Therapeutic indications and metabolic effects of metreleptin in patients with lipodystrophy syndromes: Real-life experience from a national reference network.

AIM: To describe baseline characteristics and follow-up data in patients with lipodystrophy syndromes treated with metreleptin in a national reference network, in a real-life setting. PATIENTS AND METHODS: Clinical and metabolic data from patients receiving metreleptin in France were retrospectively collected, at baseline, at 1 year and at the latest follow-up during treatment. RESULTS: Forty-seven patients with lipodystrophy including generalized lipodystrophy (GLD; n = 28) and partial lipodystrophy (PLD; n = 19) received metreleptin over the last decade. At baseline, the median (interquartile range [IQR]) patient age was 29.3 (16.6-47.6) years, body mass index was 23.8 (21.2-25.7) kg/m2 and serum leptin was 3.2 (1.0-4.9) ng/mL, 94% of patients had diabetes (66% insulin-treated), 53% had hypertension and 87% had dyslipidaemia. Metreleptin therapy, administered for a median (IQR) of 31.7 (14.2-76.0) months, was ongoing in 77% of patients at the latest follow-up. In patients with GLD, glycated haemoglobin (HbA1c) and fasting triglyceride levels significantly decreased from baseline to 1 year of metreleptin treatment, from 8.4 (6.5-9.9)% [68 (48-85) mmol/mol] to 6.8 (5.6-7.4)% [51(38-57) mmol/mol], and 3.6 (1.7-8.5) mmol/L to 2.2 (1.1-3.7) mmol/L, respectively (P < 0.001), with sustained efficacy thereafter. In patients with PLD, HbA1c was not significantly modified (7.7 [7.1-9.1]% [61 (54-76) mmol/mol] at baseline vs. 7.7 [7.4-9.5]% [61(57-80) mmol/mol] at 1 year), and the decrease in fasting triglycerides (from 3.3 [1.9-9.9] mmol/L to 2.5 [1.6-5.3] mmol/L; P < 0.01) was not confirmed at the latest assessment (5.2 [2.2-11.3] mmol/L). However, among PLD patients, at 1 year, 61% were responders regarding glucose homeostasis, with lower baseline leptin levels compared to nonresponders, and 61% were responders regarding triglyceridaemia. Liver enzymes significantly decreased only in the GLD group. CONCLUSIONS: In this real-life setting study, metabolic outcomes are improved by metreleptin therapy in patients with GLD. The therapeutic indication for metreleptin needs to be clarified in patients with PLD.

Delayed retroperitoneal liposarcoma diagnosis and management in a patient with massive obesity.

People suffering from extreme obesity may be exposed to delayed diagnosis and treatment of cancer. A 37-year-old woman (weight = 245 kg, body mass index (BMI) = 79 kg/m2), presented a sepsis associated with nonspecific abdominal pain for 4 months. After several unsuccessful attempts due to her weight and a large waist circumference, abdominal CT scan was finally successfully performed and showed a large retroperitoneal mass. An ultrasound-guided core needle biopsy was performed and was in favor of a liposarcoma. Surgery was performed to remove the entire tumor of an estimated weight of 98 kg, a giant retroperitoneal dedifferentiated liposarcoma. This case highlights the difficulties to screen, diagnose, and manage cancers encountered in patients suffering from massive obesity.

Preoperative US-guided hook-needle insertion in recurrent lymph nodes of papillary thyroid cancer: a help for the surgeon.

OBJECTIVE: The objective of this study is to investigate whether preoperative ultrasound guided insertion of a hook-needle is useful in reoperations for cervical recurrent lymph node metastases of papillary thyroid cancer. PATIENTS AND METHODS: 8 patients with operated papillary thyroid cancer were included in this study. They all had suspicious nonpalpable cervical lymph nodes discovered during follow-up. These lymph nodes were identified by ultrasound imaging and their metastatic nature was confirmed by fine needle aspiration cytology and measurement of in situ thyroglobulin. In all cases, surgical excision of these lymph nodes was decided. All 8 patients had a hook-needle inserted in the suspicious lymph node(s) preoperatively and under ultrasound guidance. RESULTS AND CONCLUSION: In all 8 patients, the suspicious lymph nodes were removed and their metastatic nature was confirmed by the final pathological examination. This localization technique is very helpful for the surgeon during the excision of small and nonpalpable lymph nodes, especially in previously operated area.