Langerhans cell sarcoma of the nasopharynx: a rare case.

Langerhans cell sarcoma, a tumour with markedly malignant cytological features that originates from Langerhans cells, is a very rare disease. We report the first case of 39-year-old male with Langerhans cell sarcoma arising in the nasopharynx. We chose the 2-chlorodeoxyadenosine (2-CDA) regimen as first-line chemotherapy, and clinical improvement of Langerhans cell sarcoma was obtained. After the fourth cycle of 2-CDA therapy, however, disease progression was observed, and we administered ESHAP regimen (etoposide, carboplatin, cytarabine, methylprednisolone) as a second-line therapy. After we administered two cycles of ESHAP, however, the patient developed aggressive progression and he died. The importance of immunohistochemical findings is obvious in Langerhans cell sarcoma diagnosis. Considering that Langerhans cell sarcoma behaves in a very malignant fashion, a more aggressive treatment approach is necessary for patients with Langerhans cell sarcoma.

Rhinocerebral mucormycosis: experience in 14 patients.

OBJECTIVE: Mucormycosis is an opportunist, often lethal fungal infection which occurs in immunocompromised patients. We present our experience in 14 patients with this condition. PATIENTS AND METHODS: A retrospective chart review was conducted for 14 patients treated for rhinocerebral mucormycosis. RESULTS: Nine patients had diabetes mellitus and six had a haematological malignancy. Nine patients had cutaneous and/or palatal necrosis. Eleven patients were treated with amphotericin B and five with liposomal amphotericin B. Endoscopic sinus surgery was performed in five patients with disease limited to the sinonasal cavity; nine patients underwent more extensive surgery. Five patients with disease limited to the sinonasal cavity survived, while nine patients with widely disseminated disease died. Five of the nine diabetic patients died, as did five of the six patients with haematological malignancy. CONCLUSION: Patients with rhinocerebral mucormycosis spreading outside the sinonasal cavity have a poor prognosis.

A newborn with bullous pemphigoid associated with linear IgA bullous dermatosis.

A 16-day-old boy was admitted to our clinic with localized blisters on the neck, cheeks, earlobes, and oral cavity and with erythema on the toes, in addition to poor weight gain and respiratory distress. A physical examination revealed several erythematous plaques with tense bullae, multiple vesicles, and erosions on the left toes, neck, earlobes, and face as well as erosive lesions on the anterior part of the oral cavity, lips, and buccal mucosae. A bronchoscopic examination revealed bullous lesions in the upper respiratory tract and on the epiglottis. A skin biopsy suggested a diagnosis of bullous pemphigoid (BP). Because of the severe mucosal involvement, further investigations including various immunological techniques were performed. The case was diagnosed as BP associated with linear IgA bullous disease (LAD). Complete remission without any scarring was achieved after three weeks of oral methyl prednisolone treatment. A correct differential diagnosis of bullous diseases is important for determining the prognosis and expected response to treatment. To our knowledge, this is the first case of BP associated with LAD reported in literature.

The role of diffusion-weighted magnetic resonance imaging in intracranial cystic lesions.

We aimed to define the diffusion-weighted magnetic resonance (MR) imaging features of intracranial cystic lesions and to investigate possible special features for the differential diagnosis. One hundred and twenty patients with intracranial cystic lesions were included in the study. There were 29 arachnoid cysts, eight epidermoid cysts, 34 primary tumors, 18 abscesses, 29 metastases and two hydatid cysts. Echo-planar diffusion-weighted MR imaging was obtained in addition to conventional cranial MR scans. The morphologic features of the cystic portion and the wall of the cyst and signal intensities on diffusion-weighted images were evaluated. All abscesses and epidermoid cysts were hyperintense on diffusion-weighted images. Arachnoid cysts, hydatid cysts, primary tumors, and metastases were hypointense except five cystic tumors. These five primary or metastatic necrotic tumors showed high signal intensity on diffusion-weighted images due to hemorrhage or superinfection. The walls of the cystic tumors were usually hyperintense on diffusion-weighted images in contrast to the wall of the abscesses, which were iso-hypointense. This was a statistically significant finding for the differentiation between tumors and abscesses (p<0.05). Diffusion-weighted MR imaging is a useful technique for the evaluation of the intracranial cystic lesions and provides additional beneficial information to conventional MR imaging. However, the presence of hemorrhage and superinfection of the tumors may cause a signal increase that results in misinterpretetations. In these cases, the appearance of tumor wall may be useful for differentiating abscesses from tumors.

