The current status of surgical treatment for middle ear cholesteatoma in Japan is discussed based on results of a national survey conducted in reference to the “Staging and classification criteria for middle ear cholesteatoma proposed by the Japan Otological Society.” In regard to the types of cholesteatoma, the pars flaccida type was the most common, accounting for 63.4% of all cases.

As for the stage of cholesteatoma, stage II was the most common stage among patients with the pars flaccida type and tense type of cholesteatomas, whereas, stage I disease was the most frequently encountered among patients with the secondary acquired type and congenital type of cholesteatomas. For the early stages of the disease, tympanoplasty without mastoidectomy and canal wall up mastoidectomy were commonly performed.

As the disease advanced, the rate of adoption of canal wall down mastoidectomy increased. The recurrence rate of cholesteatoma was 7.1%. There were no significant differences in the recurrence rates among patients treated by different surgical procedures. The postoperative hearing success rate according to the criteria of the Japanese Otological Society was 63.0%. It is necessary, via conducting global large-scale multi-institutional research, to promote optimization and standardization of treatment for middle ear cholesteatoma.

Temporal bone lesions are rarely seen in patients with acute promyelocytic leukemia (APL) during the remission phase. We report a case of APL who showed extramedullary relapse on the tympanic membrane while in the complete remission phase. A 48-year-old male patient with APL in complete remission after the initial therapy presented with left ear fullness. Endoscopic examination revealed a mass on the left tympanic membrane, and temporal bone CT showed a mass localized on the left tympanic membrane. He was diagnosed as having extramedullary relapse of APL based on the histopathological findings of the lesion on the tympanic membrane. There were no APL cells in the blood or bone marrow. However, PCR analysis revealed the presence of PML-RARα in the bone marrow. Based on these findings, we diagnosed the patient as having molecular recurrence of APL, and administered chemotherapy. During the treatment, the ear lesion decreased steadily in size, and finally disappeared. Absence of PML-RARα in the bone marrow specimen after the treatment indicated that the patient had entered the second remission phase. Subsequently, hematopoietic stem-cell transplantation was performed, and the patient remains in remission. In a patient with a history of leukemia presenting with temporal bone lesions, extramedullary relapse should be considered in the differential diagnosis even if the patient is in the remission phase.

Glomus tumor is a benign tumor that originates from the glomus bodies in peripheral arteriovenous anastomosis sites, and is usually found in the skin of the extremities. These tumors are said to account for about 1.6% of all soft tissue tumors. We encountered a patient with a glomus tumor of the nasal septum. A 41-year-old male who presented with a history of recurrent episodes of left nasal bleeding was found to have a tumor in the left nasal cavity. Biopsy revealed the diagnosis of glomus tumor. After embolization of the feeding arteries without occurrence of massive bleeding, we successfully resected the tumor with a safety margin using an endonasal, endoscopic approach. There were no complications and the postoperative course was acceptable. Until now, four years since the surgery, there has been no evidence tumor recurrence. It is thought that embolization of the feeding arteries is an effective step for operation of the nasal sinuses in the presence of active bleeding.

A tonsillolith is derived from chronic tonsillitis. Herein, we report the case of a patient with a “giant” tonsillolith, who was treated by bilateral tonsillectomy.

A 46-year-old female patient presented to our hospital with a history of pharyngeal pain on the right side. Physical examination revealed a whitish firm mass in the right tonsil and soft palate. CT, MRI scan and biopsy were performed, and based on the findings, we diagnosed the patient as having a giant tonsillolith, and not cancer of the oropharynx, as was initially suspected. Since she had chronic tonsillitis, we selected treatment by bilateral tonsillectomy with removal of the giant tonsillolith. Her symptom resolved after the surgery.

In general, a giant tonsillolith is relatively rare, but imaging studies are useful for differentiating it from other pharyngeal diseases and for obtaining a definitive diagnosis. Irrespective of the laterality, bilateral tonsillectomy is an effective treatment for a giant tonsillolith.

In recent years, the prevalence of tuberculosis has decreased due to the development of antituberculosis drugs and promotion of tuberculosis control, and opportunities to diagnose and treat tuberculosis are rare.

