Hepatosplenic gamma delta T-cell lymphoma associated with hepatitis B virus infection

Hepatitis B virus (HBV) infection has been implicated in the development of hepatocellular and hematopoietic malignancies. We describe a patient with chronic hepatitis B who developed hepatosplenic gamma delta T-cell lymphoma. A 45-year-old woman presented with marked hepatosplenomegaly and hepatic failure during the course of chronic hepatitis B. Peripheral blood examination revealed 57% abnormal lymphoid cells which expressed the gamma delta T-cell receptor. The cytogenetic analysis of tumor cells showed an abnormal karyotype; 47, XX, -13, +2mar in all 20 metaphases examined. A clonal rearrangement of the T-cell receptor genes was demonstrated by Southern blot analysis, showing monoclonal expansion of tumor cells. A liver biopsy specimen showed fibrosis of the portal areas and sinusoidal infiltration of tumor cells. HBV infection was documented by the presence of IgG anti-HBc and anti-HBs antibodies in serum. Although HBV-DNA was not detected in tumor cells by polymerase chain reaction analysis, there is a possibility that proliferation of gamma delta T cells in response to HBV infection played a role in the pathogenesis of hepatosplenic gamma delta T-cell lymphoma.     

Pediatric primary diffuse large cell lymphoma of bone with t(3;22)(q27;q11)

PURPOSE: A child with a primary lymphoma of bone (PLB) with a t(3;22)(q27;q11) is described. METHODS: An 11-year-old boy had a 5-week history of back pain and a destructive lesion of S1 that contained an epidural component. Histologic evaluation of a biopsy confirmed the diagnosis of diffuse large B-cell non-Hodgkin lymphoma. Karyotypic analysis disclosed a t(3;22)(q27;q11), but the amount of tumor tissue was insufficient for molecular studies of the BCL-6 gene. RESULTS: The patient remains free of disease 4 years after completion of intensive systemic chemotherapy and intrathecal chemotherapy. CONCLUSIONS: The lymphoma in the patient described in this report is highly unusual because of the coexistence of pediatric PLB and a t(3;22)(q27q11).     

Laminin inhibits A beta 40 fibril formation promoted by apolipoprotein E4 in vitro

Multidrug chemotherapy with concomitant interferon (IFN)-alpha was performed in a patient with chronic hepatitis lacking the hepatitis Be antigen and malignant lymphoma (ML). Levels of trans-aminases and DNA polymerase (DNA-P) values increased after the fifth course of chemotherapy. Therapy with IFN-alpha for 9 weeks induced a remission in liver disease and complete remission of ML was obtained with subsequent chemotherapy. It was possible to prevent severe liver damage by administering IFN immediately after the elevation of DNA-P. Early introduction of IFN may be effective in the prevention of fulminant hepatic failure from hepatitis from a precore mutant after chemotherapy.     

Large cell lymphoma complicating persistent polyclonal B cell lymphocytosis

Persistent polyclonal B cell lymphocytosis (PPBL) is a rare lymphoproliferative disorder of unclear natural history and its potential for B cell malignancy remains unknown. We describe the case of a 39-year-old female who presented with stage IV-B large cell lymphoma 19 years after an initial diagnosis of PPBL; her disease was rapidly fatal despite intensive chemotherapy and blood stem cell transplantation. Because we had recently identified multiple bcl-2/Ig gene rearrangements in blood mononuclear cells of patients with PPBL, we sought evidence of this oncogene in this particular patient: bcl-2/Ig gene rearrangements were found in blood mononuclear cells but not in lymphoma cells. Owing to the possible role of Epstein-Barr virus (EBV) in the pathogenesis of PPBL, we also hypothesized our patient might have an EBV-related lymphoproliferative disorder. Despite serologies consistent with past exposure to this virus, it was not found in lymphoma cells using a sensitive polymerase chain reaction technique. We conclude that non-Hodgkin's lymphoma may occur during the course of PPBL. However, longer follow-up in more patients will be needed in order to better clarity the risk of hematologic malignancy in patients with PPBL.     

