Vagotomy--a prospective, randomized study.

This is a report of a prospective randomized study of 3 operations for duodenal ulcer; parietal-cell vagotomy alone (without drainage), selective vagotomy with antrectomy, and truncal vagotomy with antrectomy. All patients in the study have been followed for a minimum of 1 yr and despite the small number of patients (total 23 in this report), there has been a statistically significant return of gastric secretory activity to preoperative levels in the parietal cell vagotomy group, while both other groups maintained decreased acid secretion at the 1-yr period. To date there has been one recurrent ulcer and one other suspected ulcer in the parietal cell vagotomy group, while both other groups have no ulcer recurrence even though morbidity has been higher (bile reflux gastritis, and so forth). This preliminary evaluation suggests strongly that extreme caution be used in applying parietal-cell vagotomy alone as a definite operation for duodenal ulcer, but because of the small number of patients involved no conclusions can be drawn at this time.     

myc family DNA amplification in 107 tumors and tumor cell lines from patients with small cell lung cancer treated with different combination chemotherapy regimens.

We studied 107 specimens (38 tumors and 69 tumor cell lines) from 90 patients with small cell lung cancer to determine the characteristics and clinical situations of patients from whom tumor cell lines could be established and the myc family DNA copy number. The proportion of extensive stage small cell lung cancer patients from whom a tumor cell line could be established prior to the initiation of therapy increased during the 10 years of the study (P less than 0.001). Amplification of one of the myc family genes occurred in 3 of 40 (8%) of the untreated patient specimens compared to 19 of 67 (28%) of the treated patient specimens (P = 0.01). The myc family DNA amplification occurred in 17 of 54 (31%) of the specimens from patients treated with cyclophosphamide-based combinations and 2 of 13 (15%) of the specimens from patients treated with etoposide/cisplatin (P = 0.25). Both tumors and tumor cell lines were obtained from 17 patients with small cell lung cancer and the myc family DNA copy number was similar in 16 of the 17 patients. We conclude that: (a) myc family DNA amplification occurs more commonly in specimens from treated than untreated patients (b) there are no prominent differences in the frequency of amplification following treatment with different chemotherapy regimens; and (c) myc family DNA amplification is similar in tumors and tumor cell lines from the same patients.     

Small cell carcinoma of the esophagus: a case report

This article reports a case of primary undifferentiated small cell carcinoma of the esophagus with lymph node metastasis which invaded the stomach wall. The patient was treated with chemotherapy alone, consisting of CDDP and VP-16. The patient had a complete response to chemotherapy, with no evidence of disease for nine months, after six courses of the regimen. Small cell carcinoma of the esophagus is an aggressive tumor with an extremely poor prognosis. Because its characteristics are similar to small cell carcinoma of the lung, small cell carcinoma of the esophagus should be treated by multi-drug chemotherapy including CDDP, with or without radiation as the first line treatment. This chemotherapy regimen may achieve a long disease-free survival time.      

Preoperative treatment of advanced skin carcinoma with cisplatin and bleomycin

In advanced skin carcinomas of the head and neck region, the tumor may be unresectable or curative resection or radiation may either fail or produce poor functional and cosmetic results. The goal of this study was to test the ability of preoperative chemotherapy to decrease the extent of needed resection and tumor response. Five patients, three with squamous cell carcinoma and two with basal cell carcinoma, were treated before surgery with three cycles of cisplatin 20 mg/m2 daily for 4 days and bleomycin 20 mg/day for 4 days by continuous infusion every 3 weeks. All patients had advanced cancer in the head and neck region, one had unresectable tumor, two had xeroderma pigmentosum, and one was a 13-year-old child. The history of prior treatments was as follows: radiation therapy (n = 3), systemic chemotherapy (n = 1), surgery (n = 1), and no treatment (n = 1). One patient had complete clinical but not pathologic response, three had partial response, and one had progressive disease. The extent of resection after chemotherapy was dramatically reduced in the patient with a complete response and minimally changed in the patients with partials responses and progressive disease. All patients who underwent surgery became tumor free. Preventable toxicity was symptomatic hypomagnesemia in one patient, reversible elevation of creatinine in one patient, and mild nausea and vomiting in three patients. With this report, the total number of patients with nonmelanoma skin cancer who were treated with cisplatin is 68, and their overall response is 80%. Only 16 reported patients were treated with preoperative chemotherapy, and study of this treatment approach to advanced skin cancer should be pursued.     

