Metastatic prolactinoma: effect of octreotide, cabergoline, carboplatin and etoposide; immunocytochemical analysis of proto-oncogene expression

A 49-yr-old woman presented with an extensive prolactinoma (serum PRL > 10,000 mU/L, normal range < 450 mU/L). Over a 5-yr period following transsphenoidal surgery and pituitary irradiation, she became increasingly resistant to high doses of bromocriptine and underwent transfrontal surgery followed by stereotactic radiotherapy. In spite of these treatments, serum prolactin estimations rose progressively to > 100,000 mU/L. Magnetic resonance imaging scanning demonstrated a massive cystic tumor invading the temporal lobes, extending into the cervical and thoracic spine, with metastases to cervical lymph nodes. High-dose cabergoline administration resulted in a 30% decrease in serum PRL. Octreotide was administered as a continuous sc infusion with a profound analgesic effect on facial pain but with no effect on tumor progression. She was treated with a course of chemotherapy consisting of carboplatin and etoposide without any noticeable effect. The patient died 6 months following chemotherapy. Immunocytochemical analysis demonstrated positive nuclear staining for WAF-1, Rb protein, c-myc, and p53 both in the original and metastatic tumors. The metastases but not the primary tumor stained for c-jun. Metastatic prolactinoma remains a therapeutic challenge. It is associated with a variable proto-oncogene expression, which may be coincidental or causal. Cabergoline had no advantage over bromocriptine. Octreotide relieved facial pain but did not alter tumor progression. An effective therapy for metastatic prolactinoma remains to be identified.     

Malignant hemangioendothelioma of the thyroid gland: new results on pathogenesis, therapy and prognosis

BACKGROUND: Malignant hemangioendothelioma of the thyroid is a rare tumor predominantly described in areas with endemic goiter like the Alpine regions. The estimated incidence of the disease is between 0.15 and 0.25 per 100,000 inhabitants per year for Western Austria. The prognosis is reported to be dismal. MATERIALS AND METHODS: Between 1982 and 1995, 10 cases with immunohistochemically confirmed malignant hemangioendotheliomas of the thyroid were referred to our department for postoperative or palliative treatment. Two patients with clear margins at surgery received no adjuvant radiotherapy and were only observed. By surgery, clear margins (R-0 resection) were achieved in 5, microscopic residuals (R-1) were left in 3, and gross residual disease in 1 patient. One patient had an inoperable primary tumor. Postoperative radiotherapy was administered in 6 cases, 4 of them additionally received the radiosensitizer razoxane. Total tumor doses ranged between 58 and 65 Gy. RESULTS: Local tumor control was achieved in 9 of 10 patients; 4 of 10 lived longer than 4 years. The median survival time has not yet been reached and is presently between 7.5 and 21+ months.--Noteworthy is a complete regression of 2 lung metastases in a 72-year-old man by a combination of vindesine, razoxane and radiotherapy. The patient is still in complete remission under a maintenance therapy with vindesine and razoxane since 14 months.--It may also be of interest that 4 of the 10 patients were strongly exposed to vinyl chloride and other polymeric materials during their occupational life. CONCLUSIONS: This small series may indicate that the outcome of this disease may not be uniformly deleterious, and that the resistance to radiotherapy reported in the literature may be questioned.--The data offer new evidence of the occurrence of vinyl chloride-induced angiosarcomas outside the liver, and support observations which have already been published in case reports.     

Merkel cell tumor. Report of case and treatment with octreotide

Merkel cell carcinoma is a rare neuroendocrine tumor of the skin. Prognosis is very poor particularly when systemic disease is present. Surgery, chemo and/or radiotherapy treatment are not able to guarantee long survival and quality of life is also very poor. We know that neuroendocrine tumor can be in possession of receptors for somatostatin; during the past years, these receptors have been demonstrated in vivo by octreoscan. We report a case of a patient suffering from metastatic Merkel cell carcinoma; because he was elderly, neither chemotherapy nor radiotherapy were possible as a consequence of the explosion of the disease after surgery. The presence of receptors for somatostatin analogues (octreoscan) allowed treatment with octreotide causing the immediate disappearance of metastasis. After ten months of treatment the patient presents a complete remission of disease. Octreotide, the most important somatostatin analogue, represents a primary role in the neuroendocrine tumor management; the drug also lacks of toxicity.     

