Multiple neoplasms of the salivary gland and the lung

Intracranial adenoid cystic carcinoma is uncommon. We report two unusual cases of intracranial adenoid cystic carcinoma. The first patient presented with a steroid-responsive optic neuropathy from an orbital mass that simulated orbital pseudotumor, and subsequently developed intracranial involvement, presumably by contiguous perineural spread. The second patient presented with proptosis of the left eye, right facial weakness and numbness, and an intracranial mass, presumably from hematogenous metastatic spread.     

Congenital onychodysplasia of the index fingers in identical twins

We describe a patient with metastatic disease to an intracranial meningioma in which MR images showed multiple foci of intense enhancement within a background of moderate enhancement. The discrete foci proved to be metastatic disease from the patient's known breast carcinoma.     

Epithelioid angiosarcoma of bone

Angiosarcoma of bone is a rare, high-grade sarcoma of vascular origin. This article describes an epithelioid angiosarcoma in the humerus of a 48-year-old man. A multilocular osteolytic lesion with undefined margins and destroyed cortical and medullary bone, associated with a large soft tissue mass was demonstrated radiologically in the proximal metaphysis of the right humerus. The tumor, resected by amputation, was composed mostly of proliferating malignant cells with an epithelioid morphology. It had a predominantly sheet-like growth pattern, and an occasional pseudoglandular or alveolar arrangement, mimicking an adenocarcinoma. The dilated anastomotic vascular spaces lined by epithelioid endothelial cells and the intracytoplasmic lumina/vacuoles that sometimes contained erythrocytes suggested focal endothelial differentiation. On immunohistochemical investigation, many neoplastic cells expressed cytokeratin and endothelial markers: factor-VIII related antigen, CD31, and UEA-I. The ultrastructure of the tumor was consistent with that of an angiosarcoma. Our patient died of disease shortly after the diagnosis, implying an aggressive clinical course. Awareness of the existence of skeletal epithelioid angiosarcoma, combined with the identification of intracytoplasmic lumina, or at least small vasoformative foci, and immunohistochemical positivity for endothelial markers provide the best guide for distinguishing this tumor from metastatic carcinomas.     

Avulsion of the flexor digitorum profundus tendon associated with a chondroma of the distal phalanx

Sarcoma of the penis is rare. We report an angiosarcoma that was essentially an incidental finding, despite the presence of metastatic lesions, in a 46-year-old man who presented with hematemesis and melena. The diagnosis of the primary lesion was confirmed by histology, and the presence of secondary lesions by fine needle aspiration cytology. The patient was given one dose of chemotherapy, but died of a myocardial infarction before being able to receive further treatment. The literature pertaining to this very unusual lesion is discussed.     

A 6p22 reference map of leukocyte DNA: exclusion of rearrangement in four cases of atypical haemochromatosis

Primary angiosarcomas of the chest wall and pleura are extremely rare and carry a dismal prognosis. Two cases are reported. One patient (case 1), presented with massive recurrent haemothorax, was found to have multifocal angiosarcoma of the pleura, treated with surgical de-bulking, chemical pleurodesis and chemotherapy, achieving control of the bleeding. She died 10 months later from complications related to chemotherapy. A full post-mortem examination confirmed this was a primary pleural angiosarcoma with no evidence of disease elsewhere. Another patient (case 2) with a large solitary angiosarcoma of the chest wall, discovered incidentally on a routine physical examination, was successfully treated with surgical excision and subsequent radical radiotherapy, remaining well 15 years post-operatively.     

Angiosarcomas associated with bone infarcts

OBJECTIVE: A bone infarct may occasionally dedifferentiate to osteogenic sarcoma, fibrosarcoma or malignant fibrous histiocytoma. However, the association of an angiosarcoma with a bone infarct is extremely rare. Such an association is presented in three patients. Their clinical course is compared with that of patients with bone infarcts associated with other sarcomas. DESIGN AND PATIENTS: The three patients were men with a mean age of 43 years. Cases 1 and 3 presented with a pathological fracture at the site of the angiosarcoma. Plain radiography was done in the three patients, computed tomography (CT) was performed in cases 1 and 3 and magnetic resonance imaging (MRI) in case 3. The femur was the site of the three tumors: midshaft in cases 1 and 3 and distal shaft in case 2. On the basis of the radiographic findings, and clinical examination, an open biopsy was performed for the three men, which confirmed the diagnosis of a high-grade angiosarcoma associated with a bone infarct. RESULTS: Case 1 was treated with high-above knee amputation and is still alive after 18 months from the time of operation. Segmental resection of the distal femur with adjuvant chemotherapy and local irradiation was the treatment for case 2, who is still alive with no tumor recurrence on metastatic disease 3 years from the operation. Intramedullary rodding was done for case 3 who died 6 months later. CONCLUSION: The association of an angiosarcoma with a bone infarct has been established in only five cases. Although the number of such associations is small, it seems that such an association may be prognostically more or less the same as in those cases in which a bone infarct is associated with either osteosarcoma, fibrosarcoma or malignant fibrous histiocytoma, where the survival rate is unfavorable. A cause-and-effect relationship may exist between a bone infarct and subsequent development of a bone sarcoma.     

