Parathyroid adenoma and chondrosarcoma after treatment of pediatric Hodgkin disease.

Treatment of Hodgkin disease (HD) with chemoradiotherapy in children is associated with increased risk for developing secondary neoplasms. Parathyroid adenoma (PTA) and chondrosarcoma (CS) are quite rare types of secondary neoplasms after HD. We describe a 5-year-old boy with stage IV HD, successfully treated with MOPP (mechlorethamine, vincristine, procarbazine, and prednisone)/ABVD (doxorubicin, bleomycin, vinblastine, and dacarbazine) chemotherapy followed by 35 Gy mantle radiotherapy who developed primary hyperparathyroidism because of benign PTA at the age of 20 years, and died of CS in thoracic vertebrae at the age of 22 years. Consecutive occurrence of PTA and CS after treatment of pediatric HD, to the best of our knowledge, has not been reported earlier.

The histopathologic evaluation of soft tissue changes in rabbit extremity after different dose-fractionation schemes of interstitial high dose rate (HDR) brachytherapy.

BACKGROUND: The use of HDR in the treatment of soft tissue sarcoma had been on the rise. However, there was limited study to evaluate the effect of different fractionation schemes on soft tissue and the optimal HDR scheme. AIMS: We aimed to assess the histopathologic changes on soft tissue after different HDR brachytherapy doses. METHODS: The subjects were divided into three groups. Each group included 10 limbs. Group A had only an applicator without radiation, group B received a total of 24 Gy at 6 Gy per fraction, twice a day, and group C received a total of 13.5 Gy in a single fraction. The histopathologic findings were grouped into soft tissue pathology-1 (edema, inflammation, endothelial proliferation, necrosis) and soft tissue pathology-2 (atrophy, calcification, vascular hyalinization, fibrosis) (STP-1-2). RESULTS: The highest mean grade values of STP-1 and STP-2 were observed in group C (0.95 and 1.45) in comparison to group A (0.45 and 0.85) and group B (0.65 and 0.9). The difference in STP-1 was found significant only between groups A and C and the difference in STP-2 was found both between groups A and C and groups B and C. CONCLUSION: In our experimental study it was shown that the fractionated interstitial HDR had both lower rate and severity of toxicity in comparison to a single high dose fraction. Before using a single fractionated regimen in the clinic, the increased morbidity related to the irreversible early toxicities or progressive late toxicities should be kept in mind.

Clinicopathological significance of insulin-like growth factor-1 receptor expression in breast cancer.

OBJECTIVE: Insulin-like growth factor 1 receptor (IGF1R) is a receptor protein tyrosine kinase that is claimed to be related with tumor development and progression of breast cancer with some conflicting results in the literature. The aims of the study are to investigate expression of IGF1R, and correlate with clinicopathological parameters to clarify the significance of IGF1R on breast cancer. MATERIAL AND METHODS: IGF1R and Ki67 were applied immunohistochemically to the tissue microarray sections of 370 female breast cancer patients. The results were correlated with clinical, prognostic, histopathological features, and other immunohistochemical findings [ER, PR, HER2, CK5/6, and CK14] statistically. RESULTS: IGF1R overexpression showed direct correlation with Ki67 index (P=0.028), HER2 positivity (P=0.001), mitotic count (P=0.004), tumor grade (P=0.015), and geographic necrosis (P=0.023); and negative correlation with ER positivity (P=0.003). There was statistically significant difference between IGF1R expression and the molecular subtypes (P<0.001), mostly HER2+ phenotype. IGF1R expression was found to be higher in invasive ductal carcinoma (IDC) than invasive lobular carcinoma (ILC) (P=0.036). Both IGF1R and Ki67 expression were negatively correlated with disease-free survival (DFS) (P=0.020, P=0.023, respectively) and overall survival (OS) [P<0.001, each] rates. The inverse association between IGF1R overexpression and OS rate was also supported by multivariate analyses (P=0.025). CONCLUSIONS: Overexpression of IGF1R was found to be directly correlated with shorter DFS and OS as well as some clinicopathological features associated with adverse prognosis such as higher Ki67 index, mitotic count, tumor grade, presence of geographic necrosis, HER2 positivity, ER negativity, HER2+ molecular subtype, histological tumor type of IDC rather than ILC. Thus, IGF1R might be considered as an useful target for comprehensive future anti-tumor therapy investigations. Additionally, using IGF1R as well as Ki67 as a part of routine pathology practice might be fruitful in breast cancer therapy and prediction of prognosis. KEY WORDS: Breast carcinoma, IGF1R, Insulin-like growth factor-1 receptor, Immunohistochemistry, Prognosis.

