Immunoglobulin G4-related disease presenting as an intracranial mass extended from the infratemporal fossa.

Background: Neurological manifestations in immunoglobulin G4-related diseases (IgG4-RD) are rare and documented in <2% of cases. It commonly involves pachymeninges forming hypertrophic pachymeningitis and rarely forms tumor-like masses. Case Description: We present our experience with a biopsy-proven case of IgG4-RD presenting with an intracranial extradural tumor-like mass infiltrating the temporal lobe. The patient was treated with high doses of corticosteroids followed by slow tapering. The neurological manifestations gradually improved and resolved after 2 months with a cerebral MRI showing a significant reduction in the tumoral size. Conclusion: When it comes to intracranial mass, IgG4-RD neuropathy is one of the rarest differential diagnoses for the central nervous system tumors. Early recognition of IgG4-RD and appropriate establishment of its long-term treatment may avoid unnecessary investigations and morbidity.

Ossifying fibromyxoid tumor of soft tissue: A case report with review of literature.

INTRODUCTION AND IMPORTANCE: The ossifying fibromyxoid tumor of soft tissue is a rare tumor of intermediate differentiation and uncertain lineage that occurs in adults mostly in the extremities and the trunk. PRESENTATION OF CASE: we present a case of 57 year-old man presenting with a right scapular mass. It was a subcutaneous and painless mass that was largely excised. The diagnosis of ossifying fibromyxoid tumor of the right shoulder was made. The follow up of 1 year was without recurrence and metastasis. CLINICAL DISCUSSION: The ossifying fibromyxoid tumor of soft tissue is exceptional, microscopic diagnosis and management is challenging, considering the scarcity of the tumor. CONCLUSION: More cases and retrospective studies are needed to understand the pathogenesis and to determine optimal treatment regimens.

Erdheim-chester disease revealed by diabetes insipidus.

Erdheim-Chester disease (ECD) is a very rare and aggressive form of non-Langerhans histiocytosis with unclear pathogenesis. Because of the heterogeneity of clinical presentation, diagnosis is often challenging and delayed. Currently, Interferon alpha is the first line treatment that is associated with a better survival. The prognosis is relatively poor, especially in case of neurological and cardiovascular involvement. Herein, we report the case of a 64-year-old Tunisian female patient presenting an aggressive form of ECD revealed by diabetes insipidus and cerebellar ataxia with a diagnosis delay of 4 years. The assessment of disease extent had also shown associated asymptomatic cardiac and bone involvement. Pegylated Interferon alpha was started at high dose allowing disease stabilization. This case illustrates that physicians should be aware of the heterogeneous manifestations of ECD in order to insure an early diagnosis and treatment. Long-term and regular follow-up is crucial because of the risk of disease progression.

Fetal Rhabdomyomatous Nephroblastoma in a 31-Year-Old Woman: A Case Report.

Fetal rhabdomyomatous nephroblastoma (FRN) is a rare variant of Wilms tumor with distinct morphologic features and biologic behavior compared to conventional nephroblastoma. It mainly occurs in patients under 4 years. In adults, extremely rare cases of nephroblastoma were reported. Among these cases, none has been interested a FRN. We report an exceptional case of a 31-year-old woman diagnosed with FRN discovered incidentally, to illustrate clinical and histopathological characteristics of this entity.

Congenital Glioblastoma: Lessons Learned from a Rare Case with Unusual Presentation.

Congenital glioblastomas are uncommon tumors of infancy with unique molecular features and usually better prognosis compared to their pediatric and adult counterparts. A 15-day old newborn harboring a congenital glioblastoma undiagnosed at pregnancy presented to our emergencies with rapid neurological deterioration. Radiological investigations revealed an 8 by 9 cm space occupying and enhancing lesion in the left temporoparietooccipital region with intratumoral hemorrhage. The patient was immediately transferred to the operative theater and was operated in a poor condition. Unfortunately he showed no improvement post-operatively and died within two days in the intensive care unit. As such presentation is unique; we present the case and review the relevant literature concerning the potential pitfalls in diagnosis, management strategies and improvements in outcome.

Epidural Venous Angioma Presenting with Spinal Cord Compression in a 42-Year-Old Woman with Previous History of Ovarian Malignancy.

BACKGROUND: Venous angioma is an extremely rare vascular malformation of the epidural space. To the best of our knowledge, only 5 cases have been documented to date and none has been reported in the setting of a previous malignancy. CASE DESCRIPTION: We report the case of a 42-year-old woman with a previous history of ovarian cancer, treated by surgery plus chemotherapy; who presented with signs of spinal cord compression for 3 weeks. Magnetic resonance imaging showed an intensely enhancing epidural mass at the T2-T6 level causing major spinal cord compression, for which urgent surgery was indicated. During surgery, the tumor was extremely hemorrhagic and the hemostasis was hazardous. Blood loss was estimated at 1.5 L, causing hemodynamic instability and requiring intensive resuscitation with fluids and blood transfusions. Gross total resection was achieved and the pathologic examination confirmed the diagnosis of venous angioma. The patient recovered quickly postoperatively and was able to walk independently within 2 weeks of starting intensive rehabilitation. She was symptom free with no clinical or radiologic evidence of recurrence at 1 year follow-up. CONCLUSIONS: Venous angioma should be included in the differential diagnosis of spinal epidural masses even in case of previous malignancy. Subtle imaging features should alert clinicians to this rare yet potentially life-threatening condition. Surgery remains the cornerstone of the treatment and can result in remarkable recovery.

Primary pure and nonsecreting embryonal carcinoma of the anterior third ventricle: a case report.

