Primary central nervous system lymphoma of the brain stem responding favorably to radiosurgery: a case report and literature review.

OBJECTIVE: To explore various therapeutic alternatives and evaluate the clinical results of patients with primary central nervous system lymphoma (PCNSL). We report a case of PCNSL with involvement of the brain stem managed with radiosurgery using the Leksell gamma knife as the treatment modality. CASE DESCRIPTION: A 55-year-old white woman presented with complaints of weakness on the left side of her body and double vision associated with a frontal headache. Nervous system examination revealed right-sided oculomotor palsy and left-sided motor paresis, grade 2/5, in both upper and lower limbs. Her Karnofsky performance score was 50. A contrast-enhanced magnetic resonance imaging scan of the brain revealed an irregular space-occupying lesion in the pons that enhanced uniformly with gadolinium. A frame-based stereotactic biopsy was performed revealing a B-cell malignant lymphoma. Subsequently, stereotactic radiosurgery using the Leksell gamma knife was performed. A dose of 11 Gy was delivered to the tumor margin. A follow-up magnetic resonance imaging scan after 2 months revealed a 50% reduction in the size of the enhancing lesion. CONCLUSION: Our own results and limited evidence from the literature suggest stereotactic radiosurgery as a potentially safe and effective treatment option in patients with PCNSL.

Histiocytic sarcoma involving the central nervous system: clinical, immunohistochemical, and molecular genetic studies of a case with review of the literature.

Histiocytic sarcoma (HS) is a rare disease, and there has been much confusion concerning the diagnostic criteria for this entity. Since immunohistochemical and cytogenetic techniques have become more universally available, many cases initially diagnosed as histiocytic sarcoma have been reclassified as other diseases. We describe a case of HS that presented as a single mass lesion in left occipital lobe. At autopsy the tumor also involved the meninges as a thick exudate. Histologic examination showed numerous large pleomorphic malignant cells with areas of necrosis, numerous neutrophils, and phagocytosis by tumor cells. Immunohistochemically, the tumor cells stained positively with antibodies directed against most histiocytic markers and did not stain with antibodies directed against myeloid markers, dendritic markers, CD30, ALK1, or other lymphoid markers. Molecular cytogenetic analysis showed no rearrangement [i.e. t(2;5) translocation or other variant] by fluorescence in situ hybridization. The T-cell receptor-gamma chain by multiplex polymerase chain reaction showed a polyclonal pattern. No heavy or light chain gene rearrangements were found. To our knowledge, this is the first reported autopsy case of this rare entity primarily involving the brain and meninges.

Differentiated thyroid carcinoma with functional autonomy.

OBJECTIVE: To present a case of papillary carcinoma in an autonomously hyperfunctioning thyroid nodule. METHODS: We chronicle the clinical and laboratory findings in a patient with a painless neck mass, with a particular focus on the pathologic findings after surgical removal of the right thyroid lobe. RESULTS: A 39-year-old woman had an enlarging nodule of the right thyroid lobe. Results of thyroid function tests suggested subclinical hyperthyroidism. Two months later, the patient complained of increasing swelling in the neck (but still had no symptoms suggestive of hyperthyroidism). Thus, resection of the right thyroid lobe was performed. Pathologic analysis disclosed low-grade papillary thyroid carcinoma within the nodule, with a small rim of compressed inactive-appearing thyroid tissue surrounding the nodule. Subsequently, she underwent total thyroidectomy and follow-up care for thyroid carcinoma. CONCLUSION: Although solitary hyperfunctioning nodules of the thyroid gland are usually considered benign, the current case suggests that the diagnosis of autonomous thyroid nodules does not preclude thyroid carcinoma in a functioning nodule.

Synovial metaplasia, a specialized form of repair.

Synovial metaplasia is a change seen most frequently in the tissues surrounding silicone breast prostheses and in healing tissue adjacent to joint prostheses. It has also been described in skin and soft tissues, most frequently in healing or healed traumatic or surgical wounds. We report a case of synovial metaplasia occurring in a hitherto unreported location, namely, adjacent to a silicone low-pressure voice prosthesis. A review of cases of synovial metaplasia reported in the literature revealed that in most cases, spaces that form adjacent to foreign material (most commonly silicone breast prostheses) and the smooth gliding surfaces of the foreign material that resist penetration by fibroblast processes are frequent associated findings that precede the occurrence of synovial metaplasia. Thus, synovial metaplasia might represent a specialized form of healing in cases that have this combination of physical features.

