Extrafollicular variant of centrally located adenomatoid odontogenic tumor]

Primary intramedullary anaplastic oligodendroglioma is a rare tumor, only four cases of which have been reported. The authors present the case of a 38-month-old boy with primary intramedullary anaplastic oligodendroglioma. He underwent partial removal of the tumor and spinal radiation therapy. The residual tumor disappeared 12 months after radiation, and 48 months after treatment there was no evidence of recurrence. This case shows that in primary intramedullary anaplastic oligodendroglioma, postoperative radiation therapy confined to the spinal cord can yield an optimal result.     

Peroral pancreatoscopy for the diagnosis of pancreatic diseases

PURPOSE: The purpose of our study was to evaluate the relevance of MR mammography in the diagnosis of early and late tumor recurrence after breast-conserving therapy. METHOD: Sixty-seven patients receiving breast-conserving therapy underwent 84 MR mammographies in a period between 1 month and 14 years after end of therapy. Dynamic measurements were made following application of contrast agent. The course of signal intensity changes was evaluated in focal lesions and irradiated and contralateral glandular tissue. RESULTS: All 10 malignant lesions (7 local recurrences, 1 chest wall recurrence, 2 contralateral carcinomas) showed a > 75% increase in signal intensity within th first minute after contrast agent application. In all patients examined during the first year after end of therapy (n = 29), increased enhancement in irradiated parenchyma was observed compared with the contralateral breast, but only in two patients the increase was > 75% within the first minute. CONCLUSION: Already in the first year after end of therapy, MRI can improve diagnostic accuracy in the assessment of breast cancer recurrence. More than 12 months following end of therapy, MR mammography can demonstrate tumor recurrence with a sensitivity of nearly 100% and a specificity rising to > 90% in differentiating tumor from therapy-induced changes.     

Use of a long-acting gonadotropin-releasing hormone agonist for treatment of steroid cell tumors of the ovary

OBJECTIVE: To report a complete serologic response in a 50-year-old women who received long-acting gonadotropin-releasing hormone agonist (GnRH-A) therapy for steroid cell tumor of the ovary, not otherwise specified. DESIGN: Case report. SETTING: University hospital-based reproductive biology unit. PATIENT(S): A 50-year-old female patient exhibited persistent elevation of T (>2.0 ng/mL) after surgery for steroid cell tumor of the ovary, not otherwise specified, stage IIA for 3 months. This elevation suggested the presence of some residual active tumor. INTERVENTION(S): All tumor evaluations, including those for tumor markers, a thorough physical examination, imaging studies, and evaluations of nuclear medicine studies were negative except for elevated serum T levels. The patient was treated with GnRH-a between the fourth month and sixth month postoperatively. MAIN OUTCOME MEASURE(S): Serum levels of T and tumor survey. RESULT(S): The serum T levels returned to normal limits after administration of the first dose of GnRH-a. Follow-up of tumor survey was negative. The patient was alive and free of disease 26 months after treatment with GnRH-a. CONCLUSION(S): GnRH-a may be an alternative choice as adjuvant therapy for managing a persistent or recurrent hormone-producing steroid cell tumor of the ovary.     

Contributions of cell kill and posttreatment tumor growth rates to the repopulation of intracerebral 9L tumors after chemotherapy: an MRI study

The drought of progress in clinical brain tumor therapy provides an impetus for developing new treatments as well as methods for testing therapeutics in animal models. The inability of traditional assays to simultaneously measure tumor size, location, growth kinetics, and cell kill achieved by a treatment complicates the interpretation of therapy experiments in animal models. To address these issues, tumor volume measurements obtained from serial magnetic resonance images were used to noninvasively estimate cell kill values in individual rats with intracerebral 9L tumors after treatment with 0.5, 1, or 2 x LD10 doses of 1,3-bis(2-chloroethyl)-1-nitrosourea. The calculated cell kill values were consistently lower than those reported using traditional assays. A dose-dependent increase in 9L tumor doubling time after treatment was observed that significantly contributed to the time required for surviving cells to repopulate the tumor mass. This study reveals that increases in animal survival are not exclusively attributable to the fraction of tumor cells killed but rather are a function of the cell kill and repopulation kinetics, both of which vary with treatment dose.     

