Mycosis fungoides(MF) is a cutaneous T-cell lymphoma of low-grade malignancy characterized by the proliferation of small to medium-sized cerebriform lymphoid cells confined to the skin. It usually shows favorable prognosis, but morphologic transformation occurs in some cases and is often associated with a more aggressive clinical course. Herein we report a case of Ki-1 positive large cell lyrnphoma (Ki-1 LCL) which developed in the plaque stage of mycosis fungoides. Although the lesions responded well to low-dose methotrexate therapy and there was no evidence of local or systemic recurrence until now, continuous follow-up is needed because the prognosis of transformed MF is known to be poor compared with primary cutaneous Ki-1 LCL. We also discussed the differential diagnosis of primary cutaneous Ki-1 positive lymphoproliferative disorders.
Diagnosis, Differential ; Follow-Up Studies ; Lymphocytes ; Lymphoma* ; Lymphoma, T-Cell, Cutaneous ; Lymphoproliferative Disorders ; Methotrexate ; Mycosis Fungoides* ; Prognosis ; Recurrence ; Skin

Eosinophilic cellulitis is a rare dermatosis originally described by Wells as "recurrent granulomatous dermatitis with eosinophilia", then called Wells' syndrome. The etiology is unknown, although a hypersensitivity mechanism is suspected. Flame figures are considered as a characteristic histologic feature of Wells' syndrome. To clarify the nature of eosinophilic cellulitis and its flame figures, the authors have reviewed five cases of eosinophilic cellulitis with its clinical and histopathologic findings. Cutaneous lesions were variable in appearance and was confused with angioedema, urticarial vasculitis, erydiema multiforme, morphea or granuloma annulare. Microscopically, early lesions (2-7 days) showed diffuse dermal eosinophilic infiltration with widespread degranulation, sometimes extended into the underlying muscle. Subepidermal bulla was present in one case. Subsequently, granulomatous features with characteristic "flame figures" became apparent (several months). Collagen alteration by eosinophilic granules resulted in flame figure formation and a granulomatous response. In two patients, there were possible relationships between drug and flare-ups of eosinophilic cellulitis, but the others, no contributory precipitating factors were found. We think that eosinophilic cellulitis represents a severe anaphylactic hypersensitivity reaction to various stimuli showing characteristic histopathology with recurrent episodes and frequent hypereosinophilia in the peripheral blood.

A hemangioma occurred in the bony epiphysis is extremly rare. A 5-year-old boy visited to our hospital with pain and flexion contracture on the right knee. MRI showed some lesions scattered in the epiphysis of the distal femur and the proximal tibia. Biopsy specimen from the distal femoral epiphysis revealed pathologic findings compatible with hemangioma. On 8 years follow-up, the lesion in the distal femoral epiphysis had been cured, and those in the proximal tibial epiphysis were spontaneously disappeared without surgery. The scanogram shows no leg length discrepancy and angular deformity. We reports a rare case of hemangioma occurred in the bony epiphysis with the results of 8 year follow-up with the review of literatures.
Biopsy ; Congenital Abnormalities ; Contracture ; Epiphyses ; Femur ; Follow-Up Studies ; Hemangioma ; Knee ; Leg ; Preschool Child ; Tibia

Esophageal involvement by malignant lymphoma is extremely rare. A case of follicular lymphoma of the esophagus, misdiagnosed as focal lymphoid hyperplasia (pseudolymphoma) in a 72-year-old man is presented. The esophagogram revealed diffuse narrowing of the lumen in the middle and distal portion without ulceration. The resected esophagus showed mural thickening without any remarkable mucosal change. Microscopically, the esophagus showed scattered follicular lymphoid aggregates in the submucosa, extending into periadventitial fat tissue. Most follicles were devoid of germinal center and consisted of loosely aggregated small cleaved cells without atypia. The surrounding stroma of the submucosa showed dense fibrosis entrapping the infiltrating small lymphocytes in a "indian-file" appearance. There were some reactive follicles with germinal center. In the lamina propria, many plasma cells and a few eosinophils were infiltrated. The gene rearrangement study showed rearranged band for Jk probe which confirmed monoclonal B-cell nature of infiltrated small lymphoid cells. The small cleaved lymphocytes arranged in follicles were positive for L26 and bcl-2 protein. This case demonstrated the necessity of immunophenotypic and gene rearrangement study in the diagnosis of pseudolymphoma in the digestive tract.
Male ; Humans

