Teratomas comprise the most common extragonadal germ cell tumors in childhood. Most teratomas involving the thyroid are benign and occur in children. However, the adult cases reported are mostly malignant and commonly arise in the thyroid. We report a case of a 31-yr-old female with a huge neck mass. Pathologic examination revealed it to be malignant teratoma composed of primitive neuroepithelial tissue with primitive neural tubes and loose myxoid to fibrous immature mesenchymal stroma. The patient underwent extensive evaluation of the thyroid gland with computed tomography (CT) scan and positron emission tomography (PET) scan, which revealed no evidence of metastatic disease. She underwent total thyroidectomy with bilateral modified radical neck dissection, intensive chemotherapy and radiotherapy. At 22-months of follow-up, the patient has remained euthyroid and showed no evidence of recurrence. This is the first case, to our knowledge, of malignant thyroid teratoma with a exuberant primitive neuroectodermal tumor component in Korea.
Adult ; Female ; Head and Neck Neoplasms/pathology ; Humans ; Neoplasm Metastasis ; Neuroectodermal Tumors, Primitive/*complications/diagnosis/*pathology ; Positron-Emission Tomography/methods ; Teratoma/*complications/diagnosis/*pathology ; Thyroid Diseases/diagnosis ; Thyroid Gland/*pathology ; Thyroidectomy ; Tomography, X-Ray Computed

BACKGROUND AND OBJECTIVEAmyloid deposition is rare. If there was a great amount of amyloid depositions in the skin tissue, it would be considered to be amyloid deposition disease at first, and then primary cutaneous marginal zone B-cell lymphoma (PCMZL). This study was to analyze the diagnosis and differential diagnosis of two cases of PCMZL with amyloid deposition.

METHODSClinicopathologic characteristics and follow-up of two cases of PCMZL were analyzed. Immunohistochemical staining was performed by EnVision method using antibodies LCA, CD19, CD20, CD79a, CD3, CD7, MUM1, kappa, lambda, Ki-67. IgH and TCRgamma gene rearrangement was detected by polymerase chain reactive (PCR).

RESULTSCase 1, a 71-year-old Chinese male, had a subcutaneous mass on the right elbow that was initially diagnosed with "amyloidosis" in 2004. Three years after the initial diagnosis, he developed recurrences on the right para-auxillary that was still diagnosed with "probably amyloidosis". Four years after the first diagnosis, the patient presented a lesion on the right para-auxillary with a diameter of 2 cm and a lesion on the temporal-parietal dural with a size of 6.0 cmx3.0 cmx3.0 cm. Case 2, a 68-year-old Chinese male, had a subcutaneous mass next to back of the left ear with a size of 9.0 cmx5.0 cm, and he underwent a operation one year previously because of subcutaneous mass in the same site. Microscopically, the tumors of both cases were located in dermis and subcutaneous, tumor cells were medium size with a nodular or diffuse distribution, and some of tumor cells were plasmacytoid/plasma cells. Morphologically, the temporal-parietal dural lesion was similar to subcutaneous lesion and infiltrated into cranial (case 1). Juxtaposed the tumor cells of two cases, there were the large amyloid deposits of amorphous hyaline material and concentrically laminated hyaline spherules in case 1, while cord-like amyloid deposits in case 2. Reactive lymphoid follicles with germinal centers and foreign body giant cells in the stroma were found surrounding the amyloid deposits. Congo red staining showed positive of amyloid deposition in tumor tissues of both cases. Immunohistochemical staining revealed that LCA, CD19, CD20, CD79a and MUM1 expressions were positive in tumor cells, and Ki-67 expression was about 8%-10%. IgL restricted expression as kappa positive while lambda negative was found in both cases. PCR results showed monoclone gene rearrangement of IgH gene in both cases.

CONCLUSIONSOur findings suggest that amyloid deposition rarely present in both primary and metastatic tumors in PCMZL, and its diagnosis should be considered to avoid misdiagnosis. The patients with PCMZL should undergo regular examinations and chemotherapy as well as a long-term follow-up since it is apt to recur or relapse.

Aged ; Amyloidosis ; complications ; drug therapy ; metabolism ; pathology ; surgery ; Antigens, CD ; metabolism ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Cyclophosphamide ; therapeutic use ; Diagnosis, Differential ; Doxorubicin ; therapeutic use ; Elbow ; Follow-Up Studies ; Head and Neck Neoplasms ; complications ; drug therapy ; metabolism ; pathology ; surgery ; Humans ; Interferon Regulatory Factors ; metabolism ; Leukocyte Common Antigens ; metabolism ; Lymphoma, B-Cell, Marginal Zone ; complications ; drug therapy ; metabolism ; pathology ; surgery ; Magnetic Resonance Imaging ; Male ; Neoplasm Recurrence, Local ; Prednisone ; therapeutic use ; Skin Neoplasms ; complications ; drug therapy ; metabolism ; pathology ; surgery ; Vincristine ; therapeutic use

PURPOSE: In treating schwannoma patients, it is critical to determine the origin of the tumor to preserve nerve function. We evaluated the validity of preoperative imaging studies in distinguishing the neurological origin of the schwannomas of the head and neck, and the efficacy of intracapsular enucleation in preserving nerve function. MATERIALS AND METHODS: In 7 cases of schwannomas in the head and neck region, we predicted whether the tumor originated from the vagus nerve or the cervical sympathetic chain through imaging studies including computed tomography (CT) and magnetic resonance imaging (MRI). All patients were performed intracapsular enucleation, and the function of the vagus nerve and the sympathetic nerve was evaluated preoperatively and postoperatively. RESULTS: Preoperative imaging studies showed 6 cases where the tumor was located between the carotid artery and the internal jugular vein, and 1 case where the tumor was located posteriorly, displacing the carotid artery and the internal jugular vein anteriorly. At the time of operation, we confirmed schwannoma originating from the vagus nerve on the first 6 cases, and schwannoma originating from the sympathetic nervous system on the last case. All patients went through successful intracapsular enucleation, and of the seven schwannoma cases, 6 patients maintained normal postoperative neurological function (85.7%). CONCLUSION: Preoperative imaging studies offer valuable information regarding the location and origination of the tumor, and intracapsular enucleation helped us to preserve the nerve function.
Aged ; Diagnostic Imaging/methods ; Female ; Follow-Up Studies ; Head and Neck Neoplasms/complications/diagnosis/*pathology ; Humans ; Magnetic Resonance Imaging/methods ; Male ; Middle Aged ; Neurilemmoma/complications/diagnosis/*pathology ; Peripheral Nervous System/injuries/physiology ; Sympathetic Nervous System/physiology ; Tomography, X-Ray Computed/methods ; Treatment Outcome ; Vagus Nerve/physiology