Familial nodular lymphocyte predominant Hodgkin lymphoma: Successful treatment with CHOP plus rituximab.

Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is a rare tumor type distinct from classical Hodgkin lymphoma and its familial form is unusual. The two cases (mother at age 48 and son at age 30 years) of NLPHL in advanced clinical stage are described. The patients were successfully treated with an immunochemotherapy schedule consisting CHOP plus rituximab (CHOP-R). This chemotherapy was well tolerated and the patients reached complete remission. These remissions were for 34 and 40 months for mother and son, respectively. In patients with NLPHL, CHOP-R regimen should be used as an alternative treatment regimen to obtain a good long-lasting response without any adverse events.

A case of subcutaneous panniculitis-like T-cell lymphoma with haemophagocytosis developing secondary to chemotherapy.

A 45-year-old woman presented with fever, generalized skin lesions and multiple lymphadenopathies. In her past history she had had six courses of cyclophosphamide and cisplatin combination chemotherapy 7 years ago because of an ovarian carcinoma. We found pancytopenia in the peripheral blood examination. Skin biopsy showed diffuse subcutaneous infiltration reminiscent of panniculitis but composed of malignant lymphoid cells that were of T lineage. Bone marrow biopsy showed normocellular myeloid tissue with abundant haemophagocytic macrophages. Subcutaneous panniculitis-like T-cell lymphoma with haemophagocytic syndrome was diagnosed. This is the first case reported of subcutaneous panniculitis-like lymphoma occurring secondary to chemotherapy.

Post-operative sequential chemo-radiotherapy in high-grade cerebral gliomas with fotemustine.

High-grade gliomas are the most common primary brain tumors in adults. Twenty-seven patients with histopathologically proven anaplastic astrocytoma and glioblastoma multiforme were enrolled in this study from November 1998 to August 2002. Radiotherapy was administered after surgery and fotemustine (100 mg/m2) was sequentially administered every 3 weeks for 6 cycles. Overall, 111 cycles were administered to the 27 patients (median, 5 cycles; range, 1 to 6 cycles). Myelosuppression was mild to moderate. The median overall survival and progression free survival were 11+/-3.1 months (95%CI, 4.9-17.1) and 8+/-0.5 months (95%CI 7.1-8.9), respectively. One-year and two-year survivals were calculated at 48% and 7%, respectively. Significant prognostic factors (P<0.05) via univariate analysis were divided into two groups: completion of 6 cycles of chemotherapy versus incompletion of 6 cycles of chemotherapy. This trial demonstrates that postoperative radiotherapy and sequential fotemustine therapy is feasible, well tolerated, and may prolong survival in patients with newly diagnosed high-grade gliomas.

Inflammatory myofibroblastic tumor of the nasal cavity: a case report and review of the literature.

Our aim is to describe clinicopathological, histochemical, and immunohistochemical findings of one case of inflammatory myofibroblastic tumor of the nasal cavity. A 10-year-old female presented with a short history of nasal obstruction, epistaxis, nasal discharge and headache. Computerized tomography (CT) scans showed a space-occupying lesion in the right nasal cavity. Histological examination of initial biopsy showed fascicles of spindle cells in a mixed inflammatory background with a predominance of plasma cells, typical of inflammatory pseudotumor. The spindle cells were positive for vimentin and actin. The mass was completely excised without any difficulty under generalized anesthesia. Inflammatory myofibroblastic tumor of the nasal cavity is a localized and completely benign lesion. Simple complete excision is curative.

Cohen syndrome with acanthosis nigricans and insulin resistance.

Cohen syndrome is a rare genetic disorder consisting of truncal obesity, hypotonia, mental retardation, microcephalia, characteristic facial appearance and ocular anomalies. Other diagnostic clinical features include narrow hands and feet, low growth parameters, neutropenia and chorioretinal dystrophy. Acanthosis nigricans is a cutaneous disorder characterized by hyperpigmentation and papillomatosis. Syndromal acanthosis nigricans may occasionally appear as a feature of several specific syndromes. We report a patient showing the typical characteristics of Cohen syndrome with acanthosis nigricans and hyperinsulinemia.

Evaluation of contralateral testicular damage after unilateral testicular torsion by serum inhibin B levels.

