Soft tissue giant cell tumor of low malignant potential of the neck: a case report and review of the literature.

Giant cell tumor of soft parts is a rare tumor that is clinically and histologically similar to giant cell tumor of the bone. We present a 53-year-old female with a giant cell tumor of low malignant potential arising from the neck. The clinicopathological features and the importance of immunohistochemistry in the differential diagnosis with other giant cell rich tumors was discussed.

Epithelioid schwannoma of soft tissue: unusual morphological variant causing a diagnostic dilemma.

Epithelioid variant of peripheral nerve sheath tumors is a rare but, at the same time, a well-known entity especially in the malignant counterpart. However, peculiar epithelioid morphology in soft tissue schwannomas is unusual and has been defined recently. These tumors may cause diagnostic errors owing to their increased cellularity and epithelioid morphology. Typical histologic features of classic schwannoma such as Antoni A and B areas, Verocay bodies, and hyalinized vessels are either absent or only present in focal areas. Furthermore, strong and diffuse S-100 protein expression is seen in both benign and malignant counterparts of epithelioid schwannoma. The findings that are suggestive of the benign nature of the lesion are long-term clinical history, small size, superficial localization, encapsulation, bland morphology, lack of mitosis and necrosis, and a benign clinical course after complete excision. Pathologists should be aware of the epithelioid variant of schwannoma to avoid false diagnosis of malignancy. We hereby report 3 cases of unusual benign epithelioid schwannoma of the soft tissue with special regard to problems in differential diagnosis.

Relation of hepatitis B core antigen expression with histological activity, serum HBeAg, and HBV DNA levels.

BACKGROUND: Hepatitis B virus (HBV) is a potentially life-threatening liver infection which may progress to liver failure and cirrhosis. Intrahepatic expression patterns of viral antigens detected by immunohistochemistry may have prognostic implications in disease process. AIM: In this study, we aimed to investigate the relationship between the HBV core antigen (HBcAg) expression and histological activity index (HAI), fibrosis, serum hepatitis B e-antigen (HBeAg) status and HBV DNA levels in patients with chronic HBV infection. MATERIALS AND METHODS: A total of 114 liver biopsies from patients with chronic HBV infection were included in the study. Immunohistochemical expression of HBcAg and its relation with HAI, fibrosis, serum alanine aminotransferase (ALT) levels, HBeAg status and HBV DNA levels were assessed. RESULTS: The presence of nuclear expression of HBcAg did not show any correlations with ALT levels, HAI and fibrosis score. When the groups were categorized according to the HBeAg status, nuclear HBcAg expression was found to be high in HBeAg positive patients. However, HBcAg nuclear expression showed significant correlations with HBV DNA levels and fibrosis scores in HBeAg negative but not HBeAg positive patients. HBV DNA levels were also significantly associated with HAI and fibrosis scores in HBeAg negative patients. CONCLUSIONS: Significant differences found between HBeAg positive and negative patients suggest that HBeAg negative disease is different from HBeAg positive disease, and also point outs that in HBeAg negative disease, patients with nuclear HBcAg expression and increased levels of HBV DNA levels are at a higher risk of developing progressive liver disease.

A case of a sporadic malignant peripheral nerve sheath tumor of the urinary bladder with concomitant in situ urothelial carcinoma treated by transuretheral resection.

Malignant peripheral nerve sheath tumor (MPNST) of the urinary bladder is a very rare clinical entity. The association of such a tumor with urothelial carcinoma is even more unusual. Differential diagnosis between coexisting two distinct primary tumors and carcinosarcoma of the urinary bladder is very important as both the treatment and prognosis vary widely. Herein, we report a case of an MPNST with a concomitant in situ urothelial carcinoma in a 53-year-old man. To our knowledge, this is the first documented case of MPNST of the bladder that is treated by transuretheral resection which is in contrast with the previous reports that used cystectomy.