Herein, we report the case of a 37-year-old female patient with tuberculosis of the tonsil, in whom multiple tests were required before the diagnosis could be established. She gave a history of having had an enlarged left tonsil from the age of 12 years. She visited an otorhinolaryngology clinic with enlarged left cervical lymph nodes, which she had first noticed 3 weeks earlier, and was referred to our department for further examination and treatment.

Histopathological examination of the tonsil showed granulomatous tonsillitis caused by acid-fast bacteria, although it was difficult to identify the species. While Mycobacterium tuberculosis could not be identified by smear tests and polymerase chain reaction assay of the tonsil, it was isolated by culture. Chest imaging did not reveal any abnormal opacities in the lung fields, and sputum culture was negative for Mycobacterium tuberculosis. Primary tonsillar tuberculosis was suspected, and the patient was treated with oral anti-tuberculosis drugs for 6 months. While her left tonsil shrank slightly, we suspected that the disease was still not well controlled. Therefore, we performed left tonsillectomy for the purpose of assessing the therapeutic effect after 4 months of treatment with antituberculosis drugs.

Histopathological examination of the resected tonsil showed no tuberculosis lesions. Smear and culture tests, as well as polymerase chain reaction assay were negative. We considered that the tuberculosis had been cured by the antituberculosis drug therapy. She has shown no signs of recurrence so far.

In cases of suspected tuberculosis, multiple tests may be needed to establish the diagnosis.

Introduction

Cardiac metastasis from head and neck malignancies is rare, and laryngeal carcinoma with cardiac metastasis is an extremely rare condition.

Case

An 83-year-old man presented with breathlessness, was diagnosed as having laryngeal cancer, stage T4aN2bM0 (Stage IVA), and was treated by total laryngectomy as the initial treatment. Curative resection had been achieved. However, after the treatment, the patient presented with signs of cardiac enlargement, a plain chest radiograph showed pleural effusion, and echocardiography and computed tomography revealed a huge right ventricular neoplasm. Cardiac lymphoma or sarcoma was suspected; however, we did not perform histopathological examination, and no treatment was initiated owing to rapid deterioration of the patient’s general condition. Laryngeal cancer with cardiac metastasis was definitively diagnosed by postmortem examination of the cardiac tissue.

Conclusion

We have presented a rare case of laryngeal carcinoma with cardiac metastasis, along with a literature review. Anatomically, cardiac metastases are more likely to occur in the right heart, and a plain chest X-ray and echocardiography are recommended in cancer patients presenting with dyspnea or pedal/generalized edema. Cytology and biopsy are necessary for diagnosis, but even when definitively diagnosed, treatment is very difficult. Possible presence of a metastatic lesion in the heart should be borne in mind during the course of treatment in patients with any malignant tumor.

Patients receiving nivolumab therapy have been reported to frequently develop colitis as an immune-related adverse event (irAE). Colitis has also been reported to occur as an adverse effect of S-1 therapy, which is the salvage chemotherapy of choice after nivolumab therapy. We report 2 cases of immune-related colitis, which were difficult to distinguish from the adverse effects of S-1 therapy.

The first case was an 86-year-old woman. Nivolumab therapy was administered for local recurrence of carcinoma of the lower gingiva. The patient showed disease progression after 6 courses of nivolumab therapy, and began to receive S-1 therapy. She developed Grade 2 colitis on the 28th day after the start of S-1 therapy. A stool culture revealed a positive result for Clostridium difficile, and the patient was diagnosed as having pseudomembranous enteritis, and appropriate antibiotic therapy was administered. However, the colitis still failed to resolve. Biopsy of the colonic mucosa showed crypt abscesses. Discontinuation of S-1 therapy did not alleviate the symptoms, whereas the initiation of prednisolone therapy on the 53rd day led to immediate improvement of the symptoms. Therefore, the symptoms were diagnosed as representing an irAE of nivolumab therapy, and not an adverse effect of S-1 therapy.

The second case was a 76-year-old man. Nivolumab therapy was administered for local recurrence of hypopharyngeal carcinoma. The patient showed disease progression after 20 courses of nivolumab therapy, and was started on S-1 therapy. The patient developed Grade 3 colitis on the 34th day, but despite discontinuation of S-1, the colitis failed to improve. Colonscopy was performed, and based on the detection of ulcerative colitis-like findings of the colonic mucosa, the patient was diagnosed as having colitis as an irAE of nivolumab. A biopsy of the colonic mucosa also showed crypt abscesses. The symptoms began to improve immediately after the start of administration of prednisolone.