BACKGROUND/PURPOSE: It is still controversial whether unilateral testicular torsion (TT) affects contralateral testis. The authors wanted to evaluate contralateral testicular damage in a rat model by the serum inhibin B levels, which is suggested as a marker of Sertoli cell function and spermatogenesis. METHODS: Fifty peripubertal male Wistar Albino rats were divided into 5 groups each containing 10 rats. Surgery was conducted under intraperitoneal 1-shot ketamine (50 mg/kg) anesthesia. Torsion-detorsion, torsion-detorsion-orchiectomies, orchiectomies, and sham operations were performed on the right testicle through a midline incision. Torsions were created by rotating the right testes 720 degrees in a clockwise direction and maintained by fixing the testes to the scrotum with a silk suture. Torsion duration was 4 hours. After each surgical intervention the incisions were closed. In group 1, 3-mL blood samples were taken to determine basal values of inhibin B in serum, and bilateral orchiectomies were performed. In group 2, 4 hours of torsion and detorsion was created and 1 month later, 3-mL blood samples were taken, and bilateral orchiectomies were performed. In group 3, 4 hours of torsion-4 hours of detorsion was created, and right orchiectomies were performed and 1 month later, 3-mL blood samples were taken and contralateral orchiectomies were added. In group 4, unilateral orchiectomies were performed, and 1 month later, 3-mL blood samples were taken, and contralateral orchiectomies were added. After the measurement of the serum inhibin B levels and histopathologic examinations, results are expressed as mean +/- SD. RESULTS: Serum inhibin B levels expressed as mean +/- SD were 108.233 +/- 21.296 pg/mL for group 1, 54.065 +/- 16.910 pg/mL for group 2, 74.195 +/- 2.779 pg/mL for group 3, 108.335 +/- 26.078 pg/mL for group 4, and 107.645 +/- 4.705 pg/mL for group 5. Inhibin B levels in group 2 and group 3 were different from group 1, group 4, and group 5 (P <.05). In histologic examination, Johnsen's scores expressed as mean +/- SD of right testes were 9.74 +/- 0.08 for group 1, 3.64 +/- 3.36 for group 2, and 9.86 +/- 0.05 for group 5. Histologic findings in group 2 were different from group 1 and group 5 (P <.05). Johnsen's scores expressed as mean +/- SD of left testes were 9.78 +/- 0.09 for group 1, 9.75 +/- 0.14 for group 2, 9.76 +/- 0.15 for group 3, 9.79 +/- 0.07 for group 4, and 9.82 +/- 0.08 for group 5, and there was no difference between groups (P >.05). CONCLUSIONS: The serum inhibin B levels decrease after unilateral TT reflecting contralateral testicular damage. Orchiectomy to prevent contralateral testicular damage after TT may not be effective after critical period. Measurement of inhibin B levels to evaluate contralateral testicular damage after unilateral TT is more effective than histopathologic examination.

Facial nerve paralysis and paraplegia as presenting symptoms of acute myeloid leukemia.

Granulocytic sarcoma is an extramedullary tumor associated with acute or chronic leukemias or myeloproliferative disorders. Rarely, the tumor may be seen before the diagnosis of leukemia. Symptomatic facial nerve paralysis and spinal cord invasion by granulocytic sarcomas are also relatively uncommon. We present here a 17-year-old-female patient who had facial nerve paralysis and paraplegia due to granulocytic sarcoma as the presenting symptoms of acute myeloid leukemia.

Therapeutic effect of tirilazad mesylate (U-74006F), mannitol, and their combination on experimental ischemia.

The effect of tirilazad mesylate (U-74006F), mannitol, and their combination was investigated on focal cerebral ischemia induced by permanent middle cerebral artery (MCA) occlusion in rabbits. Rabbits were divided into four groups receiving vehicle, U-74006F, mannitol, and U-74006F plus mannitol. Hematocrit (hct), glucose, mean arterial blood pressure (MABP), pH, PCO2, and PO2 were measured both before and after occlusion. Seventy-two hours following the permanent MCA occlusion, the neurological outcome was assessed and a quantitative neuropathologic examination was performed in all rabbits. The neurological outcome was better in the rabbits treated with U-74006F plus mannitol than in the other groups. The size of infarction of the affected hemisphere following MCA occlusion was 49.7% in the control group, 30.6% in the U-74006F group, 47.6% in the mannitol group, and 24.1% in the U-74006F plus mannitol group. There was a statistically significant reduction in infarct size in the U-74006F plus mannitol group compared with the other groups (P < 0.05). The ratio of ischemic neurons to total neurons in the cortex was smaller in the U-74006F plus mannitol group than in the other groups. The ratio of ischemic neurons to total neurons in the subcortex was significantly lower in the U-74006F plus mannitol group than in the other groups (P < 0.05). Our data provide evidence for the beneficial effects of both U-74006F and U-74006F plus mannitol in promoting neurological recovery and preservation of the ischemic area.