Histiocytic sarcoma: PET-CT evaluation of a rare entity.

Histiocytic sarcoma is a rare malignancy of hematopoietic origin. Lymph nodes, skin, and extranodal sites, especially gastrointestinal tract, are commonly involved. Some cases reported in the past as non-Hodgkin's lymphoma are now classified as histiocytic sarcoma by detailed immunohistochemical studies. Patients with clinically localized disease have a good prognosis whereas those with lymphatic involvement have an aggressive course. In our case, histiocytic sarcoma was detected, originating from the skin over the left shoulder associated with disseminated lymphadenopathy. A positron emission tomography/computed tomography examination was done for evaluating the extent of the disease which showed pathologic increased 18F-fluorodeoxyglucose uptake in the lymph nodes, indicating widespread disease. The pertinent literature is reviewed.

Angiographically occult vascular malformation of the third ventricle.

Angiographically occult vascular malformations (AOVM) are cerebrovascular malformations that are not demonstrable on cerebral angiography. The majority of AOVMs located in the third ventricle are cavernous malformations. To the best of our knowledge an angiographically occult arteriovenous malformation (AOAVM) of the third ventricle has not been previously reported. We report an unusual vascular malformation of the third ventricle presenting with hydrocephalus due to mass effect, verified histopathologically as an AOAVM.

Clinical characteristics and treatment results of pediatric B-cell non-Hodgkin lymphoma patients in a single center.

The aim of this study was to evaluate and compare the clinical characteristics of the B-cell non-Hodgkin lymphoma (NHL) patients and therapeutic efficacy of modified NHL BFM-90 and NHL BFM-95 protocols in the authors' center. From January 1993 to December 2003, 61 newly diagnosed children with B-NHL were enrolled to the study. The patients were stratified by risk factors and treated either with a modified B-NHL BFM-90 or BFM-95 protocols. The use of 1 or 3 g/m2 of methotrexate instead of 5 g/m2/24 h was the only important modification in BFM-90 protocol. Sixty-one children (12 girls, 49 boys) with a median age of 6.5 years (range: 2.5-16) were treated in the center. There were 14 patients in stage II, 28 in stage III, and 19 in stage IV. The most common initial primary tumor sites were abdomen, head, and neck. Forty-five patients were treated with modified B-cell BFM-90 and 16 patients were treated with B-cell BFM-95 regimens. The 5-year overall survival (OS) for all patients was 85.8%, and event-free survival (EFS) was 82.8%. The 5-year OS rates in modified BFM-90 and in BFM-95 protocols were 85.2 and 87.5%; the 5-year EFS rates in these 2 protocols were 84.6 and 70%, respectively (p >.05). Factors associated with lower EFS by univariate analysis were bulky disease, risk groups, and LDH level > or = 500 IU/L. By multivariate analysis only LDH level was significant. In conclusion, the treatment results in this study were similar to those of BFM group.

Laparoscopic resection of urinary bladder paraganglioma: a case report.

