoma in 1 case, thrombocytemia in 1 case and idiopathic in 7 cases (23%). The evolution was good in 20 cases (67%), minor squelaes were observed in 6 patients (20%), while major squelaes was observed in 2 cases. Two cases of death were registered. The CVT is a pathology of good prognosis once the diagnosis is promptly established and early heparin treatment initiated.

Complete androgen insensitivity syndrome with a Sertoli-Leydig cell tumor.

BACKGROUND: The complete androgen insensitivity (testicular feminization) syndrome was described in phenotypic females with 46XY karyotype, presenting with primary amenorrhea, adequate breast development, and absent or sparse pubic or axillary hair. Gonads consist usually of seminiferous tubules without spermatogenesis. CASE: We report the case of a 15-year-old girl with testicular feminization since age 4. She was admitted to surgically remove the testes from the inguinal canal. Microscopic examination of the left testis revealed a Sertoli-Leydig cell tumor and the draft of fallopian tube with adjacent seminiferous tubules. SUMMARY AND CONCLUSION: These patients have a 5%-10% risk of developing germ cell tumors. Sertoli-Leydig cell tumors are uncommon neoplasms, usually benign in testicular feminization syndrome.

[Secondary gliosarcoma: case report]. / Gliosarcome secondaire : à propos d'un cas.

Gliosarcoma is a rare tumor of the central nervous system, consisting of gliomatous and sarcomatous elements. The glioblastoma can undergo a change in phenotype, transforming into a gliosarcoma, especially when the tumor has been treated with radiotherapy. Features unique to gliosarcoma compared to glioblastoma include their potential to appear similar to a meningioma at macroscopy, repeated reports of metastases and infrequency of EGFR mutations. We present a case of secondary gliosarcoma to emphasize on the specificities, essentially diagnostical of this rare entity.

Is there any advantage to combined trastuzumab and chemotherapy in perioperative setting her 2neu positive localized gastric adenocarcinoma?

We report here a 44-year-old Moroccan man with resectable gastric adenocarcinoma with overexpression of human epidermal growth factor receptor 2 (HER2) by immunohistochemistry who was treated with trastuzumab in combination with chemotherapy in perioperative setting. He received 3 cycles of neoadjuvant chemotherapy consisting of trastuzumab, oxaliplatin, and capecitabine. Afterwards, he received total gastrectomy with extended D2 lymphadenectomy without spleno-pancreatectomy. A pathologic complete response was obtained with a combination of trastuzumab and oxaliplatin and capecitabine. He received 3 more cycles of trastuzumab containing regimen postoperatively.We conclude that resectable gastric carcinoma with overexpression of the c-erbB-2 protein should ideally be managed with perioperative combination of trastuzumab with chemotherapy. Further research to evaluate trastuzumab in combination with chemotherapy regimens in the perioperative and adjuvant setting is urgently needed.

Diagnostic challenge for ovarian malignant melanoma in premenopausal women: primary or metastatic?

BACKGROUND: In the ovary, metastatic malignant melanoma may be confused with primary malignant melanoma and presents a diagnosis challenge. Most cases are associated with disseminated diseases and poor prognosis. We present this case report of a metastatic ovarian malignant melanoma simulating primary ovarian cancer. CASE REPORT: A 45-year-old premenopausal woman was incidentally found to have an abdominal mass, 3 years after removal of a cutaneous melanoma lesion. Ultrasound and CT scan revealed left two solid masses, which were found to be an ovarian tumor at laparotomy. Left oophorectomy was performed. Histopathology and immunohistochemistry showed melanoma metastasis to the ovary. Nine months later, the patient developed epilepsy and confusion. Magnetic Resonance Imaging showed unique Wright frontal lobe lesion. She underwent stereotactic radio surgery and dacarbazine monotherapy. For months later, the patient is died from disseminate disease progression. CONCLUSION: Ovarian metastasis is an unusual presentation of cutaneous melanoma and the prognosis was dismal. As illustrated by this case report, a differential diagnosis of a metastatic malignant melanoma must be considered.

Invasive Aspergillus terreus sinusitis with orbitocranial extension: case report.

BACKGROUND: Aspergillosis of the paranasal sinuses is infrequent and usually involves the species Aspergillus fumigatus and A. flavus. The maxillary sinus is the most common sinus to be affected. Invasive cranio-orbital aspergillosis originating in the sphenoid sinus is rare and mostly occurs in immunocompromised patients with poor outcomes. We present a case of invasive A. terreus sphenoidal sinusitis with intraorbital and intracranial extension in an immunocompetent patient. CASE DESCRIPTION: This 62-year-old man presented with a 2-month history of left retroorbital pain followed by rapid decreasing vision and 2 episodes of epistaxis. Ophthalmologic examination revealed no light perception left. Computed tomographic scan and MR images demonstrated an enhanced sphenoid lesion within the left sphenoid sinus with bone destruction and intraorbital and cavernous sinus extensions. A malignant tumor was suspected. The patient underwent a transphenoidal biopsy of the sphenoid mass. Histologic analysis revealed numerous Aspergillus hyphea, and the species A. terreus was isolated from fungal cultures of specimens. No systemic fungal infection was found, and the patient had no evidence of immunosuppression. After 3 months' administration of oral voriconazole, the patient became well, and the orbitocranial mass regressed in size. It was stabilized on the ninth postoperative month. CONCLUSION: A. terreus sinusitis with orbitocranial extension had never been reported in the literature. Even in an immunocompetent host, ISOA is difficult to eradicate using surgical debridement combined with optimal antifungal agents because of the intracranial extension and the relative resistance of conventional antifungal therapy. Early diagnosis is important to prevent an unfavorable outcome of this emergent infection.

Sudden onset of paraplegia caused by hemorrhagic spinal epidural angiolipoma. A case report.

Spinal epidural angiolipoma is a rare benign tumor containing vascular and mature adipose elements. A slow progressive clinical course was mostly presented and rarely a fluctuating course during pregnancy. The authors report the original case of spontaneous spinal epidural bleeding resulting from thoracic epidural angiolipoma who presented with hyperacute onset of paraplegia, simulating an extradural hematoma. The patient was admitted with sudden non-traumatic hyperacute paraplegia during a prolonged walk. Neurologic examination showed sensory loss below T6 and bladder disturbances. Spinal MRI revealed a non-enhanced heterogeneous thoracic epidural lesion, extending from T2 to T3. A bilateral T2-T4 laminectomy was performed to achieve resection of a lipomatous tumor containing area of spontaneous hemorrhage. The postoperative course was uneventful with complete neurologic recovery. Histologic examination revealed the tumor as an angiolipoma. Because the prognosis after rapid surgical management of this lesion is favorable, the diagnosis of spinal angiolipoma with bleeding should be considered in the differential diagnosis of hyperacute spinal cord compression.