Systemic chemotherapy alone for patients with non-acquired immunodeficiency syndrome-related central nervous system lymphoma: a pilot study of the BOMES protocol

BACKGROUND: Anecdotal reports have suggested that systemic chemotherapy with agents that better cross the blood-brain barrier may result in long term disease remission in some patients with central nervous system (CNS) lymphoma. This treatment strategy has the advantage of sparing patients the late neurologic complications from brain irradiation. METHODS: Eligible patients were required to 1) have tissue-proven and measurable non-acquired immunodeficiency syndrome (AIDS)- related primary or metastatic CNS lymphoma; 2) have normal hemogram, renal function, and hepatic function; 3) be age < or = 75 years; and 4) have provided informed consent. Patients with lymphoblastic lymphoma or patients who previously had been exposed to nitrosoureas, etoposide, or high dose methotrexate were not eligible. The systemic chemotherapy (BOMES regimen) included carmustine, 65 mg/m2/day, intravenously (i.v.) on Days 1-2; vincristine, 2 mg/day, i.v. on Days 1 and 8; methotrexate, 1.5 g/m2, i.v. on day 15 followed by leucovorin rescue; etoposide, 50 mg/m2/day, i.v. on Days 1-5; and methylprednisolone, 200 mg/day, i.v. on Days 1-7; repeated every 4 weeks (BOMES regimen). Four doses of intrathecal methotrexate were given to patients who had involvement in the cerebrospinal fluid. RESULTS: Between March 1991 and March 1997 a total of 19 patients were enrolled on the study. There were 13 men and 6 women, with a median age of 57 years. Fourteen patients had primary CNS lymphoma and 5 patients had concurrent extra-CNS lymphoma. Nine patients previously had been treated by radiotherapy (four patients), chemotherapy (three patients), or both (two patients). There were 11 complete remissions (CR) (57.9%) and 5 partial remissions (26.3%), with a total remission rate of 84.2%. One patient had had progressive brain lymphoma during systemic chemotherapy with the conventional cyclophosphamide, doxorubicin, vincristine, and prednisolone regimen, but achieved CR soon after the regimen was changed to BOMES. The median time to progression of the responders was 6 months. At last follow-up, 4 patients were alive without lymphoma at 10, 47, 64, and 66 months, respectively. There were two treatment-related deaths due to sepsis. Another two patients died of fulminant hepatitis that most likely was chemotherapy-related reactivation of chronic B viral hepatitis. CONCLUSIONS: The authors believe systemic chemotherapy alone may result in long term disease remission in some select patients with non-AIDS-related CNS lymphoma. Further investigation for better protocols is mandatory.     

Primary non-Hodgkin malignant lymphoma of the male breast

A case of primary non-Hodgkin lymphoma of the male breast is reported. The patient was a 76-year-old Japanese with a history of bilateral gynecomastia. After the patient had received sex hormone treatment for the gynecomastia, rapid growth of a tumor in the right breast was noted, with regression of a contralateral breast lesion. Clinically, inflammatory breast cancer was suspected, and right mastectomy with ipsilateral axillary lymph node dissection was performed after intraarterial infusion chemotherapy using a cis-platinum derivative. The histology of the surgical specimen was non-Hodgkin malignant lymphoma of the diffuse large cell type, with focal tumor necrosis. Immunohistochemically, the tumor cells showed a B-cell nature. The patient is currently well without disease 39 months after surgery.     

A case of T-cell malignant lymphoma developing in the chest wall of chronic pyothorax after artificial pneumothorax

A 66-year-old man, with a past history of artificial pneumothorax for pulmonary tuberculosis at the age of 17, was admitted to our hospital because of progressive left precordial pain and left anterior chest wall tumor. Because of chronic pyothorax complicated by a tumor suggested by imaging diagnosis, surgery was performed for the purpose of fenestration. A diagnosis of diffuse large cell T-cell malignant lymphoma was made by the biopsy taken at surgery. Postoperatively the patient received 6 courses of combination chemotherapy with CHOP, the course turned favorable. The left precordial pain and tumor disappeared. The patient is doing well at 11 months postoperatively. Recently, cases of malignant lymphoma developing in the chest wall of chronic pyothorax have been increased and the majority of cases of the malignant lymphoma is B-cell type. T-cell type is rare. Follow-up of chronic pyothorax's patients necessarily are attentioned on the developing of the malignant lymphoma.     