The case report of a 55-year-old woman with an incidentally diagnosed urinary paraganglioma of the bladder is presented. The patient had no irritative voiding symptoms, hematuria or hypertension. She was diagnosed to have an immobile solid mass on the left side neighboring the lower segment of the uterus. Transvaginal ultrasonography revealed a well-limited ovoid mass with solid and cystic areas adjacent to the urinary bladder and the uterus. Tumor markers were within normal range. Laparoscopy was performed for the treatment of the mass and complete excision of the cyst was accomplished. Frozen section was performed revealing a benign cystic structure but the identification of the origin was left to definitive histopathological examination which showed paraganglioma of the bladder. Immunohistochemically, the tumor cells were strongly positive for chromogranin A and synaptophysin and there was focal positiveness for neuron specific enolase although vimentin and cytokeratin were negative.

An unusual presentation of ossifying fibromyxoid tumor of the mandible: a case report.

A 13-year-old boy who complained rapid swelling on the left side of mandible is presented. Histopathological examination revealed ossifying fibromyxoid tumor (OFMT). OFMT is a rare soft-tissue neoplasm that occurs usually in the subcutaneous tissue of the extremties. Head and neck involvement is relatively rare. In this case, we present the diagnosis, surgical treatment and long-term follow-up of an OFMT due to its unusual site of occurrence. The precise clinical behavior of atypical and malignant types of OFMTs is still unclear. Thus, histopathology report is important, leading surgeon to decide how often and how long to follow-up patient with OFMT.

Clinicopathological Significance of the Proliferation Markers Ki67, RacGAP1, and Topoisomerase 2 Alpha in Breast Cancer.

Objectives The aims of this study are to evaluate expressions of Ki67, RacGAP1 (MgcRacGAP) and topoisomerase 2 alpha (TOP2a), the markers related with cell proliferation that have been proposed to affect the prognosis in the literature and correlate the results with clinicopathological parameters of breast cancer patients. Methods Ki67, RacGAP1, and TOP2a antibodies were applied immunohistochemically to the tissue micrarray blocks of 457 female breast cancer patients. The results were correlated with clinical, prognostic, histopathological features, and other immunohistochemical findings (estrogen receptor [ER], progesterone receptor [PR], HER2, cytokeratin [CK]5/6, CK14, epidermal growth factor receptor [EGFR] and vimentin), statistically. Results Ki67 expression demonstrated direct correlation with TOP2a expression, mitotic count, tumor grade, geographic necrosis, basal-like phenotype. RacGAP1 expression was directly correlated with TOP2a expression, nipple invasion, and number of metastatic lymph nodes, and it was inversely correlated with PR expression. TOP2a expression was directly correlated with vimentin and Ki67 expressions, mitotic count, tumor grade, and geographic necrosis, and nipple invasion, and negatively correlated with ER and PR expressions. Higher TOP2a and Ki67 expressions were correlated with shorter overall survival. Higher TOP2a expression and RacGAP1 positivity were directly correlated with shorter disease-free survival. Conclusion This study showed that the overexpressions of Ki67, RacGAP1, and TOP2a affect the prognosis adversely, thus to develop target therapies against RacGAP1 and TOP2a as well as using Ki67 as a part of routine pathology practice might be beneficial in breast cancer therapy and prediction of prognosis.

Secondary Neoplasms in Children with Hodgkin's Lymphoma Receiving C-MOPP and Radiotherapy: Presentation of Four Cases.

Patients who survive Hodgkin lymphoma (HL) are at increased risk of secondary neoplasms (SNs). A wide variety of SNs have been reported, including leukemias, non-Hodgkin's lymphomas, and solid tumors, specifically breast and thyroid cancers. Herein we report subsequent neoplasms in four patients with HL receiving chemoradiotherapy. It is interesting that three SNs, fibrosarcoma, thyroid carcinoma, and retrobulbar meningioma, were observed in the radiation area in one of our patients. A hypopharyngeal epithelioid malignant peripheral nerve sheath tumor as an unusual secondary malignant neoplasm developed in another patient, while a benign thyroid nodule and invasive ductal breast carcinoma were observed at different times in the female patient. Follicular adenoma of the thyroid gland developed in one of our patients.