Bronchopulmonary atypical carcinoid tumour metastatic to the orbit

BACKGROUND: Bronchopulmonary carcinoid tumours metastatic to the orbit are rare. A case is presented demonstrating presentation, histopathology and treatment. METHODS: A 64-year-old man with a history of bronchopulmonary atypical carcinoid presented with an orbital mass. The nature of this metastasis was confirmed with histology and it was surgically excised and, in addition, local radiotherapy and chemotherapy were administered. RESULTS: There was a good response to the treatment for the orbital mass but other metastases developed, causing death. CONCLUSIONS: In this case of atypical carcinoid, orbital metastasis was the first sign of disease progression. Histological confirmation of the diagnosis was important in this case to allow planning of oncological therapy and for appropriate patient counselling. A good local response was obtained with a combination of surgical excision, local radiotherapy and chemotherapy. The systemic prognosis remains poor in cases of metastatic bronchopulmonary atypical carcinoid tumour, in contrast to typical carcinoid.     

Anterior capsulotomy created by radiofrequency endodiathermy and continuous curvilinear posterior capsulorhexis in a patient with intumescent cataract and primary capsular fibrosis

PURPOSE: To report a clinicopathologic correlation of angiosarcoma affecting the eyelid skin. METHODS: An 82-year-old man developed multiple bruise-like maculopapular lesions, subcutaneous nodules, and diffuse edema over his scalp, face, and eyelids. Biopsy disclosed angiosarcoma, and the patient was treated with wide-field external beam radiotherapy (5,000 cGy). RESULTS: He responded to radiotherapy with partial regression of the tumor. Twelve months later, he developed extensive lymphedema secondary to the residual tumor. He was also found to have parotid gland and bone metastases, treated with radiotherapy. The patient died of widespread bone metastases 3 months later. CONCLUSIONS: Angiosarcoma is a rare skin tumor that has a poor prognosis despite treatment. It should be considered in the differential diagnosis of eyelid tumors and edema because it may affect only the central portion of the face.     

转移到眼前房的转移性乳腺癌病例报道及讨论

Intraocular metastases are the most common malignancies of the eye. Carcinoma of the breast is the most common malignancy to metastasize to the uvea usually posterior uvea. Anterior chamber metastasis is extremely rare. We describe a patient of breast cancer who presented with pain and impaired vision in right eye 8.5 months after metastasis to bone and other viscera were detected and more than 10 years after her primary disease was diagnosed. Multiple yellowish white deposits were noted scattered in anterior chamber giving rise to symptoms of acute narrow angle glaucoma. Metastatic deposits of duct cell carcinoma cells were found by fine needle aspiration cytology. Patient received local external beam radiotherapy to right eye with moderate improvement in her symptoms. The rare features in this case were clinical presentation of acute glaucoma and anterior chamber as the site of metastasis.     

Should dialysate calcium be varied in proportion to the amount of ultrafiltration in peritoneal dialysis dwells? Directions from a computer simulation

Patellar metastases are very rare. There have been approximately 20 cases reported in the literature. We have also noted two other reports of patellar metastasis from lung carcinoma as the first manifestation of lung cancer in our literature review. We present a case of patellar metastasis as the first manifestation of lung epidermoid carcinoma in a patient who was a smoker for 33 years.     