Carcinosarcoma of the female genital tract, also called malignant mixed mullerian tumor (MMMT), is a rare and relatively aggressive tumor with various homologous and heterologous components. There have been various studies to find prognostic factors and to investigate the histogenesis, including tissue culture, electron microscopy, and immunohistochemical studies. We investigated carcinomatous, sarcomatous, and transitional areas of 6 cases of carcinosarcoma of the uterus and ovary by using epithelial and mesenchymal markers. Immunohistochemical profiles of the transitional areas were significantly different from those of carcinomatous and sarcomatous areas. Immunoreactivities for cytokeratin and epithelial membrane antigen (50% and 22.2%) were weaker than those of carcinomatous areas (95.2% and 100%), but stronger than those of sarcomatous areas (11.1% and 5.6%)(p<0.01). In transitional areas, vimentin, smooth muscle actin and S-100 protein were more strongly expressed than in carcinomatous areas, but more weakly expressed than in sarcomatous areas (p<0.01, p<0.01, and p=0.018, respectively). Myoglobin was entirely negative in carcinomatous areas and immunoreactive in minor portions of transitional and sarcomatous areas (22.2% and 16.7%, respectively). These results suggest that the transitional areas are between the carcinomatous and sarcomatous nature in differentiation, further supporting that the carcinosarcomas of the female genital tract may arise, through metaplastic change, from a type of carcinoma.
Actins ; Carcinosarcoma* ; Female* ; Humans ; Immunohistochemistry ; Keratins ; Metaplasia ; Microscopy, Electron ; Mucin-1 ; Muscle, Smooth ; Myoglobin ; Ovary ; S100 Proteins ; Uterus ; Vimentin

Primary pulmonary hemangiopericytoma is a rare, potentially malignant tumor and assumes more malignant course than that originated in the soft tissue. It can be asymtomatic until attaining a large size within the lung. We report a case of malignant hemangiopericytoma of the lung in a 29 year old man. It represented as a sharply demarcated, huge homogeneous opaque mass in the right upper lobe. The cytologic and histologic features were that of malignant hemangiopericytoma.

No abstract available.
Angioedema*

PURPOSE: To determine whether retinal nerve fiber layer (RNFL) thickness and optic nerve head parameters differ in the amblyopic and normal fellow eyes of hyperopic anisometropic amblyopic patients using spectral domain optical coherence tomography (SD-OCT). METHODS: This study included 30 patients with hyperopic anisometropic amblyopia; patient eyes were divided into 30 anisometropic amblyopic eyes and 30 normal fellow eyes. RNFL thickness, disc area, rim area, average cup-to-disc ratio, and cup volume were obtained using SD-OCT. Axial length was obtained using the IOL Master®, and the interocular differences between group were analyzed. RESULTS: Nasal quadrant RNFL thickness of amblyopic eyes was significantly thicker than that of normal fellow eyes in amblyopic patients (p = 0.010). Among optic nerve parameters, cup volume of amblyopic eyes was significantly smaller than that of normal fellow eyes (p = 0.021). No significant relationship between refractive error and RNFL thickness was observed, and a significant positive linear relationship was observed between neural rim area and RNFL thickness (rho = 0.426, p = 0.005). CONCLUSIONS: SD-OCT analysis of hyperopic anisometropic amblyopic eyes demonstrated a significant increase in nasal RNFL thickness compared to fellow non-amblyopic eyes. No optic nerve head parameters except cup volume showed significant change.
Amblyopia* ; Humans ; Nerve Fibers* ; Optic Disk ; Optic Nerve ; Refractive Errors ; Retinaldehyde* ; Tomography, Optical Coherence*

Purpose: We report a case of pseudopapilledema combined with idiopathic papilledema in a pediatric patient receiving growth hormone treatment.Case summary: An 11-year-old girl without any underlying disease presented with bilateral transient visual obscurations, a dark-adaptation disorder, and helplessness that developed while she had been on growth hormone therapy for 3 months at a local clinic. Reduced visual acuity (from 20/20 to 20/25) was observed on physical examination and bilateral optic disc edema on fundus examination, growth hormone was immediately discontinued. Four weeks later, fundus examination revealed papilledema of both eyes with mild red-green dyschromatopsia. Recombinant growth hormone-associated idiopathic papilledema combined with pseudopapilledema was suspected. Although growth hormone, had been discontinued, we scheduled further observation of the papilledema. Conclusions Papilledema was observed on fundus examination during growth hormone treatment and four weeks after discontinuation thereof. The symptoms improved, but mild papilledema combined with pseudopapilledema remained.

Purpose: We report a case of pseudopapilledema combined with idiopathic papilledema in a pediatric patient receiving growth hormone treatment.Case summary: An 11-year-old girl without any underlying disease presented with bilateral transient visual obscurations, a dark-adaptation disorder, and helplessness that developed while she had been on growth hormone therapy for 3 months at a local clinic. Reduced visual acuity (from 20/20 to 20/25) was observed on physical examination and bilateral optic disc edema on fundus examination, growth hormone was immediately discontinued. Four weeks later, fundus examination revealed papilledema of both eyes with mild red-green dyschromatopsia. Recombinant growth hormone-associated idiopathic papilledema combined with pseudopapilledema was suspected. Although growth hormone, had been discontinued, we scheduled further observation of the papilledema. Conclusions Papilledema was observed on fundus examination during growth hormone treatment and four weeks after discontinuation thereof. The symptoms improved, but mild papilledema combined with pseudopapilledema remained.