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Anatomical diversity is rather typical in the biliary region. However, it has only sometimes been documented that the arteries of a hepatobiliary origin compressed the extrahepatic bile duct. Biliary obstruction may be caused by a myriad of benign and malignant diseases. Right hepatic artery syndrome (RHAS) is described as the consequence of right hepatic artery compression of the extrahepatic bile duct. We report a case of a 22-year-old male who presented with a complaint of abdominal pain and was later admitted as a case of acute calculous cholecystitis with obstructive jaundice. Abdominal ultrasound showed a picture of the so-called Mirizzi. However, A magnetic resonance cholangiopancreatography showed a picture of RHAS, so the patient needed endoscopic retrograde cholangiopancreatography to decompress the biliary system which was later performed successfully followed by cholecystectomy. The diagnosis of RHAS is well established in the literature, and it depends on the facility of the institute, cholecystectomy versus hepaticojejunostomy or endoscopic treatment alone are the management options that have been utilized to manage such cases.
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PURPOSE: Li-Fraumeni syndrome (LFS) is a rare autosomal cancer syndrome caused by a germline mutation in the TP53 gene. Breast cancer in LFS patients is of various subtypes; however, limited data are available on the clinicopathological features of these subtypes and their appropriate treatments. This study aimed to review the clinical features and treatments for breast cancer in South Korean patients with germline TP53 mutations. METHODS: Data on the clinicopathological features and treatment of all breast cancer patients with LFS were collected retrospectively from the available database of 4 tertiary hospitals in the Republic of Korea. RESULTS: Twenty-one breast cancer cases in 12 unrelated women with confirmed germline TP53 mutations were included in the study. The median age at diagnosis was 33.5 years. The histopathological diagnosis included invasive ductal carcinoma (n = 16), ductal carcinoma in situ (n = 3), and malignant phyllodes tumor (n = 2). While 42% and 31% of the cases were positive for estrogen and progesterone receptors, respectively, 52.6% were human epidermal growth factor receptor 2 (HER2) positive, and 21% were triple-negative. The treatments included mastectomy (52%) and breast-conserving surgery (38%). Five patients underwent radiotherapy (RT). The median follow-up period was 87.5 (8-222) months. There were 3 ipsilateral and 4 contralateral breast recurrences during the follow-up, and 8 patients developed new primary cancers. In the post-RT subgroup, there were 2 ipsilateral and 2 contralateral breast recurrences in 1 patient, and 4 patients had a new primary cancer. CONCLUSION: As reported in other countries, breast cancer in LFS patients in South Korea had an early onset and were predominantly but not exclusively positive for HER2. A multidisciplinary approach with adherence to the treatment guidelines, considering mastectomy, and avoiding RT is encouraged to prevent RT-associated sequelae in LFS patients.
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BACKGROUND: Primary thyroid lymphoma (PTL) is a rare disease, accounting for 5% of all thyroid malignancies. Diffuse B-cell lymphoma (DBCL) is the most common type of PTL. The diagnosis of PTL depends on biopsy results, and its management depends on the histological type. CASE PRESENTATION: A 66-year-old female complained of a huge neck mass on the right side that had started growing 3 months previously and was associated with compressive and B symptoms. She had undergone left hemithyroidectomy 20 years previously. On examination, a huge neck mass measuring 10 × 6 cm was detected on the right side that had shifted the trachea to the contralateral side. CT scanning revealed a huge soft tissue mass in the neck with compressive signs. Fine-needle aspiration (FNA) showed variably sized lymphocytes and large epithelial cells with occasional atypical cells. Tissue biopsy revealed DBCL, which is suggestive of PTL. DISCUSSION: PTL affects only the thyroid gland and the regional lymph nodes. Most PTL originate from B cells, especially DBCL, which accounts for 50-80% of all PTL. FNA may have limited capability to differentiate between anaplastic carcinoma of the thyroid and thyroid lymphoma. If FNA fails to determine PTL tissue, it should be determined using biopsy. A multidisciplinary approach is the best management technique for PTL. Radiotherapy, surgery, or both can be used for local control, while chemotherapy can be used for disseminated or hidden disease. CONCLUSION: Tissue biopsy is needed to exclude other differential diagnoses, whereas a multidisciplinary approach is needed to manage PTL.
