Case Of Malignant Transformation Research Articles

A CASE OF A 19 YEAR OLD WOMAN WITH SQUAMOUS CELL CARCINOMA OF THE LUNG

INTRODUCTION: Juvenile Onset Recurrent Respiratory Papillomatosis (JORRP) is a rare condition characterized by recurrent growth of squamous papillomas in the upper and lower airways of children younger than twelve years old. It is caused by the cytopathogenic effect of human papilloma virus (HPV) 6, 11, 16/18 and 31/35/51, which is transmitted from active vaginal lesions of the infected mother at the time of birth. JORRP has a prevalence of 1.7–2.6/100,000 children. It typically presents with hoarseness which can progress to stridor. Multiple surgical interventions are often required to achieve local control of the disease and avoid airway obstruction. Involvement of the upper airway is common, especially the larynx. However, papillomas can also spread to the distal trachea and lung parenchyma in 5% of the cases. Several cases of malignant transformation have been reported in patients with JORRP in the setting of concomitant risk factors such as radiation or smoking. However, only few cases have been published describing malignant transformation of papillomas of the lung in the absence of these risks factors. We present a case of a 19 year-old woman with history of JORRP, who developed squamous cell carcinoma of the lung after being in clinical remission since the age of 5.

Case 144: Dysplastic Cerebellar Gangliocytoma (Lhermitte-Duclos Disease)

A 28-year-old right-handed man presented with longstanding occipital headache, progressive ataxia, and blurred vision. He had begun vomiting 8-10 days earlier. He had no other important history. Neurologic examination revealed average intelligence and was remarkable for cerebellar ataxia. Papilledema was noted at fundoscopy. Magnetic resonance (MR) imaging of the brain was performed. No abnormality was noted on apparent diffusion coefficient maps or T2 * -weighted gradient-echo images (not shown).

Endometriosis-Associated Intestinal Tumours: A Consequence of Long-Term Unopposed Oestrogen?

A case of malignant transformation in an extra-ovarian site, 13 years after total abdominal hysterectomy and bilateral salpingo-oophorectomy, is discussed in a patient on long-term oestrogen implants, which highlights the potential risks surrounding the use of long-term oestrogen replacement therapy.

Mucinous adenocarcinoma arising in ovarian mature cystic teratoma in pregnancy

We report a case of mucinous adenocarcinoma arising from mature cystic teratoma (MCT) of the ovary ascertained incidentally during pregnancy. An ovarian adnexal mass was seen in a 38-year-old pregnant woman during cesarean section. Oophorectomy revealed a mucinous adenocarcinoma arising from MCT with additional capsule invasion. Following this, staging procedures were applied. The patient was staged as IC and adjuvant chemotherapy was applied. She has remained disease-free for over 24 months. To our knowledge, this is a case of mucinous adenocarcinoma arising from MCT and the third case of malignant transformation from MCT in pregnancy in English literature.

Giant Cell Tumour and Central Giant Cell Reparative Granuloma of the Skull: do These Represent Ends of a Spectrum? A Case Report and Literature Review

Giant cell tumour (GCT) of bone is an uncommon primary bone neoplasm typically occurring at the epiphyses of long bones in young adults. They are osteolytic neoplasms with approximate local recurrence rates of 25%, and 2% of patients develop pulmonary metastases. These tumours appear very rarely in the skull, with those few reported cases arising predominantly in the sphenoid and occasionally the temporal bones. They demonstrate benign histological features, but are locally aggressive and surgical excision is the treatment of choice. It is widely believed that giant cell tumours should be distinguished from other giant cell lesions, importantly central giant cell reparative granulomata (CGCG) which are thought to have a lower recurrence rate and for which no cases of malignant transformation or metastases have been reported. Investigators have noted that giant cell lesions in the skull bones may be unique and that GCT and CGCG may be part of a spectrum of a single disease process. We present a case of a giant cell tumour of the temporal bone which illustrates and re-emphasises this concept and review the literature on these lesions.

Implantes peritoneales de tejido glial maduro (gliomatosis peritoneal) y de otros tejidos maduros asociados a teratoma ovárico inmaduro

Peritoneal gliomatosis is a very rare metastatic form of ovarian teratoma, characterized by miliary dissemination of glial tissue inside the peritoneal cavity in patients with an ovarian – usually immature – teratoma. Peritoneal gliomatosis may resemble peritoneal carcinomatosis. Despite peritoneal dissemination, if the glial tissue implants are composed of mature tissue, peritoneal gliomatosis does not adversely affect the prognosis of the primary ovarian teratoma. Consequently, conservative treatment is warranted. The main prognostic factor is the histological grade of the teratoma, which indicates the required complementary treatment. The prognosis of peritoneal gliomatosis is favorable, although cases of malignant transformation have been reported.