Inflammatory pseudotumor of the liver diagnosed by needle biopsy: report of three cases (one with neuroendocrine tumor of the rectum and lung).

Inflammatory pseudotumor of the liver is a rare disorder that can histologically and radiologically resemble malignant neoplasms. The prognosis of the patients with hepatic inflammatory pseudotumor is usually good with conservative therapy. Most of the reported cases are diagnosed in the surgical resection specimens; only very few reported cases have been diagnosed by needle biopsy. We report three additional cases of inflammatory pseudotumor of the liver diagnosed by liver biopsy. Two of these cases were treated successfully with antibiotics. The other case, who was resistant to medical treatment, had coexisting neuroendocrine neoplasms of the rectum and the lung. Since her general condition did not allow an extensive surgery, the mass of the liver could not be resected and she died approximately five months after she was voluntarily discharged from the hospital. Many of the inflammatory pseudotumor of the liver are found to be associated with variable neoplasms, but to our knowledge, the latter case is the first case of inflammatory pseudotumor associated with a neuroendocrine tumor.

Ground-glass-like hepatocellular inclusions in the course of adult-onset Still's disease.

Ground-glass hepatocytes are the cardinal biopsy feature of chronic hepatitis B virus (HBV) infection and may also be present in other specific conditions, including Lafora's disease, cyanamide aversion therapy for alcohol use, patients with transplantation, uremia, and metabolic disorders. In this report, we present the case of a patient with adult-onset Still's disease who underwent percutaneous liver biopsy, which revealed ground-glass-like cytoplasmic inclusions and which is a very unusual finding.

Primary primitive neuroectodermal tumor of the lumbar extradural space.

Primary spinal primitive neuroectodermal tumors (PNETs) of the extradural space are very rare; only 10 cases have been reported in the English language literature. The histopathological diagnosis of primary spinal PNETs has been discussed for many years. These tumors have a rapidly progressive course, and there is no current consensus on the optimal therapeutic approach for these patients. The authors present a case of primary PNET located in the lumbar extradural space in a 13-year-old girl and report the clinical, radiological, histopathological, and surgical findings. They compare their findings with those from the other 10 cases reported in the literature and review relevant literature.

Is it possible to start the treatment based on immediate cytologic evaluation of core needle biopsy of the spinal lesions?

Core needle biopsy is widely used to diagnose spinal lesions. Final histopathological report of the specimen usually can be given after several days. These several days delay may prevent early treatment in some patients. Our aim was to investigate if treatment of spinal lesions could be started according to the immediate cytologic evaluation of the core needle biopsy. The cytological materials of the 213 core needle spinal biopsy were prepared immediately after biopsy procedure. Of these biopsies, only 101 core needle biopsy had a final diagnosis which formed the backbone of the current study. Cytological materials of these cases were compared with both histopathologic results of the core biopsy and with final diagnosis obtained by open surgery or clinical follow up. According to final diagnoses 40 patients had infections, 59 patients had neoplasms and 2 patients had no pathology. When the diagnosis that determines the treatment is considered, the overall success rates (positive predictive value:PPV) of cytology and histology were 0.65 and 0.76 respectively. Moreover when the specific diagnosis were analyzed separetely in the subgroups of tuberculous infection, lymphoma and plasmacytoma; PPV of both cytological and histological assessments was 1.00. In spinal metastasis, PPV was 0.97 for cytology and 1.00 for histology. Rapid cytologic interpretation of core needle biopsy material is a useful and reliable tool to determine the spinal lesions which require urgent treatment. When the cytological specimen reveals the diagnosis of spinal metastasis, lymphoma, plasmacytoma or tuberculosis, the treatment may be started without waiting the result of the histological examination.

MIB-1 counting methods in meningiomas and agreement among pathologists.