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INTRODUCTION: Mediastinal Ectopic Thyroid Gland is a rare entity, accounting for 1% of all mediastinal tumours. Here, we present a rare case of mediastinal mass that was proved to be an ectopic thyroid with normal thyroid function tests and normal thyroid gland in the cervical location. CASE PRESENTATION: A 32-year-old lady had a road traffic accident, with the incidental discovery of a mediastinal mass on chest radiography. Thyroid function tests were normal. CT scans of the neck and chest revealed a large mediastinal mass compressing the trachea from the left side and extending to the superior part of the anterior mediastinum with normal thyroid gland in the cervical position. Midline Partial Sternotomy was done with complete surgical excision of the mass. It was well circumscribed, multinodular, had firm inconsistency, and grossly looked like a thyroid tissue. Histopathology revealed ectopic thyroid tissue negative for malignancy. Postoperative thyroid ultrasound showed normal thyroid lobes in the neck. DISCUSSION: Ectopic thyroid tissue (ETT) occurs due to failure of the thyroid gland to migrate from foramen ceacum to its normal position in the cervical region in front of the trachea. The most common site of ETT is lingual thyroid and accounts for about 90% of all cases reported in the literature. ETT in the mediastinum is very rare, with only a few cases reported in the literature. CONCLUSION: ETT in the mediastinum is very rare and can be associated with normal thyroid function and normal thyroid anatomical location. It should be differentiated from substernal goiter.
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INTRODUCTION: Hypoglossal nerve palsy (HNP) is a rare complication of airway management. Multiple factors have been postulated to contribute to its occurrence. Herein, we present a case of ipsilateral HNP following left hemithyroidectomy. CASE PRESENTATION: A 47-year-old women presented complaining of left thyroid swelling for 1 year with no symptoms of compression or hormonal impairment. Ultrasound of the neck showed a 3 × 2 cm nodule in the left thyroid lobe without lymphadenopathy. Fine-needle aspiration revealed a follicular neoplasm. Left hemithyroidectomy was carried out uneventfully. Three hours postoperatively, the patient started to complaint of dysarthria, dysphagia and odynophagia with clinical sign of tongue deviation to the left side. Head and neck CT ruled out mass effect or ischemic event, and the diagnosis of left HNP was established. Four months postoperatively, the palsy was completely resolved. Histopathology examination of the thyroid nodule showed follicular adenoma, and no further intervention was provided. DISCUSSION: Few cases of HNP are reported in the literature following oropharyngeal manipulation. Factors such as the type of surgery, position changes, and intubation characteristics have been linked to the incidence of HNP. Most of the cases recovered spontaneously, indicating a neuro-paxic type of injury. One case of HNP was reported following robotic total thyroidectomy, which was referred to as iatrogenic complication, and resulted in permanent paralysis. We believe that our case is the only reported case of transient ipsilateral HNP following a conventional left hemithyroidectomy. CONCLUSION: Strap muscles retraction and neck extension during thyroidectomy could predispose to HNP.
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INTRODUCTION: Benign Osseous metaplasia of the breast is rare, with only a few cases reported in the literature. Here we present a case of benign osseous metaplasia of the breast presenting as a breast lump. CASE PRESENTATION: 38-year-old previously well woman presented with a one-year history of bilateral breast pain and a left-sided breast lump. Ultrasound and mammography suggested calcified fibroadenoma. An ultrasound-guided true cut biopsy revealed fibrous tissue containing foci of adenosis in the presence of a myoepithelial cell layer. Excision biopsy was performed, and histopathological examination showed bone matrix deposition occupying most of the nodule with peripheral hyalinized tissue but no evidence of malignancy. A diagnosis of benign osseous metaplasia of the breast was made, and the patient recovered well without recurrence after lump excision. DISCUSSION: Only a few cases of osseous metaplasia are reported in the literature. Most reported cases are malignant, such as in fibrosarcoma, malignant mesenchymoma, osteoid sarcoma, osteogenic sarcoma, and osteochondrosarcoma.Very few cases of osseous sarcoma are reported in benign lesions such as fibroadenoma, pleomorphic adenoma, benign mesenchymoma, phyllodes tumor, and amyloid tumor of the breast. Joshi et al. first reported a case of benign osseous metaplasia of the breast presenting as breast lump in an HIV-positive patient [18]. We, therefore, consider this case to be the second case report of benign osseous metaplasia of the breast presenting as a breast lump, but the patient had no chronic illness. CONCLUSION: A breast lump can be the first presentation of benign osseous metaplasia.