Long-term pharmacological kindling increases in vitro release of IR-Met and IR-Leu-enkephalin from amygdala

Preoperative therapy with octreotide, a long-acting somatostatin analog, suppresses GH hypersecretion, shrinks GH-producing tumors and leads to an improvement in subsequent surgical remission in acromegalic patients. A continuous infusion of octreotide has demonstrated more persistent suppression of GH secretion than intermittent injections, and only a few studies were reported on the effect of the tumor shrinkage with a continuous infusion of a small dose of octreotide. We therefore investigated the preoperative effects of small doses of octreotide (120-240 micrograms/day) administered continuously (with a subcutaneous infusion pump) over a short period (2 or 4 weeks) in nine untreated acromegalic patients. Octreotide therapy resulted in suppression of serum GH and IGF-1 concentrations in 8 out of 9 patients and reduction in pituitary tumor size measured by MRI in all patients (by 7.9 to 38.5%). In particular, considerable reduction in tumor size (more than 20%) occurred in 6 of 9 patients. In three patients assessed serially throughout the preoperative period, reduction in tumor size was noted within only one week after the start of octreotide therapy and reduction rate more than 20% was obtained within the first two weeks. In one patient, suprasellar tumor expansion totally disappeared after such therapy. Our results indicate that short-term continuous subcutaneous infusion of a small dose of octreotide results in not only inhibition of GH hypersecretion but also shrinkage of tumor size prior to surgery.     

S-100 protein: serum marker of focal brain damage after ischemic territorial MCA infarction

BACKGROUND: Wilms' tumor in an adult is rare and no treatment guidelines have been established, although all authors recommend aggressive therapy based on surgery, radiotherapy, and multiagent chemotherapy. METHODS: The authors describe a case of Wilms' tumor in a 23-year-old woman who developed hepatic and pulmonary metastases after undergoing nephrectomy. Treatment was initiated with carboplatin, etoposide, ifosfamide, and epirubicin combination chemotherapy and irradiation of the tumor bed, lungs, and liver. RESULTS: Metastatic workup was negative 41 months after suspension of chemoradiotherapy. Hematologic toxicity was high, but was manageable with adequate supportive care. CONCLUSIONS: Because this multimodal treatment, which included a chemotherapeutic regimen with single agents generally used in patients with recurrent disease, had impressive activity in this patient with an advanced adult Wilms' tumor, the issue of further investigation of this alternative schedule is raised.     

Serial MR in gene therapy for recurrent glioblastoma: initial experience and work in progress

PURPOSE: To describe the MR imaging findings in a pilot study evaluating gene therapy for treatment of patients with recurrent glioblastoma. METHODS: Serial MR examinations were evaluated retrospectively in patients treated with gene therapy that included a retroviral vector containing the herpes simplex virus thymidine kinase gene and intravenous ganciclovir. Images were obtained after tumor resection and after each cycle of treatment, at approximately 40-day intervals. The volume of enhancing tissue was measured on serial MR images. RESULTS: Eleven patients with recurrent glioblastoma were entered into the clinical trial of gene therapy and seven patients completed at least two cycles of treatment. Of these seven, three patients had an early (between 40 and 80 days) increase in the volume of enhancing tissue followed by a decrease or plateau in enhancing tissue volume. A fourth patient had a stable volume of enhancing tissue for 132 days. The remaining three patients had continuous increases in volume of enhancement on all subsequent MR examinations. CONCLUSION: Although animal data show striking tumor regression in response to similar gene therapy, only limited regression was observed among the seven patients we studied. The transient increases in enhancement seen in three of seven patients might reflect an inflammatory response to local injection of the viral vector.     