Detection of intestinal bacterial translocation in subclinical ischemia-reperfusion using the polymerase chain reaction technique.

BACKGROUND/PURPOSE: The purpose of this study was to evaluate the detection of bacterial translocation after subclinical ischemia reperfusion injuries in rats with the polymerase chain reaction (PCR) technique. METHODS: Six-week-old weaning rats were divided into 3 groups. (1) Experiment rats (n = 20) were gavaged with 10(10) Escherichia coli followed by superior mesentery artery occluded for 10 minutes, then reperfused for 30 minutes. (2) Control rats (n = 20) received bacterial gavage. (3) Group 3 were sham rats (n = 20). After the procedure, 3 mL of blood was obtained from the portal vein. The terminal ileum and mesenteric lymph node (MLN) near the terminal ileum were removed. E. coli DNA was detected in blood and MLN samples by PCR, and histological changes were examined. RESULTS: E. coli DNA detection in ischemia-reperfusion (I/R) group animals was 6 of 20 (30%) in the MLN and 2 of 20 (10%) in the blood. PCR was negative in all the rats in the control group and in the sham group (P < .05). There were no significant differences in the histological examination of rat intestines. CONCLUSION: These data suggest that subclinical intestinal I/R injury results in bacterial translocation. Also, PCR is a highly sensitive and rapid method to detect the presence of microbial DNA.

Burn scar sarcoma.

Malignancies developing in burn scars have been known for a long time and are generally epidermoid carcinomas although a few sarcoma cases have been reported. A case of fibrosarcoma developing in the burn scar was presented in this report and the literature reviewed.

A new subtype of meningioma.

Three patients with small meningiomas presented with diffuse cerebral edema that was out of proportion to the size of tumors. All lesions were small and no brain invasion or unusual tumor vascularity or dural sinus involvement was noted in any of the three cases. Tumor material was subjected to conventional and immunohistochemical stains. All three tumors showed benign meningothelial components, prominent formation of hyaline inclusions (pseudopsammoma bodies), and striking vascular mural proliferation of small dark cells. All patients have remained asymptomatic without any evidence of tumor recurrence after a follow-up of 4-6 years. These tumors showed proliferation of pericytes in blood vessel walls and, therefore, represent a new subtype of meningothelial meningioma. In the study presented here, the location, size, histotype, and clinical findings that may influence the development of peritumoral brain edema are discussed in detail.

Effects of angiotensin converting enzyme inhibitor cilazapril and angiotensin II antagonist saralasin in ovarian hyperstimulation syndrome in the rabbit.

We investigated the possible effects of the angiotensin converting enzyme (ACE) inhibitor cilazapril and angiotensin II antagonist saralasin on ovulation, ovarian steroidogenesis and ascites formation in the ovarian hyperstimulation syndrome (OHSS) in the rabbit model. OHSS was induced in rabbits by human menopausal gonadotropin (hMG) and intermittent human chorionic gonadotropin (hCG). In the cilazapril group (n = 10), animals also received cilazapril 2 mg/kg intraperitoneally daily for 7 days. In the saralasin group (n = 8), animals received saralasin intraperitoneally 1 h before or 1 h after hCG administration. Control animals (n = 8), received intraperitoneal saline solution. Serial blood samples were drawn on days 1, 5, 7 and 9 to measure serum estradiol and progesterone levels. On day 9, all rabbits underwent surgical exploration. Peritoneal and pleural fluid formation, ovarian weights and number of ovulations were determined. The volume of the ascitic and pleural fluids after hyperstimulation were not statistically different between the control, cilazapril and saralasin groups. The weight gains and ovarian weights of animals were similar between treatment and control groups. Saralasin significantly (p < 0.05) inhibited ovulation, but cilazapril did not. Cilazapril and saralasin did not affect progesterone production. Only cilazapril significantly decreased estradiol production (p < 0.05). In conclusion, the ACE inhibitor cilazapril and angiotensin II antagonist saralasin did not prevent ascites formation in OHSS. The ovarian renin-angiotensin system may not be the only factor acting in ascites formation in the OHSS.