Adjuvant chemotherapy for primary cardiac sarcomas: the IGR experience

The effect of additional treatments after surgery in patients with primary cardiac sarcoma (PCS) remains unknown. The present study aims to evaluate the benefit of chemotherapy in patients with non-metastatic cardiac sarcomas after optimal resection. Between October 1979 and December 1995, 15 patients with a median age of 45 (range 16-66) and a resected primary cardiac sarcoma [angiosarcoma (six), malignant fibrous histiocytoma (three), leiomyosarcoma (two), rhabdomyosarcoma (two), liposarcoma (one) and synoviosarcoma (one)] received a doxorubicin-containing regimen within 6 weeks of surgery. Adjuvant chemotherapy combinations included cyclophosphamide, vincristine and dacarbazine in four patients; ifosfamide in nine; methotrexate and vincristine in one; and doxorubicin alone in one patient. At present, 13 patients have relapsed (five during therapy), with a median time to progression of 10 months. Twelve patients developed local relapse, in four cases without metastatic disease. Two patients remain in complete remission 27 and 25 months after surgery. The median time to progression was shorter in patients presenting a cardiac angiosarcoma than other histological types (3 vs 14 months, P < 0.01). Twelve patients have died, with a median overall survival of 12 months. The 2-year survival rate is 26%. Survival was significantly longer for patients with completely resected tumours (22 vs 7 months; P = 0.02) and those who did not have angiosarcoma (18 vs 7 months; P = 0.04). In conclusion, post-operative conventional doxorubicin-based chemotherapy failed to modify the natural history of patients with resected cardiac sarcomas. Locoregional failure remains the main problem even after histologically complete resection. New approaches must be tested in patients with primary cardiac sarcoma.     

Parotid gland metastases of two uncommon neoplasms with low metastatic potential

Oligodendrogliomas are uncommon intracranial glial tumours in which extracranial spread is only rarely reported. Similarly, both intracranial and ocular medulloepitheliomas are also rare, with metastatic spread from the ocular neoplasm being less common than its intracranial counterpart. We report cases of an intracranial oligodendroglioma and an ocular medulloepithelioma both of which metastasised to the parotid gland. To our knowledge these are only the second oligodendroglioma and the third ocular medulloepithelioma to be reported as metastasising to this site.     

Dynamic imaging of intracranial lesions using fast spin-echo imaging: differentiation of brain tumors and treatment effects

The purpose of this study was to develop a technique for differentiating between recurrent brain tumors and treatment-related changes, such as radiation necrosis, using dynamic MRI. Ninety-five patients with intracranial mass lesions were evaluated using T1-weighted fast spin-echo (FSE) MRI at 1.5 T. Pathologies included treatment-related changes (n = 32), primary tumors (n = 41), metastatic tumors (n = 5), meningiomas (n = 4), and mixed primary/treatment related changes (n = 13). Signal enhancement-time curves were analyzed by fitting to a sigmoidal-exponential function. Maximal enhancement rates were calculated as the first derivative of the fitted curve. Based on the maximal enhancement rates, treatment-related changes could be differentiated from primary tumors, metastatic tumors, and meningiomas at the P < .05 confidence level. Lesions of mixed tumor and treatment-related change had intermediate values. Dynamic MRI can be used to differentiate treatment-related changes from primary tumors in previously treated patient populations based on maximal enhancement rates. Individual case studies demonstrate the clinical significance of these findings.     

Angiosarcoma in congenital hereditary lymphoedema (Milroy's disease)--diagnostic beacons and a review of the literature

In cases of congenital lymphoedema the finding of ulceration, violaceous nodules or papules, or apparent traumatic ecchymoses should act as a diagnostic beacon warning of dangers. A case is reported of a high-grade angiosarcoma developing in a patient with congenital hereditary lymphoedema (Milroy's disease). This is the second paper to report this complication, the third case report and the first case in which the diagnosis is substantiated by immunohistochemistry and lectin histochemistry. A review of cases of angiosarcoma complicating congenital hereditary and non-hereditary lymphoedema is also presented.     

