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PubMed2006

[Thoracic findings in neurofibromatosis].

Neurofibromatosis type 1 (von Recklinghausen' disease) is an autosomal dominant hereditary syndrome. It is characterized with multiple light brown (café-au-lait) spots, Lisch nodules and neurofibromas. Thorax and lungs are affected in various forms. Four cases with symptoms of thoracic involvement were investigated in our clinic. Mean age was 46. All cases had dyspnoea and cough; two of them had chest pain. Skin lesions of neurofibromatosis type 1 (NF 1) were pathologically confirmed in all cases. Moreover, case 3 had diffuse interstitial fibrosis and honeycomb pattern. Case 2 had thorax deformity, kyphoscoliosis and intrathoracic benign mass. Case 1 had two neurofibrosarcoma masses on the right hemithorax. Case 4 had multiple intrathoracic neurofibromas. Cases 1 and 3 died within two years after diagnosis due to malignancy and respiratory failure, respectively. Cases 2 and 4 are still under our control.

Cough↗

PubMed1991

[The classification of atypical forms of neurofibromatosis].

Neurofibromatosis (NF) represents not an entity but a group of several forms which differ as to symptoms, prognosis and inheritance. In 1982 Riccardi suggested a classification into eight categories. Two of these--Von Recklinghausen NF-1 and bilateral acoustic NF-2- were defined in 1987 by a National Institutes of Health Consensus Development Conference. We describe 13 patients whose symptoms do not fit the diagnostic criteria for NF-1 and NF-2. 6 subjects can be classified as segmental NF-V. One of these patients was remarkable in having iris hamartomata, while his daughter was affected with NF-1. Another (female) patient in this group had areolar freckling, a finding not yet reported in NF patients. 4 cases belong to the late-onset NF-VII category, 3 of whom developed a neurofibrosarcoma. Two subjects are fist-degree relatives (mother and son). We are not aware of familial occurrence of NF-VII. Two further subjects were assigned to the variant form of NF, NF-IV. One patient had symptoms similar to NF-2 but the minimal diagnostic criteria were not fulfilled. Nevertheless, we consider his classification as NF-2 in view of a pattern of intracranial calcification repeatedly and exclusively found in NF-2. In general, assignment to a category of NF is important for appropriate patient management and genetic counselling. Classification of individual patients may be arbitrary at the time.

Adolescent↗

PubMed1990

Neoplasms of the peripheral nervous system.

Neoplasms of the peripheral nervous system arise from the cellular sheath surrounding the nerve trunks, that is, the pluripotential Schwann cells and related cells, and rarely affect the feet. When present, they are most frequently associated with the autosomal dominantly inherited neurofibromatosis 1. This condition has been related to chromosome 17, and it appears, from in vitro experiments, to involve defects in tyrosine metabolism. Hence, the most common neoplasm is the neurofibroma. Distinct criteria have been established for a diagnosis of neurofibromatosis 1, so that a single pedal neurofibroma may not represent this complex. However, if the complex is present, it is necessary to consider the possibility of malignant transformation to a neurofibrosarcoma. Although malignant peripheral nerve neoplasms are extremely rare in the feet, they may arise in the context of neurofibromatosis 1, or independently. Other benign or malignant schwannomas may (rarely) also arise in the feet. Surgical excision of benign lesions, according to established standards for tumor surgery, is usually curative, but a detailed personal and familial history, along with adjunctive radiologic procedures and biopsy, is necessary to determine the nature of the lesion. Since (endocrine and) other abnormalities may complicate neurofibromatosis 1, surgical procedures must not be undertaken until the patient has been medically cleared and is carefully monitored. A high mortality rate is associated with malignant peripheral neurogenic tumors, especially those arising in the context of neurofibromatosis 1. It should be recalled that neurologic manifestations in the foot may represent non-neoplastic conditions as well as peripheral nerve tumors (or other tumors involving those nerves) that are proximal to the foot and ankle area.