Interdigitating dendritic cell tumor with breast and cervical lymph-node involvement: a case report and review of the literature.

Interdigitating dendritic cell tumor (IDCT) is an extremely rare malignancy. It occurs primarily in lymph nodes, but extranodal involvement has also been reported. A 38-year-old woman with IDCT with breast and cervical lymph-node involvement is reported in this paper. To our knowledge, this is the first case of IDCT originating from the breast. In the breast and lymph node, the tumor displayed diffuse sheets, fascicles and storiform growth pattern. It was composed of oval to spindle cells with pale to eosinophilic cytoplasm, ill-defined cell outlines, oval nuclei with vesicular chromatin and prominent eosinophilic nucleoli. Mitotic activity was three per ten high-power fields. The neoplastic cells were intermingled with small mature lymphocytes and plasma cells. Immunohistochemical studies showed that the tumor cells were strongly and diffusely positive for vimentin, CD68, S-100 protein, CD45/leukocyte common antigen and fascin and focally positive for lysozyme, alpha-1 antitrypsin and CD4. Ki-67 labeling index was 10%. The patient was treated with combined therapeutic approaches, including surgery, radiotherapy and chemotherapy. IDCT has the potential for an aggressive clinical course. However, 32 months after the initial diagnosis, the patient is still alive and being followed with a stable tumor burden.

Epithelioid sarcoma-like hemangioendothelioma: a case report.

Seven cases of an unusual low-grade vascular tumor were reported in a recent study. Despite its similarity to epithelioid sarcoma, this tumor was termed epithelioid sarcoma-like hemangioendothelioma because of the subtle histopathological and immunohistochemical differences. Another case of this rare entity in a 70-year-old woman who suffered from a painful mass on the anterior aspect of the right cubital fossa is presented here, together with a review of the relevant literature.

Pathologic basis of pyogenic, nonpyogenic, and other spondylitis and discitis.

Pyogenic spondylitis and discitis are usually seen following a recent infection or surgery. A septic embolus causes an infarcted area within the bone. Pyogenic spondylitis is characterized by edema, vascular leakage, and supportive inflammatory reaction characterized with polymorphonuclear leukocytes. In tuberculosis of the spine, active lesions are characterized by formation of epithelioid granulomas with central caseating necrosis. Mycobacterium tuberculosis can be shown by histochemical stains for acid-fast bacteria or by immunochemistry. In brucella spondylitis, microgranulomatous proliferation composed of histiocytes containing numerous bacilli without caseating necrosis is characteristic. Brucella melitensis can be shown on histochemical Gram stain.

Mediastinal neurothekeoma: a rare tumor.

Neurothekeoma, also known as nerve sheath myxoma, is a benign nerve sheath tumor that usually arises in the cutaneous nerves of the head, neck, or upper extremities. Extracutaneous placement is very rare. Mediastinal neurothekeoma has not previously been reported in the English literature. A 30-year-old woman was admitted to our clinic with back pain. A smooth-edged mass found in her paravertebral region, and a neurogenic tumor was suspected. The tumor was completely excised. Final pathology revealed it to be a mediastinal neurothekeoma.

A rare case of primary inoculation tuberculosis seen after varicella.

Primary inoculation tuberculosis (TB) is a rare form of cutaneous TB resulting from direct introduction of Mycobacterium tuberculosis into the skin or mucosa of a previously uninfected, nonimmune person. We herein report the first case, to our knowledge, of primary inoculation TB to be seen after varicella; this case explains the possible mechanism of varicella-zoster virus-mediated transient cellular immune suppression that predisposed the patient to cutaneous TB. In this case, we believe that varicella-zoster virus (VZV) infection predisposed the patient to primary inoculation TB by leading to direct inoculation of tuberculosis bacilli through vesicles or by suppressing cellular immunity.

Primary Intracerebral Alveolar Soft Part Sarcoma in an 11-Year-Old Girl: Case Report and Review of the Literature.