Colitis is a frequently encountered adverse effect of anti-cancer agents. Since discrimination between irAEs and the adverse effects of chemotherapy is often difficult, close multidisciplinary collaboration is necessary for early diagnosis and appropriate treatment.

Herein, we report 2 cases of bone metastasis from follicular thyroid carcinoma that were treated by the excellent combination regimen of low-dose lenvatinib and normal-dose denosumab. Case 1 had been treated by total thyroidectomy, followed by three courses of radioactive iodine therapy (RAI) administered for lung metastasis. However, any effectiveness had not been shown. Seventeen years later, the patient presented with multiple bone metastases in the vertebrae and ribs. Case 2 was diagnosed as having primary follicular thyroid carcinoma that was found to be unresectable at diagnosis because of the presence of a metastasis in the femur. Both cases showed excellent suppression of tumor progression following treatment with the combination of low-dose lenvatinib and normal-dose denosumab.

Until now, no guidelines have been established for the treatment of bone metastasis from follicular thyroid carcinoma. Because all hospitals are not equipped to administer RAI therapy, molecular-targeted drugs are often selected as the first-line agents for patients with unresectable or metastatic follicular thyroid carcinoma. However, administration of lenvatinib at the usual dosage of 24 mg is often associated with various adverse effects, such as skin disorders, hypertension and proteinuria. Thus, discontinuation or reduction of the dose often becomes necessary. Therefore, we recommend initiation of lenvatinib treatment at the minimum dose of 4 mg, with gradual increase of the dose while carefully watching for the development of any adverse events. On the other hand, denosumab can be used at its usual dose, unless hypocalcemia occurs when it is administered together with lenvatinib.

Castleman disease is a lymphoproliferative disease of unknown cause that is often encountered in daily otorhinolaryngologic practice, as it also occurs in the head and neck region. We encountered a patient with Castleman disease arising in the head and neck region in whom the diagnosis was made by FDG-PET/CT. A 72-year-old man had been diagnosed as having ascending colon cancer at a nearby hospital, and an FDG-PET/CT performed before the operation for cancer revealed accumulation in the right supraclavicular fossa. Cervical ultrasonography revealed an internal hypoechoic tumor with a clear border measuring about 3 cm in size. FNA was performed twice, but both times, the results were categorized as Class II. Blood test results revealed no abnormalities, except for elevation of the serum level of soluble IL-2R. Cervical MRI showed a tumor with prominent vessels around it. The lesion was visualized as showing the same signal intensity as the muscle tissue on T1-weighted MR images. T2-weighted MR images and images obtained using the STIR sequency showed a high homogeneous signal intensity with a clear border. Diffusion-weighted imaging suggested it as a tumor with many cellular components. We performed right neck tumor resection under general anesthesia, and histopathological examination revealed hyaline-vascular type Castleman disease. Although Castleman disease is considered to have no characteristic imaging findings, it could possibly be diagnosed before surgery when it occurs as a solid unilateral lesion with a clear border and internal homogeneity, with no extranodal spread.

Oropharyngeal impalement injuries are rarely deep or complicated in children. We encountered two cases of accidental impalement injuries in children. [Case 1] A 32-month-old girl fell from a footstool with a toothbrush in her mouth. She was conscious, without any apparent neurologic or great vessel injuries. CT revealed that the toothbrush had reached the base of the middle cranial fossa. [Case 2] A 34-month-old boy fell from the chair with a fork in his mouth. Although his general condition was good, the fork had penetrated the soft palate toward the clivus. In both cases, the foreign bodies were removed in the operation room under general anesthesia. The postoperative courses in both were good. We review these cases and the management of oropharyngeal impalement injuries in children. The potentially life-threatening complications of impalement injuries in children are also discussed. We encountered two cases of accidental oropharyngeal impalement injuries in children. Both made satisfactory progress after surgical removal of the foreign bodies. Oropharyngeal impalement injuries in children are mild in most cases. But it is important to be aware of potentially serious complications of this injury and of how to appropriately manage such complications.