The tortoiseshell pattern in one or both sides of the submandibular glands in mucosa-associated lymphoid tissue lymphoma is related to chromosomal aberrations and the disease extent.

OBJECTIVE: Lesions of mucosa-associated lymphoid tissue (MALT) lymphoma in the submandibular glands are localized or a part of systemic involvement in association with chromosomal aberrations. This series was undertaken to investigate the sonographic features of MALT lymphoma in the submandibular glands and their relationships with chromosomal aberrations and the disease extent. METHODS: A total of 5 patients with MALT lymphoma without Sjögren syndrome in the submandibular glands were enrolled in this series. Patients underwent sonography of the submandibular glands with a high-resolution transducer before surgical biopsy of the main lesion. Sonographic characteristics of the lesions were described for their location, presence of a posterior echo, texture, and presence of an internal echo. RESULTS: Sonography in all cases showed hypoechoic and solid masses with increased posterior echo enhancement. There was an arrangement of hypoechoic small compartments demarcated by hyperechoic contour lines, which had a tortoiseshell pattern. This pattern was classified into 2 types according to its location: a lesion in the right or left side and lesions in both sides of the submandibular glands, found in 3 and 2 patients, respectively. The latter 2 cases had chromosomal aberrations of t(11;18)(q23;q23) and t(12;18)(q22;q21), respectively, and were revealed as secondary organ involvement. CONCLUSIONS: The sonographic appearance of MALT lymphoma in the submandibular glands was characterized by the tortoiseshell pattern in both primary and secondary lesions. Detection of this pattern in both sides of the submandibular glands can be an indicator of chromosomal aberrations and systematic involvement of the disease.

Clinical utility of a rapid test for uristatin.

OBJECTIVES: Uristatin is a trypsin inhibitor present in urine that is increased in most patients with bacterial or viral infections and in many with inflammatory disorders. We included the assay of uristatin as part of a screening program carried out by pediatricians on 4207 Japanese schoolchildren to judge the ability of uristatin to identify those with an infection and (or) inflammation of any cause. We used urine dipsticks for the assay of uristatin, creatinine, albumin, blood, leukocyte esterase, and protein. We also performed quantitative assays for uristatin and creatinine. Another aim was to estimate the reference range for uristatin in schoolchildren, ages 5 to 14 yr. METHODS: We prepared dipstick pads that were impregnated with a chromogenic substrate for trypsin and measured the uristatin-caused inhibition of trypsin in urine. We measured creatinine so that the ratio of uristatin to creatinine could be calculated to correct for urine concentration. RESULTS: We obtained quantitative uristatin and creatinine results for 4207 children. Of these, 177 had an abnormal urine dipstick for albumin or blood or protein or leukocyte esterase or a combination of these. We used data from 3622 children to establish the reference range for the uristatin dipsticks. The 3622 were diagnosed by their pediatricians as free from an infection or inflammation of any cause and with normal urine dipstick tests. We recommend an upper reference limit for uristatin by dipstick of < or = 7.5 mg uristatin/g creatinine. The leftover 408 children ( [4207-3622-177] = 408) fell into two groups: 205 with diagnoses of no infection, possible infection, or possible inflammatory disorders. The remaining 203 children were renal disease follow-up cases. The diagnoses were based on a physical examination, microscopic urinalysis plus urine dipstick tests for albumin, blood, creatinine, protein, leukocyte esterase and a complete blood count. In the 205 children, 46 had an abnormal uristatin dipstick test, 39 had an abnormal uristatin by immunoassay, 41 had an abnormal erythrocyte sedimentation rate (ESR), 27 had an abnormal serum C-reactive protein (CRP), and one had an abnormal urine microscopic exam. For the first 938 children in the study, the agreement was 93% of negative dipstick uristatin results and immunoassays. The agreement of positive uristatin dipsticks with immunoassays was 85%. We assumed that the immunoassay results were correct. In the evaluation of 189 children with fever, 62 also had an abnormal uristatin by dipstick. DISCUSSION: A rapid dipstick test for uristatin read on a reflectance photometer gave values that compared well with a quantitative immunoassay method. The uristatin test is sensitive but not specific for any cause of infection or inflammation. Uristatin is easy to determine and appears to be a better indicator than fever, ESR, or CRP for the diagnosis of an infection or inflammation.

A case of neonatal mature teratoma transformed to malignancy in the neck extending to the mouth floor.

A case with neonatal teratoma originating from the cervicofacial region which transformed to be malignant during treatment is reported. The case is a full-term baby girl with swallowing difficulty and has a mass at the floor of her mouth with the right neck swelling. The mass was revealed to be multi-cystic and extending deep into the sublingual space and protruding outside. Puncture and marsupialisation of the cyst could not relieve her symptom and the tumor was resected in three occasions and was diagnosed as mature teratoma without malignant component. However, three months after the last resection, the solid right neck mass enlarged rapidly and the serum alpha-fetoprotein level was elevated. Biopsied specimen demonstrated the mass to be germ cell tumor with embryonal carcinoma and yolk sac tumor component. Eight courses of JEB regimen with recurrent mass resection successfully lead to complete regression without compromising patient growth as well as cosmetics. Head and neck teratomas in children are mostly benign amenable to curative excision but its rarity and site and size of the tumor make its treatment challenging. It is important to have multi-disciplinary management for the disease from neonatal period until growth has finished. There exists a relationship between the age at diagnosis and outcome of a patient with teratoma and head and neck teratomas in neonate are mostly benign but should be removed completely as soon as the patient condition is stabilized to reduce the risk of malignant change.