Primary Chest Wall Hydatid Disease: A Case Report with Multimodality Imaging Findings.

Primary chest wall hydatid cyst is a very rare disease in endemic areas. This case report describes a 22-year-old male patient with a 3-year history of chronic left chest pain. He had a history of close animal contact in childhood. Chest computed tomography (CT) scan showed a left upper posterior paravertebral cystic mass with peripheral and intrinsic calcifications. Fluorine-18 fluorodeoxyglucose (F-18 FDG) positron emission tomography (PET) scan showed no significant FDG uptake. Magnetic resonance imaging (MRI) showed a left paravertebral cystic mass with daughter cysts and a peripheral low T2 wall, compatible with hydatid disease. Medical treatment was started, and a follow-up MRI showed rupture of hydatid cysts. The patient underwent surgical resection, and a hydatid disease diagnosis was confirmed by histopathologic examination. During the postoperative hospital course, the patient developed pneumothorax which was successfully treated with a surgical procedure. The patient was discharged with medical treatment (albendazole). In conclusion, this case highlights the importance of considering hydatid disease in the differential diagnosis of chest wall cystic masses, especially in endemic regions, and the value of multimodality imaging in diagnosis and treatment planning.

Bidirectional Intraoperative Chemotherapy Using Cisplatin and Ifosfamide for Intraperitoneal Mesothelioma in Severe Renal Impairment: A Case Report.

BACKGROUND Malignant peritoneal mesothelioma (MPM) is an aggressive neoplasm with a poor prognosis. Bidirectional intraoperative chemotherapy (BDIC) using concurrent intraperitoneal and intravenous chemotherapy in combination with cytoreductive surgery (CRS) is an emerging treatment option for selected cases of MPM. It is a locoregional treatment that involves intraoperative chemoperfusion of heated chemotherapy. The administration of systemic along with intraperitoneal chemotherapy allows for a bidirectional chemotherapy gradient in peritoneal tumor cells. The aim of this treatment is eradication of microscopic residual cancer cells after major removal of macroscopic tumor nodules. To date, there is no consensus on the chemotherapeutic regimen that can be used in BDIC to manage MPM in patients with severe renal impairment. Administering intravenous ifosfamide with hyperthermic intraperitoneal cisplatin and doxorubicin is a promising regimen in treating peritoneal mesothelioma. Nephrotoxicity is a dose-limiting adverse effect of cisplatin and ifosfamide. Therefore, dose adjustment is required in patients with renal impairment. CASE REPORT In this report, we describe a 46-year-old female patient with recurrent MPM and severe renal impairment. Her treatment was managed with hyperthermic intraperitoneal cisplatin and doxorubicin along with intravenous ifosfamide following CRS. The cisplatin dose was reduced to 50% and the ifosfamide dose was reduced by 25%. The patient tolerated the procedure well, without deterioration in her renal function. At her 9-month follow-up, she did not report experiencing chemotherapy-related adverse effects, and her kidney function remained stable. CONCLUSIONS Severe renal impairment might not be a contraindication to using potentially nephrotoxic chemotherapeutic agents in CRS-BDIC.

One genotype, many phenotypes: SDHB p.R90X mutation-associated paragangliomas.

CONTEXT: SDHB p.R90X germline mutation is the most common genetic alteration in our patients with familial or apparently sporadic pheochromocytoma/paraganglioma (PPGL). OBJECTIVE: To analyze the clinical and pathological characteristics, response to therapy, and outcome of patients with SDHB p.R90X-associated PPGL and describe the clinical phenotypic variability in the patients carrying this mutation. METHODS: We reviewed the clinical and pathological characteristics and analyzed the phenotypic variability of all 13 patients that have SDHB p.R90X mutation-associated PPGL. RESULTS: Thirteen patients (five females and eight males). The median age at diagnosis was 23 years (range 8-43). Although the mutation was the same, there was significant phenotypic variability between patients and even within the same family. Four patients (30.8%) had a family history of PPGL and six patients (46%) had distant metastasis. Surgery of the primary tumor was performed in 11 patients (84.6%). Two patients had inoperable PPGL. Patients with metastasis received different combinations of chemotherapy, Lu177 radiotherapy, multikinase inhibitors, and external irradiation. Only five patients (38.5%) were in remission at a follow-up duration of 4-9 years. The other patients either died due to their disease progression (four patients, 30.8%) or continue to have progressive disease (two patients, 15.4%) or recurrence (one patient, 7.7%). Patients with distant metastasis were older, had larger primary tumors, were more likely to have a family history of PPGL and had a worse outcome. CONCLUSION: SDHB p.R90X mutation-associated PPGL have significant phenotypic variability and are associated with a high risk of distant metastasis and mortality.