Dural-based large B-cell lymphoma masquerading as a tentorial meningioma.

A 53-year-old woman presented with a 2-week history of headache and vertigo. Computed tomography revealed a hyperdense tumor, measuring 30 × 31 × 36 mm in diameter, in the anteromedial parts of the cerebellar hemispheres. Cerebral magnetic resonance imaging 10 days later revealed an apparent extra-axial tumor with broad attachment to the medial tentorium cerebelli and rapid growth to a diameter of 40 × 41 × 46 mm. Cerebral angiography revealed no obvious feeding vessels or tumor stains. The patient underwent biopsy through the left occipital transtentorial route. The histological appearance was consistent with diffuse large B-cell lymphoma. Intracranial lymphoma may present as a dural tumor that mimics a meningioma. Rapid tumor growth incongruous with benign meningiomas should be assumed to be possible lymphoma, and prompt biopsy should be performed.

A Rare Case of Sinonasal Seromucinous Hamartoma Developing from the Nasal Septum.

A 45-year-old man presented with a history of chronic left nasal congestion. Nasal endoscopy revealed a pedunculated polypoid mass with glandular epithelium surface on the posterior nasal septum. Computed tomography revealed a 25-mm mass-like growth in the left posterior nasal cavity attached to the nasal septum with a stalk. The patient underwent transnasal endoscopic surgery, and the tumor was removed under a block with safety margin. The final pathological diagnosis was sinonasal seromucinous hamartoma (SH). Sinonasal SH is a rare tumor with only 31 reported cases. Transnasal endoscopic surgery is currently the first-line treatment for sinonasal SH. Differential diagnoses of this lesion include inflammatory polyps, respiratory epithelial adenomatoid hamartoma, and adenocarcinoma. Although SH is a benign tumor, its progression to adenocarcinoma has been reported. Therefore, unilateral posterior nasal tumors must be diagnosed precisely.

Possible New Histological Prognostic Index for Large B-Cell Lymphoma.

We conducted a retrospective analysis of GRP94 immunohistochemical (IHC) staining, an ER stress protein, on large B-cell lymphoma (LBCL) cells, intracellular p53, and 15 factors involved in the metabolism of the CHOP regimen: AKR1C3 (HO metabolism), CYP3A4 (CHOP metabolism), and HO efflux pumps (MDR1 and MRP1). The study subjects were 42 patients with LBCL at our hospital. The IHC staining used antibodies against the 17 factors. The odds ratios by logistic regression analysis used a dichotomous variable of CR and non-CR/relapse were statistically significant for MDR1, MRP1, and AKR1C3. The overall survival (OS) after R-CHOP was compared by the log-rank test. The four groups showed that Very good (5-year OS, 100%) consisted of four patients who showed negative IHC staining for both GRP94 and CYP3A4. Very poor (1-year OS, 0%) consisted of three patients who showed positive results in IHC for both GRP94 and CYP3A4. The remaining 35 patients comprised two subgroups: Good (5-year OS 60-80%): 15 patients who showed negative staining for both MDR1 and AKR1C3 and Poor (5-year OS, 10-20%): 20 patients who showed positive staining for either MDR, AKR1C3, MRP1, or p53. The Histological Prognostic Index (HPI) (the four groups: Very poor, Poor, Good, and Very good) is a breakthrough method for stratifying patients based on the factors involved in the development of treatment resistance.

Large rhabdoid meningioma presenting prominent hyperintensity in the optic nerve: An indicator of visual disturbance on constructive interference steady-state sequence?

Background: Rhabdoid meningiomas (RMs) are a rare type of malignant meningioma. Here, we report a case of intracranial RM presenting with visual disturbance and prominent hyperintensity in the optic nerve (ON). Case Description: A 20-year-old female presented with a 1-year history of headache. At presentation, her visual acuity (VA) was 20/50 on the right side and 20/40 on the left, with an intraocular pressure of 17 mmHg on both sides. Cerebral magnetic resonance imaging revealed a broad-based tumor in the right frontal convexity. It measured 82 mm × 65 mm × 70 mm in diameter, accompanied by cystic components, and was inhomogeneously enhanced. The intraorbital ONs demonstrated prominent intramedullary hyperintensity on the constructive interference steady-state sequence. Gross total tumor resection was performed and the pathology was consistent with RM. Immediately after surgery, her VA and IOP were 20/17 and 10 mmHg, respectively, with a remarkable resolution of the intramedullary hyperintensity. Conclusion: Prominent hyperintensity in the ON identified in patients with chronic intracranial hypertension may be an indicator of visual disturbance. It can rapidly resolve after resolution of intracranial hypertension with functional recovery.

Role of TGF-beta1 and TNF-alpha1 produced by neoplastic cells in the pathogenesis of fibrosis in patients with hematologic neoplasms.

We conducted this study with the objective of elucidating the mechanism of development of fibrosis in hematologic neoplasms and develop treatments for these patients. Among the suggested mechanisms of development of fibrosis is cases of hematologic neoplasms is the production of TGF-beta1 (transforming growth factor-beta-1) and TNF-alpha1 (tumor necrotizing factor-alpha-1) by the tumor cells, