Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease (RDD), is a rare histiocytic disorder of unknown etiology. Most patients with RDD have spontaneous remission, but in some patients, the disease recurs after complete remission and may not respond to general treatment. Some patients with RDD involving the extranodal system can have serious symptoms such as vital organ dysfunction due to mass effects, neurological symptoms caused by intracranial involvement, and respiratory distress with airway involvement. We report the case of a 7-year-old girl with severe dyspnea due to refractory extranodal RDD that caused progressive upper airway obstruction. She was admitted because of nasal congestion and persistent cervical lymphadenopathy, and diagnosed as having RDD by cervical lymph node incisional biopsy. The initial prednisone treatment did not improve her symptoms. The following contrast-enhanced neck computed tomography revealed a newly developed airway mass protruding in the upper trachea. After 8 weeks of chemotherapy with vinblastine, methotrexate, and prednisone, complete remission was attained. Seven months after chemotherapy cessation, the disease recurred, and chemotherapy with vincristine, cytarabine, and prednisone was resumed. Despite the chemotherapy and emergency radiotherapy, no improvement was observed in the cervical lymph node enlargement and airway obstructive symptom due to the upper tracheal mass. 2-Chlorodeoxyadenosine (cladribine) therapy was initiated, and the patient got complete remission after 6 cycles of the cladribine treatment and maintained no evidence of disease for 2 years. We suggest that cladribine is an effective treatment option for recurrent/refractory RDD.
Airway Obstruction ; Biopsy ; Child ; Cladribine ; Cytarabine ; Drug Therapy ; Dyspnea ; Emergencies ; Estrogens, Conjugated (USP) ; Female ; Histiocytosis, Sinus ; Humans ; Lymph Nodes ; Lymphatic Diseases ; Methotrexate ; Neck ; Prednisone ; Radiotherapy ; Remission, Spontaneous ; Trachea ; Vinblastine ; Vincristine

Primary rectal lymphoma is a rare disease among the gastrointestinal (GI) lymphoma. In particular, diffuse large B-cell lymphoma (DLBCL) transformed from mucosa-associated lymphoid tissue (MALT) lymphoma is often the primary type of GI lymphoma, mostly in stomach or duodenum, but has never been reported in rectum. Here we report an unusual case in which a 75-year-old male patient diagnosed with DLBCL transformed from MALT lymphoma in the rectum. The patient was diagnosed as rectal DLBCL transformed from MALT lymphoma as Lugano stage II2 and was treated with chemotherapy (R-CHOP) with CD-20 monoclonal antibody (rituxaimb). Complete remission of multiple lymphadenopathy and mass forming ulcer of the rectum was achieved after 6 cycles of R-CHOP. He has been free from disease for 12 months.
Aged ; B-Lymphocytes* ; Drug Therapy ; Duodenum ; Humans ; Lymphatic Diseases ; Lymphoid Tissue ; Lymphoma ; Lymphoma, B-Cell* ; Lymphoma, B-Cell, Marginal Zone* ; Male ; Rare Diseases ; Rectum ; Stomach ; Ulcer

Clear cell adenocarcinoma of the female urethra is extremely rare. Because the primary urethral carcinomas are rare, standard diagnostic algorism and management has not been established yet. We report a case of clear cell adenocarcinoma of the proximal urethra in a 76-year-old female. She was presented with voiding difficulty for several years after pubovaginal sling operation due to stress urinary incontinence twelve years ago. Before this period, she had managed with medication for detrusor hypocontractility. Recently she complained bloody vaginal discharge, and her vaginal examination revealed palpable mass on the anterior vaginal wall. Pelvic MRI showed a 5×4.5cm sized tumor surrounding the proximal urethra which was mimicking prostate with no lymphadenopathy. Biopsy of the mass confirmed it to be clear cell adenocarcinoma. She underwent anterior pelvic exenteration and ileal conduit with bilateral pelvic lymph node dissection. The patient received adjuvant radiotherapy and chemotherapy as treatment. We should carefully evaluate patients with voiding symptom after anti-incontinence surgery and multimodal treatment should be applied for locally advanced urethral carcinoma.
Adenocarcinoma, Clear Cell* ; Aged ; Biopsy ; Combined Modality Therapy ; Drug Therapy ; Female* ; Gynecological Examination ; Humans ; Lymph Node Excision ; Lymphatic Diseases ; Magnetic Resonance Imaging ; Pelvic Exenteration ; Prostate* ; Radiotherapy, Adjuvant ; Urethra* ; Urinary Diversion ; Urinary Incontinence ; Vaginal Discharge

Hypersensitivity to mosquito bites is defined as the appearance of intense skin reactive lesions and systemic symptoms subsequent to mosquito bites. Most cases of hypersensitivity to mosquito bites reported thus far have been associated with chronic Epstein-Barr virus infection or natural killer cell leukemia/lymphoma. In this study, we describe the case of an 18-year-old Korean boy who had hypersensitivity to mosquito bites associated with primary systemic anaplastic lymphoma kinase-positive anaplastic large cell lymphoma. After a mosquito bite, the patient developed a progressive cutaneous nodule on his left lower leg and regional lymphadenopathy in the left inguinal area. The histopathological and immunohistochemical findings suggested anaplastic lymphoma kinase-positive anaplastic large cell lymphoma. Positron emission tomography-computed tomography revealed increased fluorodeoxyglucose uptake in the left T4 vertebrae, left external iliac lymph nodes, left inguinal lymph nodes, and lateral subcutaneous region of the left lower leg. According to the clinical, histopathological, and immunohistochemical findings, as well as the imaging data, the patient was diagnosed with primary systemic anaplastic lymphoma kinase-positive anaplastic large cell lymphoma. Consequently, the patient received a total of 6 cycles of cyclophosphamide + doxorubicin + vincristine + prednisolone chemotherapy at 3-week intervals, after which the lesions regressed.
Adolescent ; Culicidae* ; Cyclophosphamide ; Doxorubicin ; Drug Therapy ; Electrons ; Herpesvirus 4, Human ; Humans ; Hypersensitivity* ; Killer Cells, Natural ; Leg ; Lymph Nodes ; Lymphatic Diseases ; Lymphoma* ; Lymphoma, Large-Cell, Anaplastic* ; Male ; Prednisolone ; Skin ; Spine ; Vincristine

A 52-year-old man was presented with a huge left testicular mass and palpable cervical lymphadenopathy with retroperitoneal lymph node enlargement on an abdominal computed tomography. A left radical orchiectomy and an ultrasound-guided neck node biopsy were performed. A pathological examination revealed spermatocytic seminoma with extensive rhabdomyosarcomatous transformation, a condition known to be highly resistant to platinum-based chemotherapy. The patient received four cycles of etoposide, ifosfamide and cisplatin (VIP) chemotherapy. A repeat computed tomography revealed a substantial regression consistent with a partial response. Retroperitoneal lymph node dissection was attempted, which revealed rhabdomyosarcoma; however, complete microscopic resection was not achieved. After surgery, the residual abdominal lymph node progressed and salvage paclitaxel, ifosfamide and cisplatin (TIP) chemotherapy was employed, which again achieved a partial response. Here, we present a first case report of a spermatocytic seminoma with extensive rhabdomyosarcomatous transformation and multiple metastatic lymphadenopathies that showed a favorable response to platinum-based systemic chemotherapy.
Biopsy ; Cisplatin ; Drug Therapy ; Drug Therapy, Combination* ; Etoposide ; Humans ; Ifosfamide ; Lymph Node Excision ; Lymph Nodes ; Lymphatic Diseases ; Middle Aged ; Neck ; Orchiectomy ; Paclitaxel ; Radiotherapy ; Rhabdomyosarcoma ; Seminoma*

BACKGROUNDBenign lymphoepithelial lesion (BLEL) is characterized by symmetric bilateral swelling of the lacrimal and salivary glands and considered a subtype of immunoglobulin G4 (IgG4)-related sclerosing disease, the etiology and pathogenesis of which has not been determined. The purpose of the present study was to analyze the clinical features of BLEL in the lacrimal gland and the relationship between the serum level of IgG4 and BLEL.

METHODSTwenty consecutive patients with BLEL diagnosed in Department of Ophthalmology at Beijing Tongren Hospital, Capital Medical University between January 2012 and December 2013 were observed. The clinical features, imaging findings, laboratory tests, treatments and follow-up status of these 20 consecutive patients were analyzed.

RESULTSIn all 20 patients, the ratio of male to female was 1:4, the ages ranged from 28 to 57 years, the ratio of unilateral to bilateral eyes involvement was 1:4, and painless uncongested symmetrical swelling of the upper eyelid was the main clinical manifestation. Orbital magnetic resonance imaging (MRI) showed that all patients involved lacrimal gland, which were obviously enlarged with equal signals in T1W images and T2W images and obvious enhancement on contrast MRI. Extraocular muscles were involved in 5 patients, salivary gland in 8 patients, and frontal nerve in 3 patients. Serum IgG4 concentration was elevated in 18 patients. The treatment strategy mainly included surgery and steroid administration. Three patients were lost to follow-up, 17 patients reached complete response, and no recurrence was observed.

CONCLUSIONSEyelid swelling is the typical symptom of BLEL. Most of the patients involved bilateral lacrimal glands. High serum IgG4 level and abundant IgG4-positive plasma cell infiltration are the important features, which can be found in most of BLEL patients. Surgery combined with glucocorticoids is an efficient treatment strategy.

Adult ; Female ; Glucocorticoids ; therapeutic use ; Humans ; Immunoglobulin G ; blood ; Lacrimal Apparatus ; pathology ; Lymphatic Diseases ; blood ; diagnosis ; drug therapy ; surgery ; Male ; Middle Aged

A 37-year-old woman was admitted to our hospital because of cervical and axillary lymphadenopathy that developed after delivery. An axillary lymph node biopsy revealed metastatic adenocarcinoma. Immunohistochemical staining indicated that the tumor cells expressed c-ErbB-2, but were negative for the estrogen and progesterone receptors. No definite evidence of breast cancer was detected. The patient underwent chemotherapy for suspected metastatic breast cancer. She complained of swelling in the left breast 22 months later, and a biopsy showed invasive ductal carcinoma. Here, we report a case of hormone receptor-negative occult breast cancer in a patient with cervical and axillary lymphadenopathy presenting as a cancer with an unknown primary site.
Adenocarcinoma ; Adult ; Biopsy ; Breast Neoplasms* ; Breast* ; Carcinoma, Ductal ; Drug Therapy ; Estrogens ; Female ; Humans ; Lymph Nodes ; Lymphatic Diseases ; Receptors, Progesterone

We report a case of common bile duct (CBD) cancer, successfully managed with pancreaticoduodenectomy, in a patient with isolated levocardia, malrotation, and situs ambiguous (without splenic anomalies). A 59-year-old male patient was referred to Chung-Ang University Hospital with epigastric pain and jaundice. CT and MRI revealed distal CBD cancer without significant lymphadenopathy. Multiple abdominal anatomic anomalies were identified preoperatively, whereas no anatomic anomalies were detected within the chest. The patient had a right-sided stomach and spleen, liver at the midline, several vascular variations around the celiac axis, and intestinal malrotation, but the inferior vena cava and portal vein were normal. A pancreaticoduodenectomy was performed to treat the cancer. The postoperative course was favorable, and the patient was started on combined chemotherapy and radiotherapy 15 days after the surgery. In this case study, we report that pylorus preserving pancreaticoduodenectomy was successful for distal CBD cancer in a patient with rare situs anomalies.
Axis, Cervical Vertebra ; Common Bile Duct ; Drug Therapy ; Humans ; Jaundice ; Levocardia* ; Liver ; Lymphatic Diseases ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Pancreaticoduodenectomy* ; Portal Vein ; Pylorus ; Radiotherapy ; Spleen* ; Stomach ; Thorax ; Vena Cava, Inferior

Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Castleman Disease ; immunology ; pathology ; Cyclophosphamide ; therapeutic use ; Diagnostic Errors ; Doxorubicin ; therapeutic use ; Humans ; Immunoglobulin G ; blood ; Kidney ; Lymph Node Excision ; Lymph Nodes ; pathology ; surgery ; Lymphatic Diseases ; drug therapy ; immunology ; pathology ; surgery ; Male ; Middle Aged ; Nephrectomy ; Pancreas ; Plasma Cells ; immunology ; pathology ; Prednisone ; therapeutic use ; Submandibular Gland ; Vincristine ; therapeutic use

Antitubercular Agents ; administration & dosage ; therapeutic use ; Biopsy, Needle ; methods ; Bronchi ; pathology ; Bronchoscopy ; methods ; Child, Preschool ; China ; Diagnosis, Differential ; Humans ; Lymph Nodes ; pathology ; Lymphatic Diseases ; diagnostic imaging ; pathology ; Mediastinum ; diagnostic imaging ; pathology ; Tomography, X-Ray Computed ; Tuberculosis, Pulmonary ; diagnostic imaging ; drug therapy ; pathology