Cerebellopontine angle lymphoma: a case report and review of the literature

BACKGROUND: Although malignant lymphomas of the central nervous system have been reported to be increasing in frequency, cerebellopontine (CP) angle lymphoma is rare and only 13 cases have been reported previously in the literature. CASE PRESENTATION: A 63-year-old woman had progressive dizziness and nausea for 2 months. Computed tomography scanning and magnetic resonance imaging (MRI) revealed a mass lesion in the left CP angle, that was compressing the lateral-dorsal aspect of the pons and the fourth ventricle. This tumor was avascular on angiography. The tumor was surgically removed through a left lateral suboccipital approach. It was considered to arise from the subarachnoid space of the CP angle cistern. For some reason, the histologic diagnosis was not definitively made, and therefore radiation therapy was not planned. The tumor recurred within 50 days after the tumor excision. Surgical excision of the recurrent tumor was performed again. The histologic diagnosis was B-cell type malignant lymphoma. Radiation therapy was performed. In the 27 months since irradiation, a recurrent tumor has not been detected on MRI. CONCLUSIONS: Although erosion and expansion of the internal auditory canal suggest an acoustic neurinoma, CP angle lymphoma can, in rare circumstances, erode the internal auditory canal. There are three distinct patterns in which malignant lymphomas occupy the CP angle: (1) an extra-axial CP angle lymphoma, (2) an intra-axial lymphoma extending to the CP angle, and (3) a leptomeningeal lymphoma presenting as a CP angle lesion. Although malignant lymphomas rarely occupy the CP angle, it should be considered in the differential diagnosis of CP angle tumors. It is desirable to obtain a frozen section in all CP angle tumors during surgery to identify the tumor, because aggressive removal is not necessary, but radiation therapy should additionally be performed for malignant lymphomas.     

Usefulness of percutaneous low output microwave tissue coagulation therapy for solitary liver cancer

The possible use of percutaneous transhepatic low output microwave tissue coagulation therapy (PMCT) using ultra-sonography under local anesthesia for solitary liver cancer was studied. The subjects were 13 patients having primary or metastatic liver cancer with solitary liver tumor less than 3 cm in diameter, including 7 hepatocellular carcinomas and 6 metastatic liver cancers. PMCT was performed continuously 3 times at an output of 30 watts for 30 seconds at a time. Tumors less than 3 cm in diameter were completely coagulated by irradiation from 2 to 6 times, judging by enhanced CT. No tumor recurrence was recognized in the coagulation area. However, in two cases of metastasis from pancreatic carcinoma, multiple metastases were found at another site in the liver by 2 months after PMCT. Thus, the results suggest that PMCT is a useful therapy for small liver tumor as a local control.     

A "distant" bystander effect of suicide gene therapy: regression of nontransduced tumors together with a distant transduced tumor

Antitumor gene therapy using herpes simplex type 1 thymidine kinase (TKh) and ganciclovir (GCV) treatment has revealed an important intratumoral bystander effect. A whole tumor can be eliminated when only a fraction of its tumor cells express TKh. We now report that the bystander effect not only acts within a tumor, but also between distant tumors. One TKh+ tumor was generated simultaneously with one or multiple TKh- tumors in different rat liver lobes such that there was no contact between the resulting tumors. Both the TKh+ and the TKh- tumors regressed after GCV treatment and showed infiltration with macrophages and T lymphocytes. This distant bystander effect, which is likely immune mediated, should be of major importance for gene therapy of disseminated tumors.     

A rare case of an antenatally diagnosed tumor of the neck--a hamartoma of the thyroid gland

A case of connatal hamartoma of the left thyroid lobe is reported. During routine prenatal ultrasonographic examination, a tumor of the neck was diagnosed. The baby was born at 30 weeks' gestation, and the tumor (measuring 7.0 x 5.5 x 4.2 cm) was extirpated. Histologically, the tumor was a hamartoma of the thyroid gland. Follow-up evaluation (3 and 6 months after resection) showed that the child was doing well and had no signs of hypothyroidism or hypoparathyroidism. The case is discussed with respect to antenatal diagnosis, postpartum diagnostic measures, management, and surgical therapy.     

Possibilities and limits of surgical therapy of pseudomyxoma peritonei

In a retrospective study, the potential and limitations of surgical therapy of pseudomyxoma peritonei were studied in seven patients. In all patients the pseudomyxoma had originated from the appendix. All patients were primarily treated by surgery. An R0 resection at the first operation was possible in only one patient with a benign pseudomyxoma, while significant tumor debulking with improved symptoms was achieved in all other patients. If the tumor recurred relaparotomy was performed to obtain tumor reduction. The perioperative morbidity even after multiple relaparotomies was low. The survival rates ranged between 2 and 20 years with chemotherapy (5-fluorouracil) which was of particular prognostic benefit in patients with malignant pseudomyxoma peritonei. Surgical therapy is the treatment of choice in pseudomyxoma peritonei, although an R0 resection is hardly feasible. Due to the low morbidity, relaparotomy in cases of tumor recurrence always appears to be indicated. In comparison to other gastrointestinal malignancies, the survival rates in pseudomyxoma peritonei, sometimes treated with additive chemotherapy, are superior.     