Alveolar soft part sarcoma (ASPS), a rarely observed tumor, is a soft tissue sarcoma with an unidentified cell origin. It constitutes 0.5-1.0% of all soft tissue sarcomas. It may appear in various parts of the body, but mostly observed in the trunk and the extremities. It has a high metastasis potential. To the best of our knowledge, only three cases of primary intracranial ASPS without a demonstrable lesion elsewhere is encountered. An 11-year-old girl was operated because of fronto-parietal mass lesion by craniotomy. Pathological examination revealed ASPS and no primary focus was detected. In spite of radiotherapy and chemotherapy as an adjuvant therapy, after 45 months she had a second operation for recurrence of the tumor. Since it is possible to observe metastases in late phases, up to 30 years, the patients must be followed up for a long period. Although radiotherapy and chemotherapy followed by surgery is the most accepted treatment strategy, the prognosis is still poor.

A proven case of cutaneous rhizopus infection presenting with severe limb pain very soon after induction treatment in a patient with acute lymphoblastic leukemia.

Objective and Importance. Invasive mucormycosis may complicate the course of patients with hematologic malignancies and has a very high mortality rate. Early diagnosis and aggressive approach combined with surgical and medical treatment have paramount importance for cure. Clinical Presentation. We report here a case of a patient with acute lymphoblastic leukemia presenting with a subcutaneous mass lesion which was sampled by an ultrasound guided needle biopsy. The pathology showed microorganisms with aseptate hyphae with wide, irregular walls and more or less branching with highly vertical angles which suggested a mold infection. The specimen was also cultured where Rhizopus spp. grew. Conclusion. Posaconazole 200 mg QID was commenced. She recovered from neutropenia and pain on day 20 of treatment. After 4 courses of hyper-CVAD chemotherapy, the remaining soft tissue mass was removed surgically and she underwent allogeneic HSCT from a full matched sibling donor under secondary prophylaxis.

Simple bone cyst in spinous process of the c4 vertebra.

Simple bone cysts are common, benign, fluid-filled, cystic lesions that occur mostly in the metaphysis of the long bones and are rarely found in vertebrae. A case of a simple bone cyst in the spinous process of the fourth cervical vertebra in a 26-year-old woman is reported. According to the radiologic findings, the lesion was identified as a simple bone cyst, and the diagnosis was verified by surgical and histopathologic examinations.

Monostotic fibrous dysplasia of the thoracic spine: clinopathological description and follow up. Case report.

The authors report the case of a 53-year-old woman with monostotic fibrous dysplasia of the thoracic spine. The patient presented with a 1-month history of pain in the thoracic spinal region. En bloc resection of the lesion was successfully performed via a transthoracic approach, and a histopathological examination confirmed the diagnosis of fibrous dysplasia. At 24-month follow-up examination, pain and vertebral instability were absent. The findings in this case illustrate that, although very rare, monostotic fibrous dysplasia of the thoracic spine should be considered in the differential diagnosis of spinal tumors. Although a consensus for management of this disease has not been achieved, the authors recommend radical removal of all involved bone as well as internal fixation or bone graft-assisted fusion to achieve long-term stabilization.

Cholestatic liver disease with ductopenia (vanishing bile duct syndrome) in Hodgkin's disease: report of a case.

Liver involvement is common in advanced stages of Hodgkin's disease. However, only a small percentage of patients with Hodgkin's disease develops jaundice due to several causes. Vanishing bile duct syndrome secondary to Hodgkin's disease is a rare cause of cholestasis in these patients. Only 20 cases, to our knowledge, have been reported so far in adults. We report a case of Hodgkin's disease presenting with obstructive jaundice without detectable liver involvement. Liver biopsies revealed intrahepatic cholestasis and ductopenia. Although the patient was given chemotherapy, he died of sepsis and disseminated intravascular coagulation after 24 weeks of admission to hospital.