Medical care in 7 Chilean cities

Sclerosing mucoepidermoid carcinoma with eosinophilia (SMECE) is a recently recognized malignant neoplasm of the thyroid gland. Two additional cases of this condition which occurred in a 70-year-old woman and a 69-year-old woman are presented. The case of the 70-year-old woman (patient 1) is the first report of distant metastasis, besides lymph node metastasis, for this type of tumor. The patient initially presented with a thyroid mass, and the thyroid gland with surrounding cervical lymph nodes was removed. Because of focal keratin "pearl" formation, the tumor was misinterpreted as a metastatic squamous cell carcinoma to the thyroid. Approximately 4 years later, the patient developed a left supraclavicular mass and lung densities. A pathological fracture of the right humeral head followed, and the left supraclavicular mass recurred along with newly developed subcutaneous nodules on the chest wall and arm. Open lung and bone biopsies revealed metastatic SMECE, which was morphologically identical to that of the thyroid mass. The 69-year-old woman (patient 2) had, in 1983, undergone thyroidectomy with left radical neck dissection; this had been diagnosed as follicular carcinoma of the thyroid with lymph node involvement. After multiple isolated lymph nodes metastases, the patient developed locally extensive, recurrent tumor that showed microscopic features of SMECE. Review of the previous thyroid tumor and lymph nodes revealed the same type of histology. To our knowledge, only a single report containing eight cases of this distinctive carcinoma of the thyroid has been published. Herein we describe characteristic morphological features of two additional cases of this rare malignancy, one with distant metastasis, and we review the related literature.     

Modern methods of wound treatment. 1. One wound is not the same as all the others

Papillary carcinoma, the commonest thyroid malignancy, has a good prognosis and low incidence of distant metastases. Brain metastasis is extremely rare with a frequency of about 1% in reported series. In this paper we present the clinical details of one case of histologically proven brain metastasis from papillary thyroid cancer, first presented with neurological symptoms, initially treated with excisional biopsy and radiotherapy in other hospital, without clinical response. The patient was then referred to our service, where he underwent a total thyroidectomy and modified radical neck dissection, with the aim of posterior radioactive iodine treatment for the brain lesion. Unfortunately, he died of neurological complications, two months after the neck treatment. Also presented is a review of the literature of this unusual clinical presentation.     

Uveal metastasis from carcinoid tumor. Clinical observations in nine cases

BACKGROUND: Carcinoid tumor is a low-grade malignancy that usually arises in the gastrointestinal tract or bronchus and rarely metastasizes to the eye. Metastasis of carcinoid tumor to the uvea can be confused clinically with other primary and metastatic uveal tumors. METHODS: The authors reviewed the records of 410 consecutive patients with uveal metastases referred to the Ocular Oncology Service at Wills Eye Hospital to identify those in whom carcinoid tumor was the primary neoplasm. The authors evaluated the clinical features of these metastases. RESULTS: Of 410 consecutive patients with uveal metastases, the primary neoplasm was a carcinoid tumor in 9 (2.2%). There were four men and five women. The mean age at ocular diagnosis was 50 years. In five patients (56%), the primary tumor was undiagnosed at ocular presentation. In the other four patients, the mean time interval from diagnosis of the primary carcinoid tumor to uveal metastasis was 89 months (range, 55-180 months). The site of the primary carcinoid tumor was the bronchus in seven patients, the esophagus in one, and the thymus in one. The site of intraocular metastasis was the choroid in six patients, the ciliary body in two, and the iris in one. All choroidal tumors had a characteristic orange color. Initial ocular treatment included external beam radiotherapy in five patients, plaque radiotherapy in two, argon laser photocoagulation in one, and local resection in one. Ocular tumor control was achieved in each patient. After a mean follow-up of 34 months, four patients (44%) are still alive. Five patients have died, with a mean survival of 34 months (range, 2-104 months) after the diagnosis of uveal metastasis. CONCLUSIONS: Uveal metastasis from carcinoid tumor is rare and tends to arise from the bronchus. Clinically, it has a distinctive orange color and may be associated with a longer systemic survival, compared with uveal metastasis from other primary sites.     

Bromocriptine therapy for "nonfunctioning" pituitary tumors

Bromocriptine therapy may cause regression of prolactinomas, but its effect on nonsecretory pituitary tumors is uncertain. Conventional treatment for such "functionless" tumors is surgery and/or radiotherapy, but recurrences pose a therapeutic dilemma. We describe a patient with such a tumor treated by surgery and radiotherapy who presented with recurrent disease 14 days later. On treatment with bromocriptine, 20 mg daily for 25 months, the intrasellar tumor recurrence had diminished in size and a suprasellar extension had almost disappeared. Bromocriptine therapy may therefore benefit some patients with nonsecretory pituitary tumors considered unsuitable for surgery or radiotherapy.     