Preservation of the vulva in stage III squamous cell carcinoma with intra-arterial chemotherapy

A patient with stage III vulvar squamous cell carcinoma who was successfully treated with intra-arterial and intravenous chemotherapy and subsequent local excision is presented. The tumor was located on the left side of the vulva. Chemotherapy initially was administered into the left internal iliac artery. After two courses of the intra-arterial infusions, the tumor was no longer visible macroscopically. Two additional courses of chemotherapy were given intravenously to treat possible metastases to the lymph nodes. After the chemotherapy, the tumor site in the vulva was excised. A few small nests of tumor cells were identified in the excised specimen. The appearance of the vulva after surgery was quite natural and similar to that of a woman without disease. The patient is alive and disease-free 36 months after surgery. Conservative therapy, which includes intra-arterial and intravenous chemotherapy, should be considered for selected women with advanced vulvar squamous cell carcinoma. Received: November 24, 1998 / Accepted: April 5, 1999     

Immunohistochemistry of the blood group A,B,H isoantigens and Oxford Ca antigen as prognostic markers for stage IB squamous cell carcinoma of the cervix

Currently, there is lack of a histologic classification of squamous cell carcinoma of the cervix that correlates significantly with patient survival. This study investigated the survival predictive value of two immunohistochemical markers, the blood group A,B,H isoantigens and the Oxford Ca antigen, on conventional histologic sections of tumor tissues from 85 surgically treated patients with Stage IB squamous cell cervical cancer. The results indicated that the two antigens are two distinct markers, neither of which correlates with tumor grade. The expression of the A,B,H isoantigens is significantly related to patient survival after adjustment for the depth of stromal invasion of the tumor, whereas the Oxford Ca antigen has no survival predictive value.     

肺上皮样血管内皮细胞瘤临床病理分析

目的分析肺上皮样血管内皮瘤(PEH)的临床病理特征、诊断、鉴别诊断、治疗及预后。方法对1例原发性PEH的临床病理和免疫组织化学特征进行回顾性分析,并结合相关文献复习。结果 CT检查示双肺多发性结节,行肺部肿物切除术。光镜下肿瘤为境界清楚的嗜酸性结节,中心可见均一的类似淀粉样变或软骨瘤的透明变性组织;瘤细胞呈上皮样,也可呈实性巢状,瘤细胞出现脂肪样空泡,有的空泡内含单个红细胞,实为新生的原始血管;细胞异型不明显,核分裂像罕见。免疫组化结果:肿瘤组织中表达CD34、CD31、Ⅷ因子、Vimentin、CK等标记物。结论 PEH是1种罕见的低度恶性肿瘤,好发于中年女性,临床表现无明显特异性,诊断该病需结合影像学及病理学相关检查。     

Utility of MRI versus tumor markers for post-treatment surveillance of marker-positive CNS germ cell tumors

Patients with marker-positive central nervous system (CNS) germ cell tumors are typically monitored for tumor recurrence with both tumor markers (AFP and b-hCG) and MRI. We hypothesize that the recurrence of these tumors will always be accompanied by an elevation in tumor markers, and that surveillance MRI may not be necessary. We retrospectively identified 28 patients with CNS germ cell tumors treated at our institution that presented with an elevated serum or cerebrospinal fluid (CSF) tumor marker at the time of diagnosis. We then identified those who had a tumor recurrence after having been in remission and whether each recurrence was detected via MRI changes, elevated tumor markers, or both. Four patients suffered a tumor recurrence. Only one patient had simultaneously elevated tumor markers and MRI evidence of recurrence. Two patients had evidence of recurrence on MRI without corresponding elevations in serum or CSF tumor markers. One patient had abnormal tumor markers with no evidence of recurrence on MRI until 6 months later. We conclude that in patients with marker-positive CNS germ cell tumors who achieve complete remission, continued surveillance imaging in addition to measurement of tumor markers is indicated to detect recurrences.     