OBJECTIVE: To evaluate the correlation of MIB-1 labeling index (LI) obtained by 2 counting methods with histologic grade and investigate interobserver variability between these methods. STUDY DESIGN: A total of 65 meningiomas were analyzed for proliferation with 2 counting methods by 2 pathologists using MIB-1 antibody. In the first method, the most densely staining areas were counted (HL method). In the second method, randomly selected areas were counted (RS method). RESULTS: MIB-1 values correlated well with histologic grade in both methods. As expected, the tumors with recurrence had significantly higher LIs than the nonrecurrent tumors in each method. However, there was a statistically significant difference in the mean MIB-1 values of between the HL and RS methods. When MIB-1 LI was compared between 2 pathologists, perfect agreement in the HL method and substantial agreement in the RS method were achieved. CONCLUSION: Our results showed that values of MIB LIs differ with different counting methods. Nonetheless, both methods showed good correlation with World Health Organization grades. Therefore standardization of 1 counting method is of great importance for determining a reliable and specific cutoff value in assessing the risk of recurrence in meningiomas.

Extranodal Rosai-Dorfman disease with bilateral orbital involvement: Report of a case treated with systemic steroid alone.

We report the case of a 41-year-old female with bilateral orbital Rosai-Dorfman disease (RDD) and the result of corticosteroid therapy. The patient developed a swollen mass of bilateral upper and lower eye-lid over a six-month period. Diagnosis was based on the characteristic histopathologic features. She was treated with oral corticosteroids 60 mg/per day for ten days and the dose of corticostreoid was tapered every week. She is currently being treated with 10 mg/per day for 4 months. In general, the prognosis is good, with experiencing remission following a course of oral corticosteroids. There is no evidence that corticosteroids are beneficial in the treatment of RDD in the literature, but data regarding such treatment are sparse. A more formalized surveillance of these cases would help to define more clearly the natural history of the disease and the effects of various treatment modalities.

Lipofibromatosis in a two-year-old girl: a case report.

Lipofibromatosis is a recently described rare benign soft tissue tumor of childhood. We report a case of a lipofibromatosis in a two-year-old girl with a painless mass on the plantar aspect of her right foot who developed local recurrence eight months after surgery. The tumor has a high rate of non-destructive recurrence, but there is no metastatic potential. Complete surgical resection is the mainstay of treatment. Nevertheless, the rareness of its presentation should be taken into account by the pediatric pathologist, and considerable surgical judgment is of great importance.

Kit expression in spindle cell rhabdomyosarcoma can possibly create a different approach for its tumorigenesis and therapy.

The use of a relatively nontoxic tyrosine kinase receptor inhibitor, imatinib mesylate (IM) (STI-571), has increasingly become a valuable therapeutic alternative in some KIT (CD117)-overexpressing neoplasms potentially because of the presence of KIT-activating mutations. As the treatment eligibility for this drug hinges on CD117 expression, KIT immunostaining has recently been widely examined in various different tumors. We examined CD117 expression in pediatric embryonal rhabdomyosarcomas (RMSs) to identify its eventual prognostic impact and to evaluate its effect on tumorigenesis. This study included two spindle cell (leiomyomatous) variants, two botryoid variants, and 21 conventional embryonal RMSs. Sections from paraffin-embedded tumor samples were immunostained by a standard SABC technique using c-kit polyclonal antibody with antigen retrieval. In all the series, the percentage of CD117 positivity was 12%. Staining was strong in two of two spindle cell variants, in zero of two botryoid variants, and in one of 21 conventional embryonal RMSs. In Spearman's correlation analysis, there was statistical relationship between the presence of CD117 expression and the histological subtype of RMS. Kaplan-Meier analysis revealed no prognostic significance of CD117 expression for survival. The present study demonstrated a very limited expression of CD117 in pediatric embryonal RMS other than in the spindle cell variant. This finding suggested that the stem cell factor/c-kit pathway may be implicated in the tumorigenesis of spindle cell RMSs. Therefore, the mutation of c-kit gene must be prospectively examined in larger series of RMSs. If it can be verified that tissue expression of CD117 reflects the mutation of c-kit gene, IM can be considered a targeted therapy for CD117-expressing RMSs, particularly the spindle cell variant.

