Pinar Sisman

Adrenocortical carcinoma: Single center experience

Objective Adrenocortical carcinoma is an aggressive endocrine malignancy with an annual incidence of 0.5-2 cases per million. The most important factors that determine prognosis are tumor stage at the time of diagnosis and the success of surgery. However, advanced age, large tumor size, hormone secretion, high Ki-67 index (>10%), tumor necrosis and high mitotic activity are other factors associated with poor prognosis. In the present study, we aimed to evaluate the contribution of the patient and treatment- related factors to the prognosis in adrenocortical carcinoma. Material and methods We included 15 adrenocortical carcinoma patients who were followed in our center between 2005 and 2015. The effects of age, gender, tumor size, type of operation, postoperative resection status and adjuvant treatment on disease-free survival and overall survival were analyzed. Results Disease-free survival was 23.32±3.69 months and overall survival was 36.60±10.78 months. Gender, tumor size, tumor stage, type of operation, hormonal activity, presence of necrosis, recurrence and development of metastasis were not found to be associated with disease-free survival and overall survival (p>0.05). Postoperatively applied adjuvant treatments including mitotane, chemotherapy and radiotherapy did not significantly affect disease-free survival in our study, but statistically significant increase in overall survival was observed in patients getting adjuvant treatments (p=0.006). Conclusion Adrenocortical carcinoma has poor prognosis and short overall survival, and in its clinical course, recurrence and development of metastasis can be commonly observed even after complete resection of the tumor. Therefore, the patients should be evaluated carefully while determining the surgical procedure during the preoperative period, and the operation and post-operative follow-up should be performed in experienced centers. However, due to the positive effects of adjuvant treatments on survival, all patients should be evaluated postoperatively for the necessity of adjuvant treatments, especially mitotane.

Is Parenteral Levothyroxine Therapy Safe in Intractable Hypothyroidism

CASE A 32-year old woman was admitted to the hospital due to intractable hypothyroidism refractory to high dose of oral l-thyroxine therapy. She underwent total thyroidectomy and radioactive iodine therapy due to papillary thyroid cancer. After excluding poor adherence to therapy and malabsorption, levothyroxine absorption test was performed. No response was detected. Transient neurologic symptoms developed during the test. She developed 3 attacks consisting of neurologic symptoms during high dose administration. The patient was considered a case of isolated l-thyroxine malabsorption. She became euthyroid after intramuscular twice weekly l-thyroxine therapy. DISCUSSION There are a few case reports regarding isolated l-thyroxine. We report successful long term results of twice weekly administered intramuscular l-thyroxine therapy. We also draw attention to neurologic side effects of high dose l-thyroxine therapy.

An unusual case of adult-onset multi-systemic Langerhans cell histiocytosis with perianal and incident thyroid involvement

Summary Langerhans cell histiocytosis (LCH) is a rare sporadic disease characterized by histiocytic neoplastic infiltration of various organ systems and a wide spectrum of clinical manifestations, ranging from benign and self-limiting to lethal. Herein, we report a rare case of adult-onset multi-systemic LCH in a 36-year-old male patient with an initial perianal presentation and incidental finding of subsequent thyroid gland involvement in the follow-up period. The patient with a history of perianal LCH treated with surgical excision and local radiotherapy was referred to our Endocrinology Department upon detection of hypermetabolic nodular lesions in the left lateral lobe of thyroid gland on positron emission tomography–computed tomography (PET/CT) scan in the nineth month of follow-up. Current evaluation revealed euthyroid status, a hypoechoic solid lesion of 13 × 9 mm in size with irregular borders in the left thyroid lobe on thyroid USG and cytologic assessment of thyroid nodule. The patient was diagnosed with suspected, oncocytic lesion, Hashimoto thyroiditis or LCH. The patient underwent total thyroidectomy and pathological assessment confirmed the diagnosis of Langerhans cell histiocytosis. Assessments in the sixth month of postoperative follow-up revealed euthyroid status with no thyroid tissue remnants or pathological lymph node on thyroid USG. In view of the multifocal lesions indicating multi-system disease, a systemic chemotherapy protocol with combination of prednisone (PRED) and vinblastine (VBL) has been planned by the hematology department. Learning points: Langerhans cell histiocytosis (LCH) shows a wide clinical spectrum and prognosis that ranges from benign and self-limiting single-system disease (with single or multifocal lesions) to a potentially lethal multi-system disease with severe organ dysfunction and death in some cases. It has been stated that the diagnosis is often delayed in perianal LCH unless LCH is specifically considered in the etiology, despite the fact that mucosal involvement may precede systemic involvement. Our findings support the statement that most of patients with LCH were PET positive at the time of initial diagnosis, while also emphasize the inclusion of this imaging modality as a part of the diagnostic workflow as well as in the setting of treatment response evaluation among adult LCH patients.

The Treatment and Follow up of Adrenocortical Carcinoma [Adrenokortikal Karsinomlarda Tedavi Ve Izlem]

Abstract Adrenocortical carcinoma is a rare endocrine malignancy. Patients present with hormone excess or a local mass effect. The tumor is agressive and characterized by a high risk of recurrence even after complete resection. Recurrence rate (particularly local recurrence) is higher in laparoscopic adrenalectomy and most recurrences occur in the postoperative first two years. Because of the high recurrence rates, most patients need adjuvan treatment after radical resection. Three adjuvant therapotic approach have been suggested: Mitotane, mitotane plus chemotherapeutic agents and radiotherapy of the tumor bed. Adjuvant mitotane is the first line medical treatment but it is associated with some adverse events and in all patients a strict follow up is required because of the drug’s narrow therapeutic index. Adverse events are manageable through reduction of the mitotane dose. Patients with adrenocortical carcinoma should be followed up regularly at least 10 years after radical surgery. Key Words: Adrenal cancer, treatment, surgery, mitotane, chemotherapy, radiotherapy Ozet Adrenokortikal karsinom nadir gorulen bir endokrin malignitedir. Hastalar hormon asiri salinimina veya kitlenin lokal etkisine bagli semptomlar ile basvurur. Agressif bir tumordur ve nuks riski kitlenin tam rezeksiyonuna ragmen yuksektir. Basta lokal nuks olmak uzere nuks gelisme orani laparoskopik adrenalektomi sonrasi daha siktir ve ozellikle operasyon sonrasi ilk iki yil icinde gozlenir. Yuksek nuks oranlari nedeniyle cogu hasta radikal rezeksiyon sonrasi adjuvan tedaviye ihtiyac duyar. Adjuvan tedavide uc yaklasim onerilir: Mitotan, mitotan-kemoterapotik ilac kombinasyonu ve tumor yatagina radyoterapi. Adjuvan mitotan medikal tedavide birinci basamaktir. Ancak yan etkileri mevcuttur ve terapotik araliginin dar olmasi nedeniyle yakin takip gerektirir. Yan etkiler ilac dozunun azaltilmasiyla kontrol altina alinabilir. Adrenokortikal karsinomlu hastalar radikal cerrahi sonrasi en az 10 yil olmak uzere duzenli takip edilmelidir. Anahtar Kelimeler: Adrenal kanser, tedavi, cerrahi, mitotan, kemoterapi, radyoterapi