A comparison of the bactericidal and photobactericidal activities of aminoacridines and bis(aminoacridines)

The nasal cavity is a rare location for primary malignant melanoma, borne out by the few cases reported in the literature since 1940. Rarest of all is nasal melanoma in a patient with oculocutaneous albinism. An albino subject, aged 33, was referred to us with this diagnosis. Because of the numerous metastases in the liver and left lung, we did not advise radical surgical intervention and, despite hyperfractionated high dose radiotherapy and chemo-immunotherapy, the advanced stage of disease caused the death of the patient within a year. We have reviewed the diagnostic, clinical and therapeutic aspects, and the literature on melanomas of the nasal cavity in albino subjects. We performed a Medline literature search, examining only 'extensive' papers and excluding abstracts or case reports presenting poor data.     

Extrarenal Wilms' tumor: diagnosis and treatment review regarding a case report

OBJECTIVES: To report a case of extrarenal Wilms' tumor, an uncommon site of presentation of this tumor type. The diagnostic and prognostic aspects of this condition are also discussed. METHODS/RESULTS: A case of retroperitoneal extrarenal Wilms' tumor in a 2-year-old child is presented. The patient underwent surgery and received postoperative chemotherapy. At 3-years' follow-up, no evidence of metastasis has been observed. CONCLUSIONS: Extrarenal Wilms' tumor is rare and has been reported in the literature principally as case reports. Its clinical presentation varies according to the extrarenal localization. The procedures utilized to determine the size of the primary tumor, regional node involvement and the presence of distant metastasis are similar to those utilized in Wilms' tumor of the kidney. Our results demonstrate the utility of chemotherapy; the cytostatic agents utilized appear to be as effective as in Wilms' tumor of the kidney. In our view, radiotherapy should be reserved for the large unresectable residual tumor mass and for distant metastasis.     

Shunt-related abdominal metastasis of cerebral teratocarcinoma: report of an unusual case and review of the literature

OBJECTIVE AND IMPORTANCE: Internal drainage of cerebrospinal fluid to the abdominal cavity via a ventriculoperitoneal shunt (VPS) is a common procedure for therapy of obstructive hydrocephalus; because this condition is often caused by brain tumors blocking the natural cerebrospinal fluid pathways, the VPS as an artificial anastomosis can provide the means for the spreading of tumor cells by the cerebrospinal fluid. We report the case of a VPS-related abdominal metastasis of a teratocarcinoma and review the pertaining literature. CLINICAL PRESENTATION AND INTERVENTION: A 24-year-old man with a history of three brain tumors that were operated on when the patient was 14, 21, and 23 years of age developed an acute ileus 7 months after VPS insertion for cerebral teratocarcinoma. Intraoperatively, a massive abdominal tumor was observed, which turned out to be a peritoneal metastasis of the aforesaid brain tumor. The patient died as a result of his illness 1 month later. RESULTS: To date, 58 VPS-related metastases of brain tumors have been described. The male-to-female ratio is 1.6:1, the mean age at shunt insertion is 12.2 years, and the interval between shunt operation and diagnosis of metastases is 16.8 months. During the observation time, 69.2% of the patients died as a result of their illness or abdominal metastases. The most common sources of the metastases were germinomas (27.7%), medulloblastomas (19.1%), and endodermal sinus tumors (10.3%). CONCLUSION: The presented case is only the second VPS-related abdominal spreading of a cerebral teratocarcinoma. Metastases via VPS are rare but should be considered as a possible complication and mode of systemic spread in patients with primary intracranial malignancy.     