Unfortunate in vitro selection of resistant Candida albicans with severe clinical consequences

Cutaneous angiosarcoma is a rare aggressive tumour of capillary and lymphatic endothelial cell origin. It presents as multiple purple and red papules and nodules on the head and neck or the extremities. We report an 86-year-old woman with angiosarcoma arising on her chronically lymphoedematous right leg. The lymphoedema, secondary to chronic immobility, had developed gradually over 40 years. No other family members had lymphoedema. The patient presented with a plaque of friable tumour tissue on the lower right leg and dorsum of the foot, and satellite lesions on the knee and groin which initially appeared to be petechial haemorrhages. The satellite lesions in the groin grew into tumour nodules. There was no evidence of a preceding malignancy, nor any operative intervention to the affected limb or abdomen. Histological examination of all tumour specimens revealed moderately to poorly differentiated angiosarcoma. She died within 5 months of the first appearance of the skin nodules.     

Retrograde intravenous perfusion with cytostatic drugs in angiosarcoma

Case-report of an 82 year old female with an angiosarcoma developing in a venous ulcer and lymphedema. The patient was successfully treated by retrograde intravenous perfusion with vinblastin and vincristin with subsequent blunt removal of the tumor.     

Oncotic aneurysm. Case report

The author describes a case of intracerebral and subarachnoid hemorrhage associated with an intracranial aneurysm caused by a metastatic tumor. The aneurysm formation is explained as being result of tumor cells invading the vessel wall while still preserving the arterial circulation.     

Angiosarcoma of the head and neck managed by a combination of multiple biopsies to determine tumor margin and radiation therapy. Report of three cases and review of the literature

BACKGROUND: Cutaneous angiosarcoma (AS) is a rare, often multicentric vascular tumor of the head and neck region with a rather poor prognosis. The original clinical size of the tumor rarely correlates to the degree of microscopic tissue invasion. Treatment by surgical excision sometimes requires very wide excision. Treatment by radiation or electron beam appears less mutilating but its efficacy is not well documented. OBJECTIVE: To present our experience with a combined surgical delineation of tumor margins followed by radiation treatment. METHODS: We treated three patients with extensive AS of the scalp and face. Prior to radiation, in two cases the tumor margins were determined by grid-pattern punch biopsies. In the third patient, the tumor margins were determined by Mohs mapping system. All three patients then received radiation either by rotational arc electron beam (n = 2) and standard radiation. RESULTS: One patient developed two local recurrences in nonirradiated areas plus a metastatic cervical node, all of which responded to additional electron beam. The patient has no evidence of disease (NED) after 30 months of observation. The other two patients were treated by electron beam and radiation have NED at 5 and 1 years follow-up, respectively. CONCLUSIONS: Local control of AS of the scalp may be achieved by assessment of the tumor margin by peripheral biopsies or Mohs technique followed by electron beam and radiation.     

Diagnostic radiology in the field of traumatology (author''s transl)

Enflurane (Ethrane) was given before operation to 13 neurosurgical patients. 11 of them received halothane for comparison of effects on intracranial pressure (ventricular catheter), blood pressure and central venous pressure during controlled ventilation. Neuroleptanalgesia was used as basic anaesthesia. The results showed that enflurane, when initial pressure levels were between 0-20 mm Hg, had better properties with regard to intracranial pressure than halothane. The intracranial pressure of 6 patients under enflurane rose. Of the remaining patients pressure did not change or even fell. Under halothane, given in a comparable doses, there was always a marked increase of intracranial pressure. If intracranial pressure of one patient under enflurane rose, then the increase by halothane always was more pronounced. The results from 1 patient had to be excluded because of spontaneously occurring plateau waves. Blood pressure was lowered by both agents while central venous pressure remained unchanged.     

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.     

Tumor suppressor genes: at the crossroads of molecular carcinogenesis, molecular epidemiology, and human risk assessment

