Radiological Category Pediatrics Principal Modality 1 CT Principal

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Radiological Category: Pediatrics Principal Modality (1): CT Principal Modality (2): MRI Case Report #

Radiological Category: Pediatrics Principal Modality (1): CT Principal Modality (2): MRI Case Report # 0906 Submitted by: Faculty reviewer: Date accepted: Megan Speer, M. D. Abhay Srinivasan, M. D. 13 March 2012

Case History • 7 month old male with no significant past medical history presents

Case History • 7 month old male with no significant past medical history presents to the emergency room for a hard bump on his left neck. Per the mother, she first noticed the left side of his neck getting puffy 3 weeks prior. She denies fevers or redness in the area. The patient and his mother were in Mexico at the time and she took him to see the doctor. The patient was started on antibiotics, however the mother reports that 4 days into the antibiotic treatment the area got bigger and became hard. The boy still had no fever and was not bothered by the mass on his neck. She continued the antibiotics for 8 days without improvement and continued worsening of the neck mass. Per the mother, the patient has not had any change in appetite or in behavior.

Radiological Presentations Image 1: Axial CT image of the neck Image 2: Coronal CT

Radiological Presentations Image 1: Axial CT image of the neck Image 2: Coronal CT image of the neck

Radiological Presentations Image 3: T 1 MRI Axial Image 4: T 2 MRI Axial

Radiological Presentations Image 3: T 1 MRI Axial Image 4: T 2 MRI Axial Image

Radiological Presentations Image 5: T 1 Post Contrast MRI Axial Image

Radiological Presentations Image 5: T 1 Post Contrast MRI Axial Image

Test Your Diagnosis Which one of the following is your choice for the appropriate

Test Your Diagnosis Which one of the following is your choice for the appropriate diagnosis? After your selection, go to next page. • Rhabdomyosarcoma • Malignant extra-renal rhabdoid tumor • Neuroblastoma

Findings and Differentials Findings: Images 1 and 2: Large, lobulated heterogeneous enhancing left-sided neck

Findings and Differentials Findings: Images 1 and 2: Large, lobulated heterogeneous enhancing left-sided neck mass extends from the submandibular region to the level of the clavicle. The mass has lobular margins and internal areas of hypodensity. The internal carotid artery and the trachea are displaced medially and the internal jugular vein is compressed without evidence of invasion. Images 3 -5: Partially hemorrhagic and necrotic, enhancing 8. 2 x 6. 2 x 5. 8 cm mass lesion arising from the left carotid space with evidence of some encasement of branches of the left external carotid artery. The mass involves the left mandibular, sublingual and left parapharyngeal spaces. Differentials: • Rhabdomyosarcoma • Malignant extra-renal rhabdoid tumor • Neuroblastoma

Discussion Rhabdomyosarcoma is the most common soft tissue sarcoma in children. The tumor is

Discussion Rhabdomyosarcoma is the most common soft tissue sarcoma in children. The tumor is a malignant neoplasm of striated muscle and may occur anywhere in the body. The most common sites are the head and neck, extremities and genitourinary tract. Metastases are found predominantly in the lungs, bones, lymph nodes, and brain. The majority of patients present younger than 15 years and rhabdomyosarcoma rarely affects adults. Symptoms usually manifest as an expanding mass, although symptoms depend on the location of the tumor. In the case of head and neck rhabdomyosacroma, involvement of the orbit may present with an orbital mass, proptosis and decreased vision. In the neck, a neck mass with pain may be seen although there is rarely airway compromise. The cause of rhabdomyosarcoma is unclear, however several genetic sydromes and environmental factors are associated with increased prevalence. Genetic syndromes include neurofibromatosis, Li-Fraumeni syndrome, and Beckwith. Weidemann syndrome.

Discussion Rhabdomyosarcoma. The best diagnostic clue is a soft tissue mass on CT with

Discussion Rhabdomyosarcoma. The best diagnostic clue is a soft tissue mass on CT with variable contrast enhancement. Osseous erosion is common but does not occur in all cases. On MRI, the mass is isointense on T 1 images and hyperintense on T 2 relative to muscle. The mass may demonstrate variable contrast enhancement on MRI.

Discussion Malignant extra-renal rhabdoid tumor. Malignant rhabdoid tumor is an uncommon childhood neoplasm that

Discussion Malignant extra-renal rhabdoid tumor. Malignant rhabdoid tumor is an uncommon childhood neoplasm that commonly occurs in the kidney. The occurrence of an extra-renal malignant rhabdoid tumor is rare, however can occur in different parts of the body including the abdomen, chest, limbs, or the brain. The concept of extrarenal malignant rhabdoid tumor has emerged due to cases with morphologic similarity to renal rhabdoid tumour in extrarenal sites. Extrarenal rhabdoid shares the distinct histology and aggressive clinical behavior of renal rhabdoid tumor. Extrarenal malignant rhabdoid tumor has a nonspecific radiological appearance, and although the diagnosis is largely based on histopathology, malignant extrarenal rhabdoid tumor should be included in the differential considerations, regardless of tumor location, when dealing with an aggressive tumor of early childhood. The treatment includes surgical debulking, chemotherapy and radiotherapy.

Discussion Histologically, malignant extra-renal rhabdoid tumor demonstrates sheets of large tumor cells which have

Discussion Histologically, malignant extra-renal rhabdoid tumor demonstrates sheets of large tumor cells which have large irregular nuclei and abundant eosinophilic cytoplasm. Mitoses are frequent and necrosis is common. These findings are seen in the adjacent photos. Immunohistochemically, these tumors are polyphenotypic and stain positive for vimentin. Malignant rhabdoid tumor also lacks INI-1 immunohistochemical staining. Photos courtesy of Kristine Konopka, M. D.

Discussion Cervical Neuroblastoma of the head and neck frequently represents metastasis from neural crest

Discussion Cervical Neuroblastoma of the head and neck frequently represents metastasis from neural crest cells in the adrenal gland, however a primary cervical location of neuroblastoma is present in 5% of cases. Infants more frequently present with thoracic and cervical tumors where older children more frequently have abdominal tumors. The lesion is usually located in the cervical lymph nodes, orbit, or skull with frequent lytic bone involvement. Extension through the intervertebral foramina with dumbbell masses is of particular importance in perivertebral space locations due to possible spinal cord compression. Neuroblastomas appear as solid, contrast-enhanced masses on both CT and MR imaging, with occasional calcifications or intralesional cysts. MR imaging can detect intraforaminal exension when present. Also, due to its high sensitivity, iodine-123 MIBG scan may help confirm the neural crest origin of cervical neuroblastoma.

Diagnosis The pathologic diagnosis in this case was consistent with extra-renal malignant rhabdoid tumor.

Diagnosis The pathologic diagnosis in this case was consistent with extra-renal malignant rhabdoid tumor.

References 1. Stat. Dx. 2. Razali bin, Ahmad, M. D. and Rahman Mohd, Abdul,

References 1. Stat. Dx. 2. Razali bin, Ahmad, M. D. and Rahman Mohd, Abdul, M. D. “Extrarenal malignant rhabdoid tumor. ” The International Medical Journal, Volume 2. 3. Armstrong, Edward. “CT of Neuroblastomas and Ganglioneuromas in Children. ” AJR September 1982, 139; 571 -576. 4. Mc. Carville, Beth M. “Rhabdomyosarcoma in Pediatric Patients. ” AJR June 2001, vol. 176, no. 6, 1563 -1569.