Rapidly progressive primary adenocarcinoma of the lung with signet-ring cells responding to 5-fluorouracil and leucovorin: A case report

A 47-year-old female patient with a tumor mass in the left upper lung was admitted to our hospital. Needle biopsy of the tumor revealed signet-ring cell carcinoma, which is relatively rare in the lung. Since no other primary site was found, she was diagnosed with primary lung adenocarcinoma with signet-ring cells. The primary tumor showed rapid growth. She was first treated with combination chemotherapy consisting of continuous 5-FU infusion, leucovorin, and CDDP. Although the size of the primary tumor was markedly diminished, a new metastatic lesion appeared after chemotherapy. Subsequent continuous 5-FU infusion and leucovorin without CDDP chemotherapy controlled aggressive growth of the primary tumor for several months. This case suggests that 5-FU should be included in the anticancer regimen for primary signet-ring cell carcinoma of the lung.     

Complete remission of bladder cancer with multiple pulmonary metastases obtained by combined chemotherapy with cis-platinum, adriamycin and cyclophosphamide

A 54-year old man with metastatic transitional cell carcinoma (grade 3) of the bladder has achieved complete remission as a result of combination chemotherapy. Plain chest X-P demonstrated multiple pulmonary metastases and computed tomography demonstrated a 35 X 40 mm-sized bladder tumor. The patient was treated with four courses of combination chemotherapy consisting of cis-platinum, adriamycin and cyclophosphamide, after which complete disappearance of the pulmonary metastases and bladder tumor was observed. The patient has been followed up, with no evidence of tumor nine months after the treatment.     

Primary small cell carcinoma of the pleura: a case report with immunohistochemical and molecular genetic analyses of <Emphasis Type="Italic">KIT</Emphasis> and <Emphasis Type="Italic">PDGFRA</Emphasis> genes

An extremely rare case of primary small cell carcinoma of the pleura with an emphasis on KIT and platelet-derived growth factor receptor-α (PDGFRA) genes is reported here. A 67-year-old man underwent left testicular orchiectomy because of a testicular tumor. The tumor was immunohistochemically shown to be diffuse large B-cell lymphoma. The patient was treated with chemotherapy and radiation and followed up in our hospital. Eight years after the orchiectomy, the patient (75 years old) developed left pleural tumor and pleural effusion, and a biopsy was performed. The biopsy revealed a medullary malignant tumor consisting of small round and spindle cells. The following three possibilities were considered: recurrent lymphoma, mesothelioma, and small cell carcinoma. Immunohistochemically, the tumor cells were positive for cytokeratin, synaptophysin, CD56, KIT, and PDGFRA, but negative for CEA, cytokeratin 5/6, neuron-specific enolase, chromogranin, CD45, CD3, CD20, CD45RO, CD15, CD30, calretinin, WT-1, B72.3, D2-40, and TTF-1. Therefore, a diagnosis of pleural small cell carcinoma was made. A molecular genetic analysis using PCR-direct sequencing identified no mutations of KIT (exons 9, 11, 13, and 17) and PDGFRA (exons 12 and 18) genes. The patient was treated with chemotherapy (cisplatin) and radiation (50 Gray). The present case is the first reported case of primary small cell carcinoma of the pleura with an examination of KIT and PDGFRA expressions and KIT and PDGFRA gene mutations.     

Radiation therapy in the treatment of difficult giant cell tumors

Thirteen patients with giant cell tumors of bone have been treated by radiation therapy because surgery was not feasible or unacceptably disfiguring. Seven patients were treated for primary giant cell tumors of the bone, four for recurrent disease, and three for metastasis (one presented with both distant metastasis and local recurrence after primary surgery). The follow-up time ranged from 18 months to 13 years, with a mean of 6.5 years. All patients except one are alive. Local control was achieved in 11 patients (85%). One patient whose tumor was located in the sacrum had no gross response and at 5 months was subjected to a partial sacrectomy. A second patient had local regrowth 1 year after treatment; salvage surgery was successful. There have been no long-term complications of radiation therapy. This study confirms that for patients with giant cell tumor of bone, radiation therapy offers an effective alternative to complex or difficult surgery and constitutes a good treatment method to medically inoperable patients.     