Central nervous system germ cell tumors (GCTs) account for less than 5% of primary brain tumors in children and adolescents but continue to attract much attention. To the best of our knowledge, a primary pure and nonsecreting embryonal carcinoma of the anterior third ventricle has never been previously reported. A 15-year-old boy presented with signs of increased intracranial pressure for the past 2 weeks complicated by 2 episodes of generalized tonic-clonic seizures 1 day before admission. Neurological examination was normal, and funduscopic examination disclosed a grade II papilledema bilaterally. CT and MRI revealed a well-demarcated and enhancing mass within the anterior third ventricle associated with a left lateral ventricle hydrocephalus. There was no evidence of tumor within the pineal or suprasellar region, and systemic and cerebrospinal fluid evaluation demonstrated normal levels of α-fetoprotein and human chorionic gonadotropin. Radical surgery was advised, and total tumor resection was achieved via a transcallosal transforaminal approach. The postoperative course was uneventful, and the final histological diagnosis was a pure embryonal carcinoma. Further screening showed no other location, and adjunctive high-dose chemotherapy was administered. The patient has been symptom free with no clinical or radiological sign of progression at the most recent follow-up examination 2 years after surgery. Primary pure and nonsecreting embryonal carcinoma can develop within the anterior third ventricle and should be considered in the differential diagnosis of anterior third ventricular masses especially in young patients. Accurate identification, radical surgery and high-dose chemotherapy can result in better tumor control and improve the postoperative outcome.

Cutaneous alternariosis in a renal transplant recipient.

Alternariosis is a fungal infection that is usually described in immunocompromised patients. We report a case of cutaneous alternariosis in a renal transplant recipient caused by Alternaria tenuissima. The diagnosis was supported by histopathologic (ie, yeastlike cells, filamentous structures) and mycologic findings from a cutaneous biopsy. Cutaneous lesions regressed 1 month following a decrease in the dosage of immunosuppressive therapy. The patient also was treated with intravenous amphotericin B followed by oral fluconazole without improvement. Cryotherapy remarkably accelerated healing of the lesions.

Neuroretinitis: a rare feature of tubulointerstitial nephritis and uveitis syndrome.

A 17-year-old female presented to our hospital with fatigue, arthralgia and fever associated with non-granulomatous anterior uveitis in the left eye. Blood tests revealed renal failure and a renal biopsy showed acute tubulointerstitial nephritis. Findings were consistent with tubulointerstitial nephritis and uveitis (TINU) syndrome. The patient received systemic corticosteroids for 2 months as well as dexamethasone and mydriatic drops. Bilateral non-granulomatous anterior uveitis occurred 10 days after prednisone discontinuation, and was treated with topical steroids; however, uveitis recurred several weeks later. Bilateral anterior chamber inflammation was associated with neuroretinitis in the right eye. Extensive posterior synechiae precluded fundus examination of the left eye. The posterior segment manifestations responded to systemic prednisone, which was slowly tapered. The final visual outcome was favorable. TINU syndrome may rarely manifest with neuroretinitis.

[Angiomatoid tumor of the thyroid gland: primitive angiosarcoma or variant of anaplastic carcinoma?]. / Tumeur angiomatoïde de la thyroïde: angiosarcome primitif ou variante du carcinome anaplasique?

Thyroid gland angiomatoid tumors are an extremely aggressive neoplasms with varied histological patterns and features of endothelial differentiation. The histogenesis of thyroid angiomatoid tumors has been controversial for many years: these tumors may be either a variant of anaplastic carcinoma, or an angiosarcoma. We report a case of thyroid angiomatoid tumor in a 68-year-old woman. We also discuss, through a review of the literature, the pathologic criteria that could be used to distinguish between angiosarcoma and anaplastic carcinoma of the thyroid.

[Myxoid plexiform fibrohistiocytic tumor without multinucleated giant cells]. / Tumeur fibro-histiocytaire plexiforme myxoïde sans cellules géantes multinucléées.

Plexiform fibrohistiocytic tumour is a rare soft-tissue tumour of intermediate malignancy. It is more prevalent in children and in young adults. We report a case of this tumour presenting as a nontender swelling on the shoulder of an 8-year-old girl. Clinically, the mass was subcutaneous and measured 2 cm in greatest diameter. The histopathological examination revealed a mesenchymal dermic and subcutaneous tumour composed of confluent nodules. These nodules were made of aggregates of histiocyte-like cells and concentric fascicles of spindle cells. The stroma was myxoïd or collagenous. This case was particular by the lack of giant cells and the focally myxoid background. The epidemiologic, clinicopathologic features and outcome of this lesion are discussed and similar published cases are reviewed.

[Micropapillary bladder carcinoma]. / Carcinome micropapillaire de la vessie.

Micropapillary bladder carcinoma is a recently described, rare variant of urothelial carcinoma reputed for its poor prognosis. The authors report the case of a 59-year-old man with invasive bladder tumour presenting with haematuria. Histological examination of resection chips revealed the typical histological features of high nuclear grade micropapillary carcinoma with invasion of the musculosa and the presence of numerous endolymphatic emboli. The authors discuss the clinicopathological and pathogenic features, treatment and clinical course of this tumour.

[Inverted papilloma of the ureter: a case report]. / Papillome inversé de l'uretère.

Inverted urothelial papilloma is a rare tumour particularly in the ureter. It is difficult to distinguish microscopically from low-grade urothelial carcinoma, inverted type. The authors report a new case of ureteric inverted papilloma in a 64-year-old man and discuss the histological diagnostic criteria, clinical features and prognosis of this tumour.