Patients with primary sclerosing cholangitis (PSC) are at increased risk of developing cholangiocarcinoma, which adversely affects their survival especially after orthotopic liver transplantation. All CT scans of patients with PSC referred to the Liver Unit at the Queen Elizabeth Hospital since 1992 were reviewed. The location of any lymph node with a short axis diameter greater than normal was documented. The incidence of lymphadenopathy and cholangiocarcinoma was also documented. 36 scans are reviewed, including eight with cholangiocarcinoma as well as PSC. Abdominal lymphadenopathy was present in 26 cases (66%) and 45 separate lymph node groups were involved in these patients. There were eight cases of cholangiocarcinoma; five were detectable on CT, but only four had significant lymphadenopathy. The remaining three cases of cholangiocarcinoma were not detectable on CT and only one of these had lymphadenopathy. Follow-up of the remaining patients has not demonstrated the development of cholangiocarcinoma. Lymphadenopathy is commonly demonstrated by CT in PSC patients, but does not imply malignancy and should not exclude a patient from undergoing liver transplantation. Conversely cholangiocarcinoma may develop without significant lymphadenopathy.
Testicular germ cell tumors (TGCTs) are the most common malignancy in males aged 20 to 39, and the incidence is increasing. TGCTs have a tendency to grow rapidly with a high risk of metastatic spread. TGCTs generally present with a palpable testicular mass, yet may present less commonly with symptoms arising from metastatic disease. A 24-year-old otherwise healthy male presented with progressive headaches. Initial imaging reported a single mass in the right frontal lobe. Complete surgical resection revealed suspicion for metastatic poorly differentiated carcinoma with an inconclusive immunohistochemical profile. Further staging scans revealed pulmonary and pelvic tumor deposits. Tumor markers with alpha-fetoprotein, beta-human chorionic gonadotropin, and lactate dehydrogenase were not elevated. Follow-up cranial magnetic resonance imaging revealed intracranial disease progression and he underwent whole brain radiation therapy. Additional outside pathology consultation for chromosomal analysis revealed features consistent with a TGCT. A scrotal ultrasound revealed a minimally atrophic right testicle. With evidence supporting the potential for response to chemotherapeutic treatment in TGCT, the patient was started on cisplatin and etoposide. Bleomycin was planned for the second cycle of chemotherapy if his pulmonary function improved. A salient feature of all invasive TGCTs is a gain in material in the short arm of chromosome 12, and is diagnostic if present. Although the initial pathology revealed a non-diagnostic metastatic tumor, further testing revealed amplification of chromosome 12p. The examination of poorly differentiated carcinomas of an unknown primary site using light microscopy and immunohistochemical profiling alone may be inadequate, and should undergo molecular chromosomal analysis.This case is presented for its unconventional presentation and rarity of occurrence. It brings forward the discussion of both the commonality of TGCT in young male adults, as well as the anomaly of a 'burned out' phenomenon. With unreliable tumor markers, nonspecific symptoms, and pathological findings, ‘burned out’ TGCTs may account for a challenging diagnosis in a variety of cases, especially with the presenting symptom arising from a less common metastatic site. This case adds to the increasing literature on a rare entity of the 'burned out' TGCT, and upon literature review, presents itself as the first reported case presenting with brain metastasis.
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