Devic disease: clinical course, pathophysiology, and management.

Devic disease is characterized by an attack or attacks of optic neuritis and necrotizing myelitis without clinical or magnetic resonance imaging (MRI) evidence of brain involvement or presence of oligoclonal bands in the cerebrospinal fluid (CSF). More than a century after its first systematic study by the French physician, Eugene Devic, its cause as well as its pathogenesis, remains unknown. Devic disease is both a diagnostic and therapeutic challenge to clinicians. The authors present the latest review on the nature, course, possible mechanisms, and therapeutic options of Devic disease.

Aggressive fibromatosis of the parapharyngeal space: two cases and treatment recommendations.

Aggressive fibromatosis is an uncommon tumor that is locally aggressive but not malignant. Therefore, its classification falls between the benign and malignant neoplasms of soft-tissue origin. In the past, these lesions were misdiagnosed as low-grade fibrosarcomas. Otolaryngologists should be familiar with these lesions because as many as 15% of them occur in the head and neck. The treatment of choice is wide surgical excision, which is often difficult. Postexcision recurrence rates are high. Nonsurgical treatment includes radiation and chemotherapy, both of which are usually reserved for recurrences. We describe two cases of aggressive fibromatosis of the parapharyngeal space, and we review the available treatment options.

Pilocytic astrocytoma presenting as an intrinsic brainstem tumor: case report and review of the literature.

OBJECTIVE: Pilocytic astrocytomas are typically circumscribed, slow growing tumors found in children and young adults. This type of tumor can arise from various locations in the neuraxis. In the brainstem, pilocytic astrocytomas grow as dorsal exophytic masses. To our knowledge, pilocytic astrocytomas arising entirely within the brainstem have not been reported in the literature. CLINICAL PRESENTATION: A 22-year-old man presented with double vision for three months. A neurological exam revealed a right sixth nerve palsy. INTERVENTION: Magnetic resonance imaging revealed an intra-axial mass with a cystic center arising from within the brainstem. Intraoperative findings confirmed an intra-axial brainstem tumor. Histological analysis revealed that the tumor was a low-grade glioma most consistent with a pilocytic astrocytoma. CONCLUSION: This case report confirms that pilocytic astrocytomas can present as brainstem gliomas, and histopathology plays a major role in determining the aggressiveness of operative intervention.

Acantholytic squamous cell carcinoma in upper aerodigestive tract: histopathology, immunohistochemical profile and epithelial mesenchymal transition phenotype change.

Acantholytic squamous cell carcinoma is a rare variant of squamous cell carcinoma in the mucosa of upper aerodigestive tract. Histomorphologically, acantholytic squamous cell carcinoma may lose the typical features of conventional squamous cell carcinoma and mimic other epithelial or mesenchymal malignancies due to advanced acantholysis and dyskeratosis. Because of its rarity, information of prognosis, pathologic features and immunohistochemical profiles is limited. We have studied clinicopathologic features and immunohistochemical profiles of four acantholytic squamous cell carcinoma cases arising from upper aerodigestive tract. Clinical results indicate an aggressive biologic behavior. Morphologically, all tumors revealed significant acantholysis with separation of tumor cells and intratumoral spaces. The tumor cells were highly pleomorphic and growth patterns were variable. In immunohistochemical studies, all tumor cells revealed positive reactions for AE1/AE3 and p63 supporting a squamous epithelial origin. In contrast to conventional aerodigestive squamous cell carcinoma, acantholytic squamous cell carcinoma showed significant reductions of cytokeratin19, E-cadherin and concomitant up-regulation of vimentin expression. Both morphologic features and immunohistochemical profiles indicate that acantholytic squamous cell carcinoma has acquired an epithelial mesenchymal transition phenotype. However, in contrast to other solid malignant tumors, the epithelial mesenchymal transition phenotype change in acantholytic squamous cell carcinoma is not limited to the invasive front of the peripheral tumor but, rather, diffusely involves entire neoplastic lesion. In addition, because cytokeratin 19 staining is attenuated, this would be an insensitive marker for following up and/or in detecting disseminated tumor cells in cases of acantholytic squamous cell carcinoma in upper aerodigestive tract.

Increased expression of p53 protein correlates with the extent of myocyte damage in cardiac allograft rejection.

