Diagnosis and Treatment > Medication
Search for other papers by Tejhmal Rehman in
Google Scholar
PubMed
Search for other papers by Ali Hameed in
Google Scholar
PubMed
Search for other papers by Nigel Beharry in
Google Scholar
PubMed
Search for other papers by J Du Parcq in
Google Scholar
PubMed
Search for other papers by Gul Bano in
Google Scholar
PubMed
Summary
Beta-human chorionic gonadotropin (βhCG) is normally produced by syncytiotrophoblasts of the placenta during pregnancy and aids embryo implantation. However, it is also secreted in varying amounts in non-pregnant conditions commonly heralding a neoplastic process. We present a case of 50-year-old man, who presented with bilateral gynaecomastia with elevated testosterone, oestradiol, suppressed gonadotropins with progressively increasing levels of human chorionic gonadotropin (hCG). Biochemical and radiological investigations including ultrasonography of testes, breast tissue, MRI pituitary and CT scan full body did not identify the source of hCG. FDG PET scan revealed a large mediastinal mass with lung metastasis. Immunostaining and histological analysis confirmed the diagnosis of primary choriocarcinoma of the mediastinum. It is highly aggressive and malignant tumor with poor prognosis. Early diagnosis and management are essential for the best outcome.
Learning points:
-
High βhCG in a male patient or a non-pregnant female suggests a paraneoplastic syndrome.
-
In the case of persistently positive serum hCG, exclude immunoassay interference by doing the urine hCG as heterophilic antibodies are not present in the urine.
-
Non-gestational choriocarcinoma is an extremely rare trophoblastic tumor and should be considered in young men presenting with gynaecomastia and high concentration of hCG with normal gonads.
-
A high index of suspicion and extensive investigations are required to establish an early diagnosis of extra-gonadal choriocarcinoma.
-
Early diagnosis is crucial to formulate optimal management strategy and to minimize widespread metastasis for best clinical outcome.
Lebanese University, Hadath, Lebanon
Search for other papers by Carine Ghassan Richa in
Google Scholar
PubMed
Lebanese University, Hadath, Lebanon
Search for other papers by Khadija Jamal Saad in
Google Scholar
PubMed
Mount Lebanon Hospital, Beirut, Lebanon
Search for other papers by Georges Habib Halabi in
Google Scholar
PubMed
Mount Lebanon Hospital, Beirut, Lebanon
Search for other papers by Elie Mekhael Gharios in
Google Scholar
PubMed
Search for other papers by Fadi Louis Nasr in
Google Scholar
PubMed
Mount Lebanon Hospital, Beirut, Lebanon
Search for other papers by Marie Tanios Merheb in
Google Scholar
PubMed
Summary
The objective of this study is to report three cases of paraneoplastic or ectopic Cushing syndrome, which is a rare phenomenon of the adrenocorticotropic hormone (ACTH)-dependent Cushing syndrome. Three cases are reported in respect of clinical presentation, diagnosis and treatment in addition to relevant literature review. The results showed that ectopic ACTH secretion can be associated with different types of neoplasm most common of which are bronchial carcinoid tumors, which are slow-growing, well-differentiated neoplasms with a favorable prognosis and small-cell lung cancer, which are poorly differentiated tumors with a poor outcome. The latter is present in two out of three cases and in the remaining one, primary tumor could not be localized, representing a small fraction of patients with paraneoplastic Cushing. Diagnosis is established in the setting of high clinical suspicion by documenting an elevated cortisol level, ACTH and doing dexamethasone suppression test. Treatment options include management of the primary tumor by surgery and chemotherapy and treating Cushing syndrome. Prognosis is poor in SCLC. We concluded that in front of a high clinical suspicion, ectopic Cushing syndrome diagnosis should be considered, and identification of the primary tumor is essential.
Learning points:
-
Learning how to suspect ectopic Cushing syndrome and confirm it among all the causes of excess cortisol.
-
Distinguish between occult and severe ectopic Cushing syndrome and etiology.
-
Providing the adequate treatment of the primary tumor as well as for the cortisol excess.
-
Prognosis depends on the differentiation and type of the primary malignancy.
