Hepatoid adenocarcinoma (HAC) is a rare but important special type of extrahepatic adenocarcinoma with clinicopathological presentation mimicking hepatocellular carcinoma (HCC), and prompt and correct diagnosis can be a challenge, especially in endemic areas with a high incidence of HCC. To date, HAC has only been reported in case series or single case reports, so we aimed to review the clinicopathological characteristics of HAC to obtain a more complete picture of this rare form of extrahepatic adenocarcinoma. All the articles about HAC published from 2001 to 2011 were reviewed, and clinicopathological findings were extracted for analysis. A late middle-aged male with high serum α-fetoprotein and atypical image finding of HCC should raise the suspicion of HAC, and characteristic pathological immunohistochemical stains can help with the differential diagnosis. Novel immunohistochemical markers may be useful to clearly differentiate HAC from HCC. Once metastatic HAC is diagnosed, the primary tumor origin should be identified for adequate treatment. The majority of HAC originates from the stomach, so panendoscopy should be arranged first.
The presenting symptoms of sinonasal lymphoma are usually similar to those of benign inflammatory diseases. Adequate amount of biopsy tissue is required for a definitive diagnosis because tumor coexisting with necrosis or inflammation is not uncommon. Therefore, the diagnosis of sinonasal lymphoma is a challenge for rhinologists. Thirty-two patients diagnosed as having sinonasal lymphoma from 1990 to 2010 in our hospital were included in this study. The presenting symptoms of these patients included nasal obstruction, rhinorrhea, bloody discharge/epistaxis, post nasal drip, facial swelling, neck mass, orbital symptoms, fever, and body weight loss. The average period between patients' awareness of their symptoms and their decision to seek medical help was 8.9 months. When they were referred to our hospital, the first impression of 20 patients (62.5%) was benign or malignant nasal neoplasm, and that of the other 12 patients (37.5%) was rhinitis or rhinosinusitis. These patients then received image studies and biopsy or surgical intervention. Most patients needed repeated biopsies, endoscopic sinus surgery, turbinectomy, or Caldwell-Luc operation for a definitive diagnosis. Their histopathologic classification included NK/T cell lymphoma (n = 13, 40.6%), peripheral T cell lymphoma (n = 12, 37.5%), and diffuse large B cell lymphoma (n = 7, 21.9%). Peripheral T cell lymphoma and NK/T cell lymphoma mostly occurred in the nasal cavity, whereas sinus involvement without nasal disease is common in B-cell lymphoma Our results reveal that patients with sinonasal NHL tend to ignore their symptoms until they become serious, and a definitive diagnosis usually requires repeat and deep biopsy.
Lymphoma-associated hemophagocytic lymphohistiocytosis (HLH) is a rare but fatal disease. Differences between B cell and T cell lymphoma-associated HLH remain unclear, specifically clinical characteristics and survival. We retrospectively analyzed 30 lymphoma-associated HLH patients from July 2004 to October 2012. Patients were divided into B cell (n = 13) and T cell (n = 17) lymphoma groups. Patients' age, performance status, presence of Epstein-Barr virus infection, international prognostic index, presence of disseminated intravascular coagulopathy, serum triglyceride, fibrinogen, and lactate dehydrogenase levels were not significantly different between B cell and T cell lymphoma groups. HLH was an indicator for treatment resistance in patients with B cell (p = 0.048), but not T cell (p = 0.217), lymphoma. Patients in the T cell lymphoma group, however, had higher serum ferritin levels than patients in the B cell lymphoma group (11,525.6 versus 3,790.6 ng/mL; p = 0.043). The median survival time for patients in the B cell and T cell lymphoma groups was 330 and 96 days, respectively. Although the difference was not statistically significant (p = 0.273), our results suggested a trend toward a better overall survival time in patients with B cell lymphoma. This survival advantage could be at least partially due to use of rituximab (p = 0.045) for the treatment of patients with B cell lymphoma. Our results also suggested that allogeneic hematopoietic stem cell transplantation could possibly provide survival benefits to T cell lymphoma-associated HLH by graft-versus-lymphoma effect.
Hemophagocytic lymphohistiocytosis (HLH), presenting with fever, cytopenia, liver dysfunction, hepatosplenomegaly, hypertriglyceridemia, and hyperferritinemia, is associated with various etiologies, including infections, collagen vascular diseases, and malignancies. The present report describes a 28-year-old woman who developed HLH combined with autoimmune hemolytic anemia (AIHA) at 23 weeks of gestation. Without response to corticosteroid, the patient completely recovered from both HLH and AIHA after termination of the pregnancy. Pregnancy-induced immune dysregulation and cytokine overproduction in genetically susceptible women may play critical roles in HLH. The differential diagnosis of pregnant women with fever and cytopenia should include HLH. Pregnancy termination should be considered when pregnancy-induced HLH is refractory to medical treatment.
To date, about 70 cases have been reported. We treated our second patient with antiestrogen therapy for 4 years and achieved good quality of life and stable disease status. However, further study on hormone therapy for ACC of Bartholin's gland is needed.
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