The parotid gland as a filtering station of metastatic head-neck melanoma. Retrospective study of 884 patients

Formation of metastases was observed in 246 cases (27.8%) out of a group of 884 patients with melanoma of the head and neck region treated in the years 1967-1991. In the group of patients with metastases, regional metastases were found in the cervical lymph nodes in 136 cases (55.3%). In 74 patients (30.1%) the first metastasis was a distant metastasis, i.e., the tumor had spread by hematogenic dissemination. In 53 patients (21.5%) the first metastasis was located in the parotid gland. Evaluation of the clinical data of the patients led to interesting results regarding prognosis following the different types of surgical treatment. The 5-year survival rates were established by means of multivariant analysis using the Cox model, taking into account sex and tumor thickness: Following radical tumor removal, including neck dissection and parotidectomy, the 5-year survival rate amounted to 61.8%. If the parotid gland was not removed and only tumor and cervical lymph nodes were resected, 66.2% of the patients were still alive 5 years following surgery. The difference between these two groups was statistically not significant (P = 0.07). In those cases where only the primary tumor was removed, the 5-year survival rate was 85.8% and thus significantly better than in the two other groups (P < or = 0.0001). Two conclusions can be drawn: In metastasizing melanoma of the head and neck the parotid glands are affected in 20% of the cases and thus more frequently than expected. The retrospective analysis of groups of patients differing with regard to the prognosis of their disease cannot be used to provide information on which therapy is the best. This is particularly true for the question whether or not the parotid gland should be removed in addition to a neck dissection. It will be necessary to perform a prospective randomized study in order to find answers to these questions. Such a study could be performed within DOSAK (Deutsch-Osterreich-Schweizerischer Arbeitskreis für Tumoren im Mund-Kiefer-Gesichtsbereich = German, Austrian, Swiss Working Group on Tumors in the Maxillo-Facial Region) in cooperation with different hospitals.     

Symptomatic renal metastasis from testicular cancer

The clinical course of a patient who presented with a massive left renal metastasis from testicular carcinoma is described. Treatment with cisplatin-based chemotherapy produced a dramatic response. Symptomatic renal metastases from testicular cancer are rare and may be reflective of a high tumor burden or refractory disease.     

Drug resistant typhoid fever

BACKGROUND: Malignant schwannomas are rare malignant mesenchymal tumors often associated with neurofibromatosis. They occur less frequently in the head and neck than in other regions. PATIENT: A case history of a primary malignant schwannoma of the head and neck area in a 27-year-old man is reported. The tumor was located in the left submandibular region. The patient did not have any functional deficits. The tumor was totally removed. There have been no signs of either recurrence or metastasis within the two years following diagnosis and surgery. DISCUSSION: The microscopic and immunohistochemical findings are presented, and the importance and therapy of this very rare malignant tumor of the head and neck area are discussed. CONCLUSION: Malignant schwannoma in the head and neck region is rare. Radical resection is the treatment of choice.     

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.     

In vitro antiphagocytic effect of Melia azedarach leaf extracts on mouse peritoneal exudate cells

The first reported case of cerebellar metastasis from primary clear cell adenocarcinoma of the fallopian tube is presented. Initially diagnosed as stage Ia, the patient underwent total abdominal hysterectomy, bilateral salpingo-oophorectomy and omentectomy, followed by cisplatin-based chemotherapy and whole pelvic irradiation. Thirty six months later multiple pulmonary metastases were detected that did not respond to chemotherapy. Later the patient presented with cerebellar metastasis. She received whole brain radiotherapy and steroids. The brain lesion partially responded and the patient's neurologic symptoms improved. Throughout there was no evidence of local recurrence. This case suggests that with the prolonged survival achieved by aggressive treatment occult brain metastases might become apparent.     

Complete remission after radiotherapy for recurrent rectal cancer

Although the efficacy of radiotherapy for the palliation of recurrent or inoperable cancer has been proven, it results in complete tumor remission only rarely. We report a case of pelvic recurrence in which complete histological remission followed radiotherapy. A 68-year-old man developed a small pelvic recurrence near the rectal stump 8 months after a Hartmann procedure for rectal cancer. Histologic examination of the biopsy specimens revealed adenocarcinoma consistent with the primary tumor. He received radiotherapy to the whole pelvis, with a total dose of 39.4 Gy administered in 22 fractions over 4 weeks, with a continuous infusion of 5-fluorouracil as a radiosensitizer. After regression of the tumor was confirmed, resection of the pelvic recurrence was performed. Histologic examination of the resected specimen revealed no tumor. Thirty-six months after the second operation, the patient was alive with no evidence of disease. The experience of our case and a review of the literature suggests that small pelvic recurrences may be more responsive to radiotherapy than larger ones.