Solitary metastasis in a nasal fossa as the first manifestation of a renal carcinoma

Renal cell carcinoma is an uncommon tumor in adults. metastasis in the nasal fossa is rare, and can become apparent as a result of repeated epistaxis. We report a patient with renal cell carcinoma presenting with epistaxis secondary to a metastasis in the right nasal fossa. The primary tumor was treated with nephrectomy and the nasal fossa metastasis was treated successfully with embolization, chemoimmunotherapy, surgery, and radiotherapy. The presence of repeated epistaxis may very occasionally be the first symptom of renal cell carcinoma, and systemic treatment combined with local treatment may enable adequate control of the disease.     

Primary non-Hodgkin's lymphoma in the dorsolumbar muscles

We report a case of primary soft tissue lymphoma with pulmonary involvement in a 57-year-old man, successfully treated with surgery and chemotherapy. The patient presented with a giant mass (16 × 20 cm) in the left dorsolumbar region. Computed tomography (CT) demonstrated a right-sided pulmonary tumor, in addition to the giant tumor in the left dorsolumbar muscles. On an incisonal biopsy, the left dorsolumbar muscle tumor was suspected to be a sarcoma, and en-bloc resection was therefore performed. During surgery, four left pulmonary tumors that had not been observed on CT were found and surgically resected. The patient was finally diagnosed with soft tissue non-Hodgkin's B cell lymphoma with pulmonary involvement. After postoperative chemotherapy, the right pulmonary nodule disappeared. The patient was well and had no evidence of disease 22 months after surgery. To the best of our knowledge, primary soft tissue lymphoma is extremely rare. Received: August 14, 1997 / Accepted: May 8, 1998     

Radiotherapy for the treatment of giant cell tumor of the spine: a report of six cases and review of the literature

Optimal treatment for giant cell tumors in the axial skeleton (GCTS) remains challenging. Surgical excision remains the treatment of choice, but the potential spinal cord injury may limit the extent of resection. We report the long-term results of treatment of six patients diagnosed with giant cell tumor of the spine treated with radiotherapy and review the literature regarding therapy. Between 1971 and 1995, six patients with GCTS were treated with conservative surgery and radiotherapy. The surgery consisted of either biopsy or subtotal resection of tumor. The involved vertebrae were then irradiated with doses ranging from 3000 to 5400 cGy. The mean follow-up was 13 years, and three of six patients had follow-up of 17 or more years. Five of six patients are alive with no evidence of disease. One is alive with disease, although it is not clinically apparent. No patient was lost to follow-up. We conclude that radiotherapy is useful in the management of GCTS and that conservative surgery with local radiotherapy is a reasonable treatment alternative for tumors that cannot be completely excised or in which surgery would result in significant functional morbidity. Although there is no clear dose response, review of the literature suggests that doses ranging from 3500 to 4500 cGy are safe and effective in controlling giant cell tumor.     

Optically guided stereotactic radiotherapy for lacrimal sac tumors: a report on two cases

Adjuvant radiation treatment is often offered for lacrimal sac tumors. However, due to the adjacent critical structures, conventional radiation technique may result in severe side effects. We have treated two patients with lacrimal sac tumors using optically guided stereotactic radiotherapy. One patient with lacrimal sac melanoma was treated with optical-guidance intensity-modulated radiotherapy (IMRT). The other with mixed transitional and squamous cell carcinoma was treated with optical-guidance 3-D conformal radiation. Dose volume analysis revealed excellent target coverage and sparing of critical structures. Both patients tolerated the treatment well with no significant acute or late side effects. One patient died of metastatic melanoma 30 months after radiation; another died of coexisting disease 41 months after radiation. Both had no clinical evidence of local recurrence at the time of death. Our report show that optically guided stereotactic radiotherapy is well tolerated. It offers excellent tumor coverage and sparing of critical structures. It can be used for tumors adjacent to radiation sensitive critical structures such as skull base tumors.     

Primitive Neuroectodermal Tumor of the Jejunum; A Case Report and Literature Review

Objective and Importance

The aim of this paper is to report an unusual presentation of extranodal follicular dendritic cell tumor of neck with spinal metastasis. Follicular dendritic cells are nonlymphoid immune accessory cells present in the germinal centers of lymphoid follicles and play a crucial role in the induction and maintenance of the humoral immune response. Tumors from these cells are rare and treatment modality poorly defined.