Selective tumor kill of cerebral glioma by photodynamic therapy using a boronated porphyrin photosensitizer

The prognosis for patients with the high-grade cerebral glioma glioblastoma multiforme is poor. The median survival for primary tumors is < 12 months, with most recurring at the site of the original tumor, indicating that a more aggressive local therapy is required to eradicate the unresectable "nests" of tumor cells invading into adjacent brain. Two adjuvant therapies with the potential to destroy these cells are porphyrin-sensitized photodynamic therapy (PDT) and boron-sensitized boron neutron capture therapy (BNCT). The ability of a boronated porphyrin, 2,4-(alpha, beta-dihydroxyethyl) deuteroporphyrin IX tetrakiscarborane carboxylate ester (BOPP), to act as a photosensitizing agent was investigated in vitro with the C6 rat glioma cell line and in vivo with C6 cells grown as an intracerebral tumor after implantation into Wistar rats. These studies determined the doses of BOPP and light required to achieve maximal cell kill in vitro and selective tumor kill in vivo. The data show that BOPP is more dose effective in vivo by a factor of 10 than the current clinically used photosensitizer hematoporphyrin derivative and suggest that BOPP may have potential as a dual PDT/BNCT sensitizer.     

Endothelial cell death, angiogenesis, and microvascular function after castration in an androgen-dependent tumor: role of vascular endothelial growth factor

The sequence of events that leads to tumor vessel regression and the functional characteristics of these vessels during hormone-ablation therapy are not known. This is because of the lack of an appropriate animal model and monitoring technology. By using in vivo microscopy and in situ molecular analysis of the androgen-dependent Shionogi carcinoma grown in severe combined immunodeficient mice, we show that castration of these mice leads to tumor regression and a concomitant decrease in vascular endothelial growth factor (VEGF) expression. Androgen withdrawal is known to induce apoptosis in Shionogi tumor cells. Surprisingly, tumor endothelial cells begin to undergo apoptosis before neoplastic cells, and rarefaction of tumor vessels precedes the decrease in tumor size. The regressing vessels begin to exhibit normal phenotype, i.e., lower diameter, tortuosity, vascular permeability, and leukocyte adhesion. Two weeks after castration, a second wave of angiogenesis and tumor growth begins with a concomitant increase in VEGF expression. Because human tumors often relapse following hormone-ablation therapy, our data suggest that these patients may benefit from combined anti-VEGF therapy.     

Superselective bronchial arterial infusion therapy with cisplatin and epirubicin hydrochloride, mitomycin C-iohexol-Lipiodol emulsion (EMILE) for hilar lung adenocarcinoma: preliminary clinical experience

A case of hilar lung adenocarcinoma was treated by superselective bronchial arterial infusion therapy with cisplatin and epirubicin hydrochloride, mitomycin C-iohexol-Lipiodol emulsion (EMILE) using Tracker -18 infusion catheter. The tumor size was reduced on follow-up CT scans. However, EMILE was also distributed to nontumorous lung tissues around the tumor, and a shrinkage of the right upper lobe and elevations of the right hilus and diaphragm followed. No major complaints and clinical complications during and after the treatment occurred. This therapy was safe and effective for local tumor reduction in a case of hilar lung adenocarcinoma.     

Adenovirus-mediated combined suicide gene and interleukin-2 gene therapy for the treatment of established tumor and induction of antitumor immunity

The antitumor effect of the combined transfer of a suicide gene and a cytokine gene was evaluated in the present study. Adenoviruses expressing Escherichia coli cytosine deaminase (AdCD) and adenoviruses expressing murine interleukin-2 (AdIL-2) were utilized for the treatment of established tumors. The mice were inoculated s.c. with FBL-3 erythroleukemia cells and 3 days later received an intratumoral injection of AdCD in the presence or absence of AdIL-2 followed by intraperitoneal 5-fluorocytosine (5-FC) administration. The results demonstrated that tumor-bearing mice treated with AdCD/5-FC in combination with AdIL-2 showed more potent inhibition of tumor growth and survived much longer than did mice treated with AdCD/5-FC, AdIL-2, adenovirus expressing beta-galactosidase/5-FC or phosphate-buffered saline. The tumor mass showed obvious necrosis and inflammatory cell infiltration, and more CD4+ and CD8+ T cells infiltrating the tumor after combined therapy. The splenic natural killer and cytotoxic T lymphocyte activities increased significantly in the mice after combined therapy with AdCD/5-FC/AdIL-2. Our results demonstrate that therapy combining a suicide gene and IL-2 gene can inhibit the growth of established tumors in mice significantly and induce antitumor immunity of the host efficiently.     