Adult↗

PubMed1986

Detection of N-myc oncogene expression in human neuroblastoma by in situ hybridization and blot analysis: relationship to clinical outcome.

We studied N-myc oncogene expression in 13 human neuroectodermal tumors and one teratoma by in situ hybridization. In four of six neuroblastomas, there was increased N-myc expression (15 to 49% of the cells). Many of the primitive neuroblastic cells had an increase of N-myc RNA not observed in the larger, more differentiated cells. Two neuroblastomas matured to ganglioneuromas; no biopsies performed during this progression expressed increased N-myc RNA. Three ganglioneuroblastomas, two tumors presenting as ganglioneuromas, a cerebral neuroectodermal tumor, a neurofibrosarcoma, and the teratoma did not have increased N-myc expression. The data obtained by in situ hybridization correlated well with data obtained by blot analysis. Neuroblastomas/ganglioneuroblastomas with a favorable course did not have appreciable elevation of N-myc expression over 10 to 77 mo of follow-up; thus N-myc may not be involved in the maintenance of the neoplastic state. However, such tumors with a fatal outcome 2 to 14 mo after diagnosis usually had elevated N-myc expression. These findings suggest a relationship between elevated levels of N-myc RNA and poor prognosis.

Brain Neoplasms↗

PubMed1989

Incidence, types, and management of cancer in patients with neurofibromatosis.

Neurofibromatosis Type 1 (NF1) is a common genetic disease with an incidence of 1 in 3,000 to 4,000. Clinical manifestations may include cafe-au-lait macules, axillary freckling, neurofibromas, Lisch nodules, skeletal dysplasias, learning disabilities, and a variety of neoplasms. In persons with NF1, some malignancies occur at an increased incidence compared to the general population. These include central nervous system astrocytomas, especially involving the visual pathways; ependymomas; meningiomas; neurofibrosarcomas; rhabdomyosarcomas; nonlymphocytic leukemias; and pheochromocytomas. The authors discuss the dysplastic and malignant features associated with NF and the concept of NF as a "neurocristopathy."

Adult↗

PubMed1989

Neurofibromatosis update.

This paper presents a review dealing with three major aspects of a modern understanding of the disorders known collectively as the neurofibromatoses (NF). In one section, the focus is on NF heterogeneity, clarifying the distinction between NF-1, NF-2 and atypical forms of NF. In another section, the focus is on NF-1 neural-crest tumors, with particular emphasis on the four types of neurofibromas (cutaneous, subcutaneous, nodular plexiform and diffuse plexiform), schwannomas, astrocytomas and neurofibrosarcomas. In the final section, focus is on NF-1 nontumor features, both as a clinical guide and as a reminder of the breadth of concerns that a pathogenetic schema will have to consider.

Adrenal Gland Neoplasms↗

PubMed1989

Clear cell sarcoma. An immunohistochemical analysis of six cases and comparison with other epithelioid neoplasms of soft tissue.

Clear cell sarcoma (CCS) is an uncommon, clinicopathologically distinct neoplasm that typically arises in association with tendons and aponeuroses. It shares several histologic and ultrastructural features with malignant melanoma. Clear cell sarcoma occasionally may be confused with other tumors of soft tissue that have a predominantly epithelioid appearance, including epithelioid leiomyosarcoma, epithelioid neurofibrosarcoma, synovial sarcoma, and epithelioid sarcoma. To assess the potential contribution of immunohistochemistry to this differential diagnosis, a panel of immunostains was applied to examples of each of these neoplasms. All six CCSs contained vimentin, and five were reactive with the melanoma-specific monoclonal antibody HMB-45. In addition, five CCSs expressed neuron-specific enolase, four cases displayed S100 protein, and four examples contained LN3 antigen. Synaptophysin and Leu-7 antigen were present in one case each. Cytokeratin, epithelial membrane antigen, carcinoembryonic antigen, desmin, muscle-specific actin, and leukocyte common antigen were invariably absent. No other primary epithelioid neoplasm of soft tissue reacted with HMB-45. Clear cell sarcoma could be separated from epithelioid leiomyosarcoma by the presence of desmin and muscle-specific actin in the latter neoplasm. Similarly, both synovial sarcoma and epithelioid sarcoma differed from CCS by their expression of cytokeratin and epithelial membrane antigen. Clear cell sarcoma and malignant melanoma were immunohistochemically indistinguishable, supporting the concept that they share a common pattern of differentiation.