Sonographic diagnosis of fibromatosis

To investigate the value of sonography in the diagnosis of fibromatosis. We retrospectively reviewed the sonographic findings, pathologic results, and other medical records of 42 patients with pathologically proven fibromatosis. The tumor's size and location were recorded. Echogenicity, margins, vascularity, and relationship with the adjacent tissue were analyzed. A total of 42 patients with 44 lesions were included in the study. In 21 patients, the tumor was a primary tumor. In 7 of these patients, a preoperative diagnosis of fibromatosis was obtained via sonographically guided biopsy or surgical biopsy. In another 21 patients, the tumor was a recurrence (including 1 case of malignant transformation). Well-defined and regular margins were demonstrated in 26 lesions (59%), whereas poorly defined and irregular borders were seen in the other 18 lesions. On Doppler imaging, vascularity was absent in 66% (29/44) lesions, moderate in 23% (10/44), and marked in 11% (5/44). Infiltration into the adjacent skeletal muscles, cortical bone, or encasement vessels, nerves, or tendons was found in 21 (48%) lesions. In patients with fibromatosis, sonography demonstrates a nonspecific hypoechoic solid mass and can guide the percutaneous needle biopsy.

MUCINOUS LOW-GRADE ADENOCARCINOMA ARISING IN AN INTRACRANIAL ENTEROGENOUS CYST

Enterogenous cysts (ECs) of the central nervous system are developmental malformations that occur in the spinal canal, posterior fossa, or cerebral hemispheres. They are usually benign lesions, and malignant transformation is rare. To date, only three cases of malignant transformation have been reported in the literature. We present a case of a cerebellopontine EC showing foci of epithelial dysplasia and malignant transformation into a low-grade papillary mucinous adenocarcinoma. A 25 year-old man with a 6-year history of hypoacusia presented to our department with facial nerve deficit, visual disturbances, and gait instability. A magnetic resonance imaging scan demonstrated a multiloculated cerebellopontine angle cyst with supratentorial hydrocephalus. A retrosigmoidal approach was used to achieve cyst removal. This was followed several months later by ventriculoperitoneal shunt placement. The cyst was adherent to the brainstem, cranial nerves, and vessels, and it resembled a thin encapsulated structure filled with mucinous-like substance. No solid component was identified. Histopathological examination revealed an EC with foci of malignant transformation in a mucinous papillary adenocarcinoma. Magnetic resonance imaging was performed 5 months postoperatively due to progressive clinical worsening; this scan revealed lesion recurrence with severe brainstem compression. Emergency surgery was performed, and a large decompression was achieved. Subsequent follow-up computed tomographic scans showed progression of the residual tumor. The patient's neurological condition rapidly worsened, ultimately resulting in death. The present report suggests that a careful histological examination of all ECs after surgery should be made to exclude dysplastic foci or carcinomatous transformation. Although the clinical behavior of ECs with malignant transformation is unpredictable, surgery remains the treatment of choice. The use of possible adjuvant chemo- or radiotherapy has not been established.

Sonographie der Halszysten und ihre Differentialdiagnose

Among 22 proven branchiogenic or thyreoglossal cysts, 15 presented with a characteristic pattern of homogeneous smooth reflections within a well-demarcated thin and elastic, deformable wall when using a high-resolution transducer. The other cases, showing an inhomogeneous texture and/or irregular walls, cannot be distinguished from inflammatory or neoplastic processes with central necrosis. Complete surgical removal is recommended especially in these latter cases. One case of malignant transformation was found.

A massive ameloblastic fibro-odontoma of the maxilla

Ameloblastic fibro-odontoma is a rare, benign mixed odontogenic tumor. It occurs exclusively as an intraosseous lesion. It usually has a slow growth and is seen in children and young adults. A painless swelling is the most common clinical sign. Radiographically, ameloblastic fibro-odontoma appears as a circumscribed radiolucency which may contain radiopaque foci. Most cases of ameloblastic fibro-odontoma exhibit benign behavior, but cases of malignant transformation have been reported. The treatment modality in most cases involves conservative surgery, but cases with malignant transformation will require more radical treatment. A massive ameloblastic fibro-odontoma involving the maxilla has been described here with its clinical, radiological, and histopathological features.