Acute cardiac allograft rejection (ACAR) has been associated with a poor prognosis. The early diagnosis of ACAR necessitates the accurate detection of myocyte damage. Nuclear damage activates p53, a transcription factor that initiates apoptosis and repair. Endomyocardial biopsies (n=25) from 10 cardiac allograft recipients were stained for nuclear p53. The biopsies were divided into rejection groups based on the grading of ACAR: group 1, grade 0; group 2, grade Ia and Ib; group 3, grades II and III. While clinical indices did not correlate with myocyte damage, significantly more myocytes in group 3 stained for nuclear p53 (2.48+/-0.60/mm(2)) compared with group 1 (0.22+/-0.12/mm(2)) and group 2 (0.43+/-0.18/mm(2)). Increased expression of p53 in cardiac myocytes with grade II or grade III rejection provides an objective quantification as an aid in the diagnosis of ACAR.

Regulation of dextran sodium sulfate induced colitis by leukocyte beta 2 integrins.

Inflammatory bowel diseases (IBD) are chronic inflammatory disorders whose etiology remains unknown. Reports have shown that infiltration of leukocytes into intestinal tissue is a pathognomonic hallmark for this disease. Leukocyte beta(2) integrins are heterodimeric adhesion membrane proteins that are exclusively expressed on leukocytes and participate in immune cell adhesion and activation. In this study, we examined the pathophysiological role of the beta(2) integrins CD18, CD11a, and CD11b in the pathogenesis of dextran sodium sulfte (DSS)-induced experimental colitis. Disease activity was measured by daily assessment of clinical parameters including stool consistency, weight loss, occult blood, and gross rectal bleeding. Histopathological changes including severity of inflammation, surface epithelial/crypt damage, and depth of injury were also determined. The CD18 null and CD11a null mice had significantly lower disease activity and cumulative histopathological scores compared to wild-type mice. Interestingly, CD11b null mice did not show protection against DSS colitis and displayed increased disease activity compared to wild-type mice. Examination of specific leukocyte populations in the distal colon from various mice revealed significant attenuation of neutrophil and macrophage infiltrates in CD18, CD11a, and CD11b null mice. Surprisingly, the CD11b null mice showed a significant increase in plasma cell infiltration in response to DSS suggesting that this molecule may influence plasma cell function during colitis. This study demonstrates that genetic loss of CD18 or CD11a is protective during experimental colitis and that CD11b may serve a regulatory role during development of disease.

Plexiform neurofibroma of the uterine cervix: a case report and review of the literature.

The female genital system is rarely affected in von Recklinghausen neurofibromatosis. The vulva is the most frequent genital location, but vaginal, cervical, uterine, and ovarian neurofibromas have rarely been reported. We describe a case of plexiform neurofibroma affecting the uterine cervix in a patient with chronic pelvic pain and menorrhagia who had multiple cutaneous neurofibromas and 1 large paraspinal neurofibroma. A small plexiform neurofibroma, which was not grossly visible, was confined to the uterine cervix and coexisted with a uterine leiomyoma and adenomyosis. There were no neurofibromas in the myometrium, fallopian tubes, or ovaries. Plexiform neurofibroma is a neoplasm that should be considered in the differential diagnosis of spindle cell neoplasms of the uterine cervix, especially in specimens from patients with neurofibromatosis.

CD56 reactivity in small cell carcinoma of the uterine cervix.

Small cell carcinoma (SCC) of the uterine cervix, like its pulmonary counterpart, is a rare but distinctive neoplasm that should be separated from nonendocrine carcinomas because of its highly aggressive clinical course and response to chemotherapy and irradiation. CD56 (neural cell adhesion molecule) has recently been shown to be the best marker for the diagnosis of pulmonary SCC. In this study, we assessed the sensitivity and specificity of CD56 in the diagnosis of SCC of the uterine cervix compared with those of chromogranin and synaptophysin. Twenty-two (88%) of 25 SCCs of the uterine cervix labeled with CD56 in a predominantly membranous and diffuse pattern, whereas 16 of 25 (64%) stained with synaptophysin in a predominantly diffuse pattern and 8 of 25 (32%) showed predominantly focal immunoreactivity for chromogranin. In contrast, 3 of 21 (14%) moderately to poorly differentiated squamous cell carcinomas and 1 of 16 (6%) moderately differentiated adenocarcinomas showed focal immunoreactivity for CD56. Although not specific, CD56 seems to be the most sensitive marker for the diagnosis of SCC of the uterine cervix. Moreover, its diffuse reactivity reduces the possibility of obtaining negative results in small biopsy samples.