Adolescent↗

PubMed1989

Phase II evaluation of doxorubicin for treatment of various canine neoplasms.

One hundred eighty-five dogs with histologically confirmed, measurable malignant tumors were used in a prospective study to determine the response to 2 doses of the anthracycline antitumor antibiotic, doxorubicin. Eighty-three dogs had been refractory to one or more previous treatment modalities (surgery, n = 54; chemotherapy, n = 22; radiation, n = 10; hyperthermia, n = 1; biological response modifier, n = 1). The extent of neoplastic disease was determined immediately prior to and 3 weeks after 2 doses of doxorubicin were administered (30 mg/m2 of body surface area, iv) 21 days apart. Eighty-four percent (n = 157) of the dogs received 2 doses of doxorubicin and were evaluated. Of the 28 dogs ruled ineligible, 4 had serious side effects to the first dose of doxorubicin, and 24 others acquired complications resulting from their malignant tumors. A partial or complete remission was obtained in 41% (64/157) of all evaluable dogs: 26% (11/43) of the dogs with carcinoma, 67% (42/63) of the dogs with lymphoma, and 22% (11/51) of the dogs with sarcoma. Tumors in which there was at least a 50% volume reduction (partial or complete remission) included malignant lymphoma (42/63), fibrosarcoma (1/14), solid follicular thyroid carcinoma (3/13), mammary adenocarcinoma (2/8), hemangiosarcoma (2/8), osteosarcoma (1/6), circumanal carcinoma (3/5), synovial cell sarcoma (2/3), undifferentiated sarcoma (2/3), nasal adenocarcinoma (1/2), liposarcoma (1/2), infiltrating lipoma (1/1), malignant melanoma (1/1), sclerosing mesothelioma (1/1), and neurofibrosarcoma (1/2).(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗

PubMed1989

Sacral and presacral tumors: problems in diagnosis and management.

We reviewed 9 cases of sacral tumors with presacral extension. These included 2 chordomas, 1 metastatic renal cell carcinoma, 2 schwannomas (1 malignant, 1 benign), 1 neurofibroma, 1 neurofibrosarcoma, 1 aneurysmal bone cyst, and an exceedingly rare meningioma. The sex of the patients was not significant. The age of the patients at diagnosis ranged from 13 to 68 years (mean, 47 years). Initial symptoms of low back and radiating leg pain were present in all but 1 patient. The duration of symptoms prior to diagnosis ranged from 1 month to 9 years (mean, 2.6 years). A delay in diagnosis of 2 years or more occurred in 6 of the 9 patients. Progressive perineal numbness and/or sphincter dysfunction were seen in 6 patients, and a palpable rectal mass was noted in 6 of 9 patients. The efficacy of various diagnostic tests is presented, as are the surgical options--needle biopsy and anterior and posterior approaches. Despite improved radiographic imaging techniques, these unusual tumors are often diagnosed at an advanced stage, and may masquerade as discogenic radiculopathy. Late diagnosis contributes to the difficulty of surgical extirpation. Anterior and posterior surgical approaches involving general, orthopedic, and urological surgeons may be required.

Adolescent↗

PubMed1989

Interleukin-6 immunoreactivity in human tumors.