Human ageing and telomeres.

Telomerase expression is repressed early in development in all normal somatic human tissues investigated to date, whereas activity and the expression of the RNA component for this enzyme are upregulated in almost all cases of malignant transformation and late-stage cancer. The telomere hypothesis of ageing and immortalization postulates that sufficient telomere loss on one or more chromosomes in normal somatic cells triggers cell senescence, whereas reactivation of the enzyme is necessary for cell immortalization. Measurements of telomere length and telomerase activity in cancer and during normal and accelerated human ageing in skin, blood, haemopoietic, skeletal muscle, vascular and CNS tissues support this model. Tissue culture studies of cell ageing and transformation have added to our understanding of telomere dynamics in these processes. Evolution of telomerase repression and mortality in somatic cells of long-lived organisms is consistent with antagonistic pleiotropy models in which cell senescence is a tumour suppressor mechanism: stringent repression of telomerase has a beneficial early effect in reducing the probability of cancer, but a deleterious, unselected late effect in its contributions to age-related disease.

Polyostotic Paget's Disease with Sarcomatous Change in a Skull Lesion

The most serious complication of Paget's disease is sarcomatous degeneration of pagetic bone. Multifocal sarcomatous degeneration occurs mainly in polyostotic Paget's disease. Paget sarcomas are the most threatening sarcomas of bone irrespective of the histopathology, their prognosis being far more negative than primary sarcomas. Paget's disease is a common entity but osteosarcoma arising in Paget's disease is an infrequent complication. The skull involvement is even rarer. We report a case of malignant transformation of a skull lesion in a patient of polyostotic Paget's disease. Skull radiographs, computed tomography and magnetic resonance imaging scans of the tumour area show interesting results.

Benign solitary schwannomas

We reviewed 234 benign solitary schwannomas treated between 1984 and 2004. The mean age of the patients was 45.2 years (11 to 82). There were 170 tumours (73%) in the upper limb, of which 94 (40%) arose from the brachial plexus or other nerves within the posterior triangle of the neck. Six (2.6%) were located within muscle or bone. Four patients (1.7%) presented with tetraparesis due to an intraspinal extension. There were 198 primary referrals (19 of whom had a needle biopsy in the referring unit) and in these patients the tumour was excised. After having surgery or an open biopsy at another hospital, a further 36 patients were seen because of increased neurological deficit, pain or incomplete excision. In these, a nerve repair was performed in 18 and treatment for pain or paralysis was offered to another 14. A tender mass was found in 194 (98%) of the primary referrals. A Tinel-like sign was recorded in 155 (81%). Persistent spontaneous pain occurred in 60 (31%) of the 194 with tender mass, impairment of cutaneous sensibility in 39 (20%), and muscle weakness in 24 (12%). After apparently adequate excision, two tumours recurred. No case of malignant transformation was seen.

Ungewöhnlicher Kombinationsbefund eines zystischen Ovarialtumors mit einer Leiomyomatosis peritonealis disseminata

Leiomyomatosis peritonealis disseminata (LPD) is a rare disease, which is most often found in women in reproductive age - seldom pre- and postmenaupausal. LPD has been described in women taking oral contraceptives or during pregnancy. LPD is mostly asymptomatic and discovered during operations like section caesarea, etc. Because of the macroscopic aspect (multiple small nodules <2 cm) a metastasing tumour is suspected during operation. These nodules consist of smooth muscle fibres and show benign histologic features. An increased level of cases with similar endometriosis (10%), myoms of uterus or functional ovarian tumours have been reported. Few cases of malignant transformation have also been described. In the pathogenesis of LPD a metaplastic change of submesothelial multipotential mesenchymal cells as an abnormal response on hormonal stimulation is debated. A regression of LPD after delivery or after stopping oral contraceptives is possible. Another therapeutic option is the application of GnRH-agonists. We present a case of a 35-year old female patient with sudden abdominal pain and suspicious findings in abominal ultrasound and CT-abdomen. The intraoperative aspect shows diffuse intraabdominal nodules consisting of smooth muscle fibres and a cystic left ovary. LPD, endometriosis and a borderline lesion of the left ovary were diagnosed by intraoperative histological examination. A bilateral salpingo-oophorectomy and omentectomy were performed.

Mucinous Nonneoplastic Cyst of the Pancreas: A Novel Nonneoplastic Cystic Change?