Clinical Presentation

A 37-year-old lady presented with recurrent neck swelling which was initially reported as malignant paraganglioma. The primary disease was treated with surgery and radiotherapy. Eleven years later, the patient presented with metastasis to the spinal cord. Subsequent immunohistochemical analysis of the primary site tumor and the metastatic deposits revealed it to be a follicular dendritic cell tumor.

Intervention

The patient was treated with surgery followed by radiotherapy to spine, and one and half year after treatment, the patient is doing well and has regained complete motor functions.

Conclusion

Metastasis to spinal cord for follicular dendritic cell tumor is very rare, and to the best of our knowledge, no such case has been previously reported in the scientific literature so far. In the present case, good local control was achieved with initial surgery and radiotherapy but resulted in distant failure after 11 years. This underlines the need for adjuvant systemic therapy, and understanding the biology of the tumor may help in formulating targeted therapy in the future for this rare disorder.     

EGFR amplification induces sensitivity to antiEGFR therapy in pancreatic acinar cell carcinoma

Pancreatic acinar cell carcinoma (PACC) is a rare cancer. When the tumor is metastatic, few therapeutic options are available. Precision medicine using next-generation sequencing is defined by the administration of drugs based on the tumor genetic mutations. The usage of precision medicine for finding new therapeutic options for rare cancers is an emerging field. We have reported here the case of a patient bearing a multitreated metastatic PACC. This patient underwent somatic and constitutional exome analyses. The analyses revealed in the liver metastasis an amplification of the EGFR gene. Accordingly, the patient was treated with off-label usage of panitumumab. We observed rapid response with necrosis of the liver metastasis, while no efficacy was observed in the primary tumor. An exome analysis of the primary tumor revealed amplification of HER2 and MET with EGFR amplification. Such amplifications are known as a resistance mechanism to antiEGFR therapy. Our results suggest that exome analysis may be helpful to highlight targets in rare cancers, such as PACC. EGFR amplification in this pathology should be determined and could be used as a biomarker to propose antiEGFR therapy.     

Mixed germ cell-sex cord stroma tumor of the testis. A report with ultrastructural findings

The case of a 41-year-old man with testicular mixed germ cell-sex cord stroma tumor is reported. The patient noticed a gradual, painless, right-sided testicular enlargement 4 years before being treated by radical orchiectomy. The testis contained a centrally located tumor. There was no evidence of metastases. An histologic examination and an ultrastructural study showed that the tumor was composed of germ cells and sex cord derivatives; the typical features of mixed germ cell-sex cord stroma tumor were present. The patient is well and disease-free 2 years after the operation. Currently, none of the testicular tumors of this type has been associated with metastases or was overgrown by malignant germ cell tumors, and radical orchiectomy resulted in complete cure. The literature regarding this entity is reviewed, the differential diagnosis is discussed, and the importance of making the correct diagnosis is emphasized.     

Nonlymphoid mesenchymal tumors of the parotid gland

Salivary gland tumors are uncommon and most of them are of epithelial origin. Mesenchymal tumors affecting the parotid are extremely rare, and we present a series of 19 cases. All parotid tumors (600 cases) treated at the Department of Head and Neck Surgery from A.C. Camargo Hospital, Brazil from 1953 to 2003 were reviewed and 19 cases of nonlymphoid mesenchymal origin were selected. The histological characteristics were reviewed and clinical features were obtained from the medical charts. 15 out of 19 were benign tumors, including 5 lymphangiomas, 5 neurofibromas, and one case each of schwannoma, lipoma, solitary fibrous tumor, meningioma and giant cell tumor. Four malignant tumors were classified as rhabdomyosarcoma, fibrosarcoma, Langerhans cell histiocytosis and endodermal sinus tumor. From the malignant cases, only the patient with fibrosarcoma died due the tumor, the other three are alive with no signs of recurrence. In our series of 600 cases of parotid gland tumors, nonlymphoid mesenchymal tumors corresponded to 3.16% (19 cases; 15 benign and 4 malignant). All cases were treated by surgery with no recurrences, except one case of fibrosarcoma whose patient died of distant metastasis.