BACKGROUND AND PURPOSE: Marfan syndrome is a heritable disorder of connective tissue caused by a deficiency of the glycoprotein fibrillin. In several publications and neurological textbooks, a relationship between Marfan syndrome and intracranial aneurysms has been assumed. METHODS: The records of 135 patients classified as having Marfan syndrome who visited the Amsterdam Marfan clinic or were admitted to the departments of neurology and neurosurgery and the records of all patients with a subarachnoid hemorrhage or intracranial aneurysm who visited or were admitted to the departments of neurology and neurosurgery between January 1, 1982, and January 1, 1994, were retrieved. The literature was reviewed regarding Marfan syndrome and intracranial aneurysms. RESULTS: No patient visiting the Marfan clinic had a symptomatic intracranial aneurysm. No patient with Marfan syndrome had been admitted with a ruptured intracranial aneurysm at the departments of neurology or neurosurgery in this period, while during that period 826 patients with symptomatic intracranial aneurysms had been admitted. During follow-up of 129 of the 135 patients with Marfan syndrome (2850 retrospective patient observation years and 581 prospective patient observation years), none presented a symptomatic intracranial aneurysm. The suggested relationship between Marfan syndrome and intracranial aneurysms is based mainly on 10 case reports. However, the diagnosis of Marfan syndrome is doubtful in several of these reports. Several large studies of patients with Marfan syndrome did not mention a ruptured intracranial aneurysm as a clinical manifestation. CONCLUSIONS: We conclude that there is insufficient evidence to presume a relationship between symptomatic intracranial aneurysms and Marfan syndrome on the basis of currently available data.     

Salmonella typhimurium encodes an SdiA homolog, a putative quorum sensor of the LuxR family, that regulates genes on the virulence plasmid

OBJECTIVE: Despite improved systemic control of metastatic breast cancer, the incidence of brain metastases from breast carcinoma continues to rise, in part because most systemically administered agents have poor central nervous system penetration. Therefore, as a method of optimizing drug delivery into the central nervous system, we studied the safety and efficacy of chemotherapy delivered locally via biodegradable polymers in a mouse model of breast carcinoma metastases to the brain. METHODS: The chemotherapeutic agents carmustine (BCNU), carboplatin, and camptothecin were incorporated into controlled release polymers and tested individually against intracranial challenges of EMT-6 breast tumor in BALB/c female mice. For each drug, four groups were tested: Group 1, empty polymer (no drug); Group 2, external beam radiotherapy (XRT) alone; Group 3, local chemotherapy from biodegradable polymer alone; and Group 4, local chemotherapy and XRT together. Polymers were implanted 5 days after intracranial tumor inoculation; XRT was administered on Days 7 through 9 (300 cGy/d). RESULTS: BCNU polymer alone (n = 10; median survival time, >200 d; P < 0.0001) and BCNU and XRT together (n = 10; median survival time, 41 d; P = 0.02) significantly improved survival in mice with intracranial EMT-6 breast cancer in comparison with control animals (n = 20; median survival time, 17 d). Carboplatin and camptothecin, either with or without XRT, and XRT alone did not have any significant effect on survival. CONCLUSION: Local delivery of BCNU with biodegradable polymers can significantly prolong survival in a murine model of intracranial metastatic breast cancer. Surgical resection and placement of BCNU polymers into the resection cavity may decrease the incidence of local recurrence of breast cancer metastases with minimal morbidity.     

Fibromuscular dysplasia associated with intracranial giant aneurysm: report of a case

Although the correlation between fibromuscular dysplasia (FMD) and intracranial aneurysm is well established, the combination of FMD with a giant aneurysm is rare. This paper reports a patient with extracranial FMD associated with a giant intracavernous aneurysm compromising the trigeminal and abducens nerve. A review of the literature uncovered only four documented cases of FMD with concurrent giant intracranial aneurysms. The present case adds further weight to the argument for including FMD in the differential diagnosis list when confronted with a patient with a giant intracranial aneurysm. Absence of adequate collaterals in this patient eliminated ligation as a treatment strategy for the aneurysm.     

Rare intracranial plasmacytoma manifestations. Case reports and review of the literature in diffuse plasmocytoma, in primary solitary extramedullary plasmacytoma in in primary solitary osseous plasmacytoma

Plasmacytomas can be divided into multiple, solitary osseous and solitary extraosseous/extramedullary plasmacytomas. Intracranial plasmacytomas of the dura, leptomeninx and cerebrum are well known from the literature. They are manifestations of multiple myeloma, intracranial extramedullary plasmacytoma or metastatic disease of extramedullary plasmacytoma in distant locations. We describe a cerebellar manifestation of a solitary plasmacytoma of the bone, and a leptomeningeal carcinomatosis of a multiple plasmacytoma. A summary of the literature concerning intracranial plasmacytomas is given. Dural manifestations of plasmacytoma have the same features as meningiomas in CT or MRI. Cerebral or cerebellar manifestations cannot be differentiated from brain tumors by means of CT or MRI. In CT, plasmacytomas show high-density lesions. T2w-MRI reveals a low-intensity lesion. In T1w-MRI, intense homogeneous contrast enhancement can be demonstrated.