Cystic lesions and neoplasms of the pancreas are uncommon, but they are of special interest because they can usually be cured by resection. During the last decade, the spectrum of these tumors has increased considerably. We present a series of five cystic lesions of the pancreas that differ from all categories described so far. The patients affected by these tumors were three men and two women (mean age, 57 y). Four lesions were unifocal and involved the head of the pancreas; one was multifocal and involved the pancreatic head and tail. Grossly, these tumors presented as unilocular or multilocular thin-walled cysts that contained turbid fluid, or, in two cases, blood, and lacked any communication with the duct system. Microscopically, the cysts were lined by cuboidal to columnar mucin-producing cells, supported by a small band of dense fibrous stroma. Immunocytochemically, the epithelial cells were positive for cytokeratins 7, 8, 18, 19, and 20 (except one), and Ca 19-9 but were negative for trypsin, CEA, synaptophysin, chromogranin A, calretinin, and α-inhibin. In four of the five lesions, the epithelial cells expressed MUC5AC, and in one of the five, MUC1. MUC2 and MUC6 were not expressed in any of the lesions. The stromal cells lacked the nuclear progesterone positivity that is typical of mucinous cystic neoplasms. During a mean follow-up period of 2 years, there were no recurrences or cases of malignant transformation after resection. The results suggest that these cystic lesions are distinct from mucinous cystic neoplasms, the most important entity in the differential diagnosis. Because they may represent a nonneoplastic cystic change of the pancreas, we propose the descriptive term mucinous nonneoplastic cyst for these tumors of unknown pathogenesis.

Leiomioma de uretra femenina

Leiomyoma of the female urethra is a benign mesenchymal tumour highly infrequent in the clinical practice. There is controversy as to its degree of oestrogen hormone dependency and its diagnosis is reached only after pathohistological study of the resection specimen. This type of tumour has excellent prognosis, with few tumoral relapses and no case of malignant transformation having been reported for the time being. Contribution of one case report of leiomyoma of the female urethra with typical clinical-epidemiological features but very uncommon from a topographic insight, as it was located in the anterior side of the distal urethra.

Malignant transformation of craniopharyngioma

Craniopharyngioma is a well recognised intracranial tumour; malignant transformation has been rarely described. In that case, malignant change occurred following irradiation for local recurrence and information is not provided on the biological behaviour of the tumour or the response to management. We report a further case of malignant transformation in recurrent craniopharyngioma following radiotherapy. The biological behaviour of malignant change in craniopharyngioma is uncertain so the treatment modalities require consideration of toxicity and other adverse sequelae balanced against anticipated outcome and comorbidities from previous treatments. The pathophysiology and therapeutic strategies are discussed. We review the possible pathogenic link between radiation therapy and malignant transformation.

Malignant transformation in pleomorphic xanthoastrocytoma–cfa report of two cases

Pleomorphic xanthoastrocytomas (PXA) with malignant transformation are reported in two adult men with a long history of seizures, recent onset of neurological symptoms and superficially located, temporal lobe lesions. Although PXA is generally described as having relatively benign behaviour, this report adds two further cases of malignant transformation to the literature.

Ectopic Cervical Thymus: Case Report

Ectopic cervical thymus is rarely considered in the differential diagnosis of cervical masses: this lesion is essentially asymptomatic and generally occupies a position in the neck along the carotid sheath, underneath the sterno-cleido-mastoid muscle. It is supposed that most of these masses arise as a consequence of migration defects during glandular embryogenesis. Ectopic thymus rarely invades contiguous structures but in the literature some cases of malignant transformation of aberrant cervical thymus have been reported. Some non-invasive investigations (MRI, ultrasonography) are useful but accurate diagnosis depends eventually on surgical excision and histologic examination.

A case of malignant transformation in thoracic vertebral hemangioma following repetitive irradiation and extraction

We report a rare case of thoracic vertebral hemangioma which developed into angiosarcoma during the course of repetitive operations and irradiation. A 44 year old female was operated on for hemangioma of the first thoracic vertebra. The diagnosis of hemangioma was confirmed histopathologically with the specimen from the first operation. The tumor developed multiple lesions later in the clinical course after the first operation, these lesions were removed in four consecutive operations and each histological diagnosis was that of hemangioma. Throughout the period of these operations, the patient was treated with steroid, and with radiotherapy simultaneously. The patient underwent the fifth operation for the recurrence of the tumor on 26 March 1990, and the histopathological diagnosis was not hemangioma but hemangiosarcoma which was considered a malignant transformation. The tumor cells immunohistochemically revealed positive staining with UEA-I, Factor-VIII, as the tumor immunohistochemically showed a vascular endothelioid nature.