肝血管肉瘤7例临床分析

Objective: The aim of our study was to evaluate and sum up the experience with hepatic angiosarcoma. Methods: Retrospective study of 7 patients with hepatic angiosarcoma. Results: Symptoms and signs such as abdominal pain were extremely nonspecific. The levels of the tumor markers were within the normal range. Four cases were positive for hepatitis B viruses. Among the 5 patients who received chemotherapy, improvement in survival was seen in 3 cases which also received surgery. Conclusion: Hepatic angiosarcoma is a rare and difficult-to-diagnose malignant liver tumor. Surgery is the definitive treatment and adjuvant chemotherapy after surgery may improve survival.     

Six-year survival after excision of cardiac malignant lymphoma

We report a patient with a postoperative survival period of 6 years after the surgical excision of a cardiac malignant lymphoma. A 35-year-old woman underwent total excision of the tumor arising from the left ventricular outflow tract. After the operation, she was treated with chemotherapy for 6 months. She has been doing well thereafter without any medication. To date there is no evidence of recurrence.     

Primary non-Hodgkin's lymphoma of the liver

Primary non-Hodgkin's lymphoma of the liver is an extremely rare lymphoma subset that often presents with diagnostic difficulties to both clinicians and pathologists. Using MEDLINE search, 90 cases of primary hepatic lymphomas reported in the literature were reviewed. The epidemiology and etiology, clinical presentation, pathologic features, management, and outcome of these patients have been summarized and described. Results of this review show that middle-aged males are most often affected. Abdominal pain or discomfort, weight loss and fever are the most frequent presenting symptoms. Most cases have a solitary or multiple mass lesions in the liver, and are frequently misdiagnosed as having a primary liver tumor or metastatic cancer. Diffuse large cell lymphoma is the most commonly encountered histologic subtype. Surgery, chemotherapy and radiotherapy have been used alone or in combination as treatment but the outcome is generally poor. Although primary hepatic lymphoma is an aggressive disease, it is resectable, and responsive to chemotherapy and radiotherapy. Because of the profound therapeutic implications, it should be considered in the differential diagnosis for patients presenting with mass lesions in the liver or hepatic disease.     

Primary hepatic high-grade non-Hodgkin's lymphoma and chronic hepatitis C infection

Little is known about the coincidence of hepatitis C virus infection (HCV) and non-Hodgkin's lymphoma, although there is an increased incidence of chronic HCV infection with cryoglobulinemia type II and, interestingly, low-grade non-Hodgkin's lymphoma (NHL) in a few patients. We therefore report on a 74-year-old white male with known chronic hepatitis C virus infection who was admitted to the clinic due to weight loss and pain in the right upper quadrant. Ultrasound examination was performed for suspected hepatocellular carcinoma since a lesion in the left lobe of the liver was seen. X-ray of the lungs showed a few scattered lesions, suggestive of metastases. The ultrasound-guided fine-needle puncture revealed a high-grade malignant B-cell NHL While alpha-fetoprotein was normal, both cryoglobulin type II and the polymerase chain reaction (PCR) for HCV were positive. After six cycles of chemotherapy consisting of CHOP, the patient showed complete remission over three years. Ultimately, he died due to a sudden myeloic blast crisis. In summary, we discuss the possible etiopathologic role of the hepatitis viruses in the occurrence of non-Hodgkin's lymphoma. As we and others showed that HCV infects peripheral mononuclear blood cells (PBML), the infected PBML not only may be a source for reinfection after orthotopic liver transplantation, but also could be the cause for transformation and monoclonal propagation of lymphomatous tissue.     