Imaging of huge lingual thyroid gland with goitre

One year after a nonspecific trauma and with a history of pain of four weeks only, an osteoid osteoma of the first phalanx of the left thumb was diagnosed in a 31-year-old man. The radiologic appearance as well as a bone scan were suggestive for an osteoid osteoma. The diagnosis was confirmed histologically after resection of the tumor. As indicated in the literature, osteoid osteoma of the hand is relatively rare. The symptoms and radiologic features (osteolytic nidus and sclerosis) of osteoid osteomas are independent of the tumor location. Surgery with resection of the nidus is the only known curative therapy. The etiological role of trauma is discussed and a review of the literature is done with 15 other cases of posttraumatic osteoid osteoma having been reported.     

Potentiation of E7 antisense RNA-induced antitumor immunity by co-delivery of IL-12 gene in HPV16 DNA-positive mouse tumor

Down-regulation of oncogene expression by antisense-based gene therapy has been extensively studied, and in some cases, therapeutic effects have been demonstrated. We have previously shown that down-regulation of HPV16 E6 and E7 gene expression inhibited HPV DNA-positive C3 mouse tumor growth. Although not all of the tumor cells were transfected by pU6E7AS plasmid, complete tumor regression was achieved if the tumor size was small at the start of therapy in a syngeneic host. This suggests that some other antitumor mechanisms may be involved in addition to the direct down-regulation of HPV16 E7 oncogene expression by the antisense effect of E7AS. In the current study, we demonstrated that E7AS induces tumor cell apoptosis. More importantly, a strong antitumor immune response was elicited in the pU6E7AS-treated and tumor-regressed mice. There was no tumor growth after rechallenging the tumor-regressed mice with 1 million C3 cells. This E7AS-induced antitumor immune response was augmented by co-delivery of mIL-12 cytokine gene. The combination therapy strategy resulted in complete regression of 26 of 28 (93%) tumors. Only 12 of 31 (38%) tumors from the group treated with pU6E7AS alone and 14 of 28 (50%) tumors from the group treated with pCMVmIL-12 alone had completely regressed. Complete regression was also demonstrated in tumors located 1 cm from the treated tumors, which indicates that a systemic antitumor effect was induced by E7AS and mIL-12. Immunohistochemistry demonstrated that a significant amount of CD4+ and CD8+ cells infiltrated into tumors treated with pU6E7AS, pCMVmIL-12 and pU6E7AS+pCMVmIL-12. These data indicate that host immunity is an important factor for antisense-based gene therapy approach which can be further enhanced by combination with cytokine gene therapy.     

Adenocarcinoma combined with malignant peripheral nerve sheath tumor of the gallbladder

Adenocarcinoma of the gallbladder combined with a malignant peripheral nerve sheath tumor (MPNST) in the gallbladder in an 81-year-old woman is reported. The resected gallbladder showed two distinct tumor components, the epithelioid type of MPNST and adenocarcinoma with areas of mucin production. Although the immediate postoperative course was uneventful, a pathologic fracture of her right upper femur developed 4 months after the cholecystectomy. The pathology was determined to be a feature of metastatic MPNST rather than of adenocarcinoma. A whole body bone scan revealed multiple metastases, including the left parietal skull, left ninth rib, seventh thoracic vertebra, and right upper third of the femur. Despite cholecystectomy and postoperative irradiation therapy, she died 6 months after diagnosis of the tumor. Without an autopsy the primary site of the MPNST was unknown. We found that the prognosis was very poor in patients with distal metastatic MPNST, especially in older patients.     

Inferior vena cava filter used for unresectable renal cell carcinoma with tumor thrombi

A titanium Greenfield inferior vena cava filter was used for the treatment of 2 patients with unresectable renal cell carcinomas with tumor thrombi to prevent a fatal pulmonary embolism induced by tumor clots released during systemic interferon therapy and embolization of the primary tumor. After treatment, the size of the renal cell carcinomas at the primary site and the tumor thrombi decreased by 50%. There were no fatal pulmonary embolisms or complications related to the filter during the observation period (24 and 25 months) after therapy. This method may be useful in the prevention of a fatal pulmonary embolism induced by embolization and systemic interferon therapy in these patients.