Percutaneous computed-tomography-guided biopsy of the spine: 229 procedures.

OBJECTIVES: Percutaneous biopsy of the spine is an effective and well-evaluated procedure. Only very few series containing more than a hundred patients have been reported so far and there is no agreement about the factors affecting the diagnostic rate. We aimed to find out if there is any factor influencing the success rate of the spinal biopsy using our biopsy series. METHODS: Two hundred and twenty-nine procedures were performed in 201 patients between November 2001 and August 2005. All procedures were performed under computed tomography guidance. The side was chosen according to the extension of the lesion. When the lesion was in the midline or extended to both sides, we preferred to obtain biopsy from the right side. The puncture point and the needle trajectory were planned on both lateral computed tomography scout scan and axial scans. RESULTS: We found that the diagnostic rate was not affected by the variables such as age, gender, type and diameter of the biopsy needle, diagnosis as well as lesion localization and level. The success rate of the repeat biopsies was considerably lower than the first procedures. CONCLUSIONS: The diagnostic rate is not affected by any of the variables but the approach, chosen can vary with the level, localization, and lesion characteristics.

Primary rhabdoid tumor of the brain in an adult.

Rhabdoid tumor (RT) is an uncommon childhood neoplasm that typically arises within the kidney. It is characterized by an aggressive clinical course. Since its description in 1978, several cases of primary extrarenal RT, including a CNS localization, have been reported. The first case in the CNS was reported in 1985 and was defined as "rhabdoid tumor" initially, and was classified as grade IV in the most recent classification of the World Health Organization under the term of "atypical teratoid/rhabdoid tumor". Nearly 200 cases of atypical teratoid/rhabdoid tumor of the CNS have been reported to date, most of them occurring in childhood. We report a case of primary RT of the brain located in the right frontal lobe with the clinical, radiographic and pathological features presenting at an unusual age. This tumor, which was composed purely of rhabdoid cells with no additional primitive neuroectodermal, epithelial and mesenchymal components, was in a 27-year-old male patient. In conclusion, RT should be considered also in the differential diagnosis of intracerebral neoplasms of adult patients.

A succesfully managed inflammatory pseudotumour of liver without surgery: report of a case.

The inflammatory pseudotumor (IPT) of the liver is an extremely rare focal lesion of the parenchyma with unknown aetiology. It has the appearance of a malignant tumor but has a benign histology and clinical course. Herein, we report a case of IPT in a 55 year-old woman. She had complained of right upper quadrant pain and subfebrile fever. Imaging studies revealed a heterogeneous, solitary mass in right lobe of liver which was 90 x 81 mm in size. Histopathological examination of ultrasonography-guided true-cut biopsy demonstrated IPT. After the initiation of the antibiotic and nonsteroidal anti-inflammatory drugs (NSAID), the size of hepatic pseudotumor decreased (20 x 20 mm) and then completely disappeared. To rule out a malignancy and to reach the diagnosis of IPT, biopsy from the mass is mandatory. After the diagnosis, IPT gave an excellent response to short course of antibiotic and NSAID therapy. To prevent unnecessary resective liver surgery, IPT should be kept in mind in the differential diagnosis of liver mass which can be successfully managed medically.

[Four cases of inverted papilloma]. / Inverted papillomlu dört olgunun sunumu.

Inverted papilloma is a benign lesion fo the nasal cavity and paranasal sinuses. Its aggressiveness and association with malignancy have been emphasized in the literature. In this paper, four patients (3 females, 1 male) with inverted papilloma are presented. Their ages ranged between 45 and 68 years. Surgery was performed using the degloving method which enables a wide exposure and radical resection with minimal scar on the face. No recurrences were detected in the postoperative period in patients with regular controls. The importance of postoperative histopathologic examination is addressed with a review of the literature.