Primary malignant lymphoma of the duodenum: report of a case

We present a case of long-term survival in primary malignant lymphoma of the duodenum treated with a curative surgical resection and post-operative chemotherapy. A 72 year-old man was admitted to our hospital complaining of epigastric discomfort. Endoscopy revealed an ulcerative tumor in the duodenal bulb. The tumor was diagnosed histologically as malignant lymphoma by endoscopic biopsy. A distal gastrectomy and duodenal bulb resection were performed with lymph nodes dissection. The tumor was histologically classified as B-cell phenotype, large cell, immunoblastic lymphoma of high grade, according to the Working Formulation. The patient received CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone) chemotherapy after the operation, and he has no sign of recurrence during a follow-up period of 6 years.     

Effects of systemic and regional chemotherapy after hepatic resection for colorectal metastases

BACKGROUND: Although the survival benefit of hepatic resection for colorectal metastasis has been established, some controversy remains regarding the significance of adjuvant chemotherapy after hepatic resection. METHODS: One hundred thirty-two consecutive patients who had liver resection for colorectal metastasis at our hospital between 1980 and 1997 were studied. After curative hepatic resection, 37 patients underwent systemic chemotherapy, administered orally or intravenously, and 38 patients underwent regional chemotherapy, given intra-arterially or intraportally. Forty patients had no adjuvant chemotherapy. The chemotherapeutic agents used for oral administration were uracil and Tegafur or Tegafur alone. Mitomycin C (MMC) or 5-FU was used for IV chemotherapy. Combinations of 5-FU/leucovorin or MMC/5-FU (doxorubicin) were used for regional chemotherapy. Univariate and multivariate analyses were applied to test the significance of adjuvant chemotherapy for patient survival or disease-free survival. RESULTS: Overall 5-year survival was 42.2% (95% CL: 31.2%, 53.2%). Among the possible prognostic factors studied, univariate analysis showed a significant difference in survival based on the number of tumors and lymph node metastases in the hepatic hilum. There was a significant difference in disease-free survival based on adjuvant chemotherapy and lymph node metastasis. The multivariate analysis for patient survival selected four prognostic factors (P < .05), including adjuvant chemotherapy, lymph node metastasis, disease-free interval, and tumor size. The multivariate analysis for disease-free survival selected adjuvant chemotherapy, lymph node metastasis, and disease-free interval as significant factors. The most common recurrence site was remnant liver, regardless of adjuvant chemotherapy. CONCLUSIONS: Adjuvant chemotherapy significantly improved survival and disease-free survival after hepatic resection for colorectal metastases. It did not decrease recurrence rate in the remnant liver.     

High-dose chemotherapy with autologous peripheral blood stem cell transplantation for metastatic gastric leiomyosarcoma

We report a case of metastatic gastrointestinal leiomyosarcoma treated with high-dose combination chemotherapy and autologous peripheral blood stem cell transplantation. After incomplete surgical resection, enteral, peritoneal and hepatic involvement remained. Postoperatively, standard-dose chemotherapy with etoposide, ifosfamide, cisplatin and epirubicine, and high-dose chemotherapy with the same agents (carboplatin replacing cisplatin) was given. Treatment was well tolerated and the patient remains in complete remission at 36+ months. We conclude that high-dose chemotherapy followed by autologous peripheral blood stem cell transplantation may be of use as treatment for inoperable residual disease after resection of the primary lesion in gastrointestinal and other soft tissue sarcomas.     

Primary ovarian lymphoma. A case report

A 44-year-old woman with stage IV non-Hodgkin's lymphoma (NHL) of the left ovary is described. She had an extended hysterectomy and bilateral salpingo-ophorectomy and thereafter received six courses of combination chemotherapy (cyclophosphamide, adriamycin, vincristine and predonisolone; CHOP). The patient is well with no evidence of recurrence at 12 months after surgery. The literature on primary ovarian lymphoma is reviewed.     

Vascular biology of the radial artery

Two cases with spontaneous regression of a histologically confirmed hepatocellular carcinoma (HCC) are presented. This rarely seen phenomenon of a spontaneous tumor involution is discussed and compared with the current literature. The clinical symptoms were very similar to that of a liver abscess. A 56-year-old male suffered from a multicentric, highly differentiated, trabecular HCC. First symptoms were epigastric pain, septic fever and arthritis. The tumor marker AFP was constantly normal and no hepatitis could be verified. A resection of the tumor was performed. In patient 2, a 74-year-old male, a multicentric, clear cell HCC was found. The patient had completely recovered from hepatitis type B and within the liver tissue no viruses could be identified. Clinical symptoms were mainly characterized by upper abdominal pain and septic fever. AFP was excessively elevated (3850 ng/ml) but returned to normal preoperatively. In both cases, the specimen showed a subtotal necrotic HCC with insignificant amounts of vital tumor cells. Neither patient had a liver cirrhosis macroscopically, however patient 2 had local periportal fibrosis histologically. After 24 and 41 months of follow-up, respectively, both patients are in good health     

Thought control strategies in acute stress disorder

Relapse after transplant for malignant lymphomas remains the main cause of treatment failure. Most conditioning regimens contain total body irradiation (TBI). We investigated the toxicity and efficacy of an intensified chemotherapy conditioning regimen without TBI in patients with relapsed or high-risk malignant lymphoma who had received prior radiation therapy and were therefore not eligible for TBI. Twenty patients with a median age of 38 (18-56) and relapsed or high-risk malignant non-Hodgkin's lymphoma (NHL, n = 16) or Hodgkin's disease (HD, n = 4) underwent high-dose chemotherapy consisting of busulfan (16 mg/kg), cyclophosphamide (120 mg/kg) and etoposide 30 mg/kg (n = 8) or 45 mg/kg (n = 12) followed by peripheral stem cell support (n = 14), autologous bone marrow (n = 3), allogeneic (n = 2) or syngeneic (n = 1) transplantation. All but two had chemosensitive disease before high-dose chemotherapy. The main toxicity -- according to the Bearman score -- was mucositis II in 18 (90%) patients; five patients (25%) suffered a grade I hepatic toxicity. GI toxicity I occurred in three (15%) and renal toxicity I in two patients (10%). Sixty percent of the patients developed transient dermatitis with erythema and three of them (15%) had skin desquamation; one patient experienced asymptomatic pancreatitis. Toxicity was slightly higher in patients treated with 45 mg/kg etoposide. One patient (5%) died of treatment-related venoocclusive disease. After a median follow-up of 50 months (24-84) the disease-free and overall survival were 50% and 55%. One of the nine relapsing patients developed secondary AML 18 months after transplant. High-dose busulfan, cyclophosphamide and etoposide is an effective regimen resulting in long-term disease-free survival in 50% of patients with relapsed malignant lymphoma and prior radiation therapy. The toxicity is moderate with a low treatment-related mortality (5%).     

Choledocholithiasis at the hepatic confluence mimicking a hilar cholangiocarcinoma

Adenocarcinomas of the bifurcation of the hepatic ducts (Klatskin tumors) are a relatively rare cause of obstructive jaundice. Differential diagnosis includes other neoplastic lesions, sclerosing cholangitis, Mirizzi's syndrome and benign strictures. We present a 46 year-old white female with a 2 month history of epigastric pain and progressive jaundice. Endoscopic retrograde cholangiopancreaticography (ERCP) revealed a filiform stenosis of the right hepatic duct and an obstructed left hepatic duct, an image strongly suggestive of a Klatskin tumor. The correct diagnosis was achieved, however, by percutaneous transhepatic cholangiography (PTC), which disclosed a gallstone at the common hepatic duct bifurcation and multiple small concrements in the left hepatic duct. After endoscopic removal of the gallstones in the biliary tree and laparoscopic cholecystectomy, the patient was discharged on the third post-operative day. Protuberant tumors and round biliary stones may be confused at ERCP.