The cytokine, interleukin-6 (IL-6), has emerged as a likely mediator of many of the systemic alterations observed in patients with cancer (fever, increased erythrocyte sedimentation rate, and alterations in plasma protein composition) and may also mediate local effects such as alteration in proliferation of tumor cells, increased tumor cell motility, and decreased intercellular adhesions between tumor cells. The distribution of IL-6 immunoreactivity in different human tumors was studied. IL-6 immunoreactivity was detected by the avidin-biotin-complex (ABC) procedure using a polyclonal rabbit antiserum raised against an E coli-derived human IL-6 (rIL-6). Preimmune rabbit serum used as a control did not yield specific staining and preadsorption of the IL-6 antiserum with rIL-6 abolished specific staining. Strong-to-moderate IL-6 immunoreactivity was observed in the neoplastic elements present in primary squamous cell carcinomas, in adenocarcinomas of mammary, colonic, ovarian, and endometrial origin, in various adenocarcinomas metastatic to lymph nodes, and in soft tissue tumors including leiomyosarcoma and neurofibrosarcoma. Weak-to-moderate IL-6 immunostaining was observed in Hodgkin's and non-Hodgkin's lymphomas. This study demonstrates that most human tumors stain positively for IL-6, adding weight to the hypothesis that IL-6 is a key cytokine that participates in the host-tumor interaction.

Cell Line↗

PubMed1989

Anchoring fibrils in a spindle cell squamous carcinoma.

To the best of our knowledge anchoring fibrils in tumours have been reported to occur only in cutaneous cylindromas and squamous cell carcinomas of oral mucosa and larynx. In this paper we describe a difficult-to-diagnose malignant spindle cell tumour where the striking feature was the presence of large numbers of anchoring plaques and anchoring fibrils in the tumour matrix. On the basis of light microscopic and immunohistochemical studies several diagnoses were preferred, such as malignant fibrous histiocytoma, neurofibrosarcoma, malignant schwannoma, desmoplastic melanoma and spindle cell squamous carcinoma (more fully referred to as 'spindle cell variant of squamous cell carcinoma'). The presence of anchoring fibrils in this tumour strongly supports the diagnosis of spindle cell squamous carcinoma. Our knowledge about the occurrence of anchoring fibrils in tumours is scant, perhaps because they are likely to be missed unless they are present in large numbers, or perhaps because they are mistaken for native collagen fibrils. It seems to us that a systematic search for anchoring fibrils in tumours is warranted, for knowledge about their occurrence or absence in various types of tumours may add yet another diagnostic marker in the armamentarium of the diagnostic electron microscopist and it may also assist in resolving the histogenesis of some controversial neoplasms.

Actin Cytoskeleton↗

PubMed1989

Spontaneous tumours of the European hamster (Cricetus cricetus L.).

The incidence and spectrum of spontaneously occurring neoplasms was evaluated in 285 European hamsters (strain Mhh:EPH) ranging in age between 1 and 5 years. The mean lifespan of the 107 male and 135 female hamsters examined amounted to 122 and 135 weeks, respectively. The overall incidence of tumour-bearing animals was 51.2% (males: 57.9%, females: 47.2%). Malignant tumours were more frequent than benign neoplasms and affected more males than females. Neoplasms of the haematopoietic/lymphoreticular system were the most common tumours (males: 16.8%, females: 13.5%). In males, these were followed in a decreasing order of incidence by pheochromocytomas (15.9%), malignant schwannomas (8.4%) and tumours of the prostate (6.5%). In females, pheochromocytomas as well as granulosa cell tumours were the second commonest tumour types (6.2% each), followed by malignant schwannomas (5.1%). Other tumours did not exceed the 5% incidence level. Two unusual rare tumours were a locally invasive cementoblastoma arising in the maxilla and a carcinosarcoma of the skin with components of a squamous cell carcinoma and a neurofibrosarcoma. The results of the study are compared with data on the Syrian and Chinese hamster.

Animals↗

PubMed1985

Primary sarcoma of the adult vagina: a clinicopathologic study.

Combination chemotherapy has dramatically improved the results of treatment for embryonal rhabdomyosarcoma of the vagina in children, but little attention has been directed toward vaginal sarcomas in adults. This report of 17 cases of primary sarcoma of the adult vagina includes ten leiomyosarcomas, four mixed mesodermal tumors, one undifferentiated sarcoma, one stromal sarcoma, and one neurofibrosarcoma. The leiomyosarcomas, mixed mesodermal tumors, and the stromal sarcoma were morphologically similar to their uterine counterparts. Thirty-five percent of the patients had received pelvic irradiation for carcinoma of the cervix. Three of four patients treated by pelvic exenteration are alive and disease free after 84 to 161 months of observation. All 13 patients who received other forms of primary therapy died of recurrence. The pelvis was the first site of recurrence in all treatment failures and the only site of failure in 50%.

Adenocarcinoma↗

PubMed1986

Neurofibromatosis in children.

The clinical diagnosis of neurofibromatosis in childhood will usually be based on the presence of numerous café-au-lait spots. Early diagnosis allows for continuing follow-up and appropriate counselling. Symptomatic therapy can be provided if necessary. The disorder has a tendency via its mesodermal route to affect almost every system in the body; however, few laymen have even heard of the disorder and, except for the "Elephant Man" notoriety, are totally unaware of it, whereas muscular dystrophy, cystic fibrosis, and Down syndrome although occurring less frequently are well known to the general public. The management of neurofibromatosis in children covers an extremely wide spectrum: at times the management appears to be simple, involving little more than clinical evaluation and simple investigations. However, in view of the protean manifestations of the condition, a complete history including family history is obligatory, and investigation must include radiographic studies of the abdomen, chest, spine, and skull, the latter to include special views of the orbits and optic foramina. My investigation of this disorder has been extremely frustrating because of the progressive character of the disease. Nothing seems to alter the natural course of the disease. I cannot say that my investigative efforts have revealed any breakthroughs in treatment. An aggressive surgical approach to the myriad of lesions associated with this disease, especially neuromata or segmental problems, is probably advisable. The early treatment of tibial pseudarthrosis by polyprophylene orthotic and pulsating electromagnetic fields shows encouraging results over the short course, although I am not so sure as to whether or not the patients would do as well with the custom fit orthotic with or without the electronics. Early stabilization of spinal deformity has proven to be more than moderately successful and is strongly recommended following appropriate intraspinal evaluation. The management of tumors of the brain and spinal cord, as well as those associated with limb hypertrophy and congenital tibial pseudarthrosis, is undergoing innovations at this time which may result in a better cure rate. Procedures include the use of CT to evaluate tumors [Coleman et al. have attempted to differentiate neurofibromas from neurofibrosarcoma by contrast enhancement methods], the use of CO2 lasers to remove previously inoperable CNS tumors, microvascular bone transplantation and pulsating electromagnetic field to treat pseudarthrotic bones.4+he National

Bone Diseases↗

PubMed1987

Neurofibromatosis.

Neurofibromatosis (NF) is an inherited disorder characterized by the development of a wide variety of clinical manifestations, including characteristic "freckle-like" pigmentations (cafe au lait spots) that develop in infancy, followed by skin tumors that may vary widely in size, number, and distribution. In addition to skin tumors, bone, neurologic, and endocrine abnormalities are common. NF is recognized in eight different types, with clinical heterogeneity being the hallmark of this disease. Malignant degeneration of the tumor to neurofibrosarcoma is rare. Early diagnosis, genetic counseling, treatment of symptoms, and appropriate surgery are the tools available for management of NF patients. Surgical procedures generally involve judicious subtotal resection, combined with reconstructive efforts aimed at functional improvement and cosmesis.

Adult↗

PubMed1987

[Incidence of the degeneration of cutaneous nerve tumors in Recklinghausen's disease].

A retrospective study was conducted in 85 patients with clinically certain von Recklinghausen's neurofibromatosis seen between 1972 and 1982. A similar study was conducted in 105 patients without neurofibromatosis observed during the same period who underwent excision of a cutaneous neurofibroma or schwannoma. Thirty-nine of the patients with neurofibromatosis never had surgical treatment for a nervous skin tumour; 46 had one or several tumours excised for diagnostic purposes or for cosmetic reasons. More than 300 tumours had been removed in these 46 patients. The post-operative follow-up period is now at least 2 years for all patients, 5 years for 85 p. 100 of them and more than 10 years for one-third. Only one case of malignant nervous skin tumour was observed in a patient who never had surgical treatment; it was a neurofibrosarcoma which resulted in death within two years. Two other deaths related to neurofibromatosis were recorded: one after surgical treatment of a duodenal schwannoma, the other due to septicaemia after infection of ulcers on a giant plexiform neurofibroma. Three other deaths unrelated to neurofibromatosis occurred. Local relapses without suspicion of malignancy were observed in 8 of the 46 surgical patients; 3 cases concerned large plexiform neurofibromas of the face; excision was histologically incomplete in most cases with or without relapse. No case of malignancy was recorded in the follow-up of the 105 patients without neurofibromatosis but with nervous skin tumours.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

PubMed1986

[Surgical management of soft tissue sarcoma].

From 1964 to 1978, 313 patients with soft tissue sarcoma were treated in our hospital by resection and followed over 5 years. 192 patients were males and 121 were females. The sex ratio was 1.5:1. In the 190 cases who developed postoperative recurrence, 55 were in the head and neck, 136 in the trunk and 122 in the limbs and 23 (7.4%) as metastasis in the lymph nodes. By pathology, the common types were fibrosarcoma (86 cases), neurofibrosarcoma (66 cases) and rhabdomyosarcoma (32 cases). All the 313 patients were treated by resection, the overall 5 and 10 year survival rates were 47.6% and 35.8%. The 5 year survival rate was 47.9% in the 117 patients treated by local resection, 62.7% in the 75 by extensive resection, 18.6% in the 43 by amputation and 48.7% in the 78 by combination of resection and irradiation. According to the UICC TNM staging system, the 5 year survival was 60% in stage I, 46.9% in stage II, 40% in stage III and 22.2% in stage IV. In this series, discussion of the treatment modalities and prognosis factors in emphasized.

Adolescent↗

PubMed1985

Phase I clinical evaluation of diaziquone in childhood cancer.

Diaziquone (AZQ), a new lipid-soluble antitumor agent, was given by 15-30-minute infusion on a daily X 5 schedule to 47 children with refractory solid tumors and leukemia. The starting daily dose of 6 mg/m2 was escalated to 10 and 35 mg/m2 in patients with solid tumors and leukemia, respectively. In patients with solid tumors, myelosuppression was dose-limiting at a daily dose of 10 mg/m2. In patients with leukemia, prolonged pancytopenia and bone marrow hypoplasia were observed at daily doses greater than or equal to 25 mg/m2. At these higher doses, significant hyperbilirubinemia associated with sepsis was also seen. Corresponding increases of transaminases or alkaline phosphatase and significant hemolysis were not noted. The maximum tolerated dose for this daily dose schedule was 9 mg/m2 in children with solid tumors and 25 mg/m2 in children with relapsed leukemia. Responses to AZQ included stabilization of disease in osteosarcoma, neurofibrosarcoma, pinealoma, and ependymoma. A patient with juvenile chronic myelocytic leukemia in blast crisis converted back to the chronic phase. A patient with acute lymphoblastic leukemia had a substantial decrease in cerebrospinal fluid blast count. Bone marrow aplasia was achieved in children with acute lymphoblastic leukemia and acute nonlymphoblastic leukemia; however, remissions were not achieved. A phase II study of AZQ in children with refractory malignancies is now being performed by the Childrens Cancer Study Group.

Adolescent↗