Sera from patients with multiple myeloma usually display a single monoclonal immunoglobulin band on serum protein immunofixation electrophoresis. Multiple bands may be seen if the myeloma is bi- or triclonal or if the monoclonal immunoglobulin has rheumatoid factor activity. We describe a patient with light chain–predominant IgA lambda myeloma; the patient’s serum displayed 2 spatially distinct bands reacting for alpha heavy and lambda light chains. The methods used to establish monoclonality are addressed.
Although neuroblastoma is one of the most common extra-cranial tumors in the pediatric population, it is rarely seen as a metastasis to the mandibular bone. The following is a case report of a 3-year-old male who initially presented with a submandibular mass that was proven to be a poorly differentiated metastatic neuroblastoma through excisional biopsy. This report is one of the few case reports that demonstrates metastatic submandibular neuroblastoma with mandibular bone involvement in the pediatric population.
Figure 1. A-B: Biopsy of both polyps showed polypoid colonic mucosa with a lamina propria expanded by atypical clusters of spindled mast cells and prominent background eosinophilia. C: Immunohistochemistry for C-kit/CD117 confirmed a marked increase in clustered and spindled mast cells. D: CD25 was also positive in the mast cells, a marker typically positive in systemic mastocytosis. E: Cecal picture with very subtle nodularity change. F: NBI picture of nodularity change in cecum.
Conventional ossifying fibromas (OF) are benign, slow growing neoplasms, typically found in the gnathic bones, and thought to originate from the osseous mesenchyme or the periodontal ligament [1,2]. These tumors typically present in females in their third and fourth decade of life, with a propensity for the teeth-bearing areas of the mandible or maxilla [3]. Conversely, juvenile aggressive ossifying fibromas (JAOF) typically occur in the paranasal sinuses and craniofacial bones with rapid growth leading to local destruction, facial deformity, and associated complications. There are two histologic variants of JAOF: Juvenile trabecular ossifying fibroma (JTOF) and juvenile psammomatoid ossifying fibroma (JPOF). Both entities are rare in comparison to conventional ossifying fibroma and are indistinguishable on imaging. Therefore, clinical, radiologic, and pathologic correlations are required to reach the correct diagnosis. Herein, we present a case of juve-
Casestudy: Neuroblastoma is the most common solid tumor in the pediatric population. It is derived from neural crest cells and can arise from anywhere along the sympathetic chain. Fifty-to-sixty percent of patients with neuroblastoma present with disseminated disease. However, neuroblastoma rarely presents with metastasis to the submandibular gland or head and neck region. We present a case of a 3-year-old male with a one-month history of left submandibular swelling, lower back pain, fever, decreased urine output and constipation. An abdominal ultrasound demonstrated hydronephrosis. The ultrasound of the left mandible showed an echogenic mass with prominent Doppler flow. An excisional biopsy was performed on the left submandibular mass revealing small round blue cells with syncytial pattern, scant pink cytoplasm, and dark blue nuclei. Tumor cells were strongly positive for synaptophysin immunohistochemical stain. His urine vanillylmandelic acid and homovanillic acid were 121.7 and 79.3, respectively. Subsequent imaging revealed multifocal metastatic lesions involving the cervical, thoracic and lumbar vertebrae as well as the sacrum and bilateral iliac bones and posterior epidural soft tissue on MRI. While, CT scan demonstrated a right adrenal calcified mass that was presumed to be the primary malignancy site for his metastatic neuroblastoma. He was treated with chemotherapy and follow up was recommended. Although an initial presentation of neuroblastoma as a sub-mandibular mass is rare, diagnostic workup by way of radiography, bone marrow biopsy, and laboratory investigations is crucial in the accurate diagnosis, staging, and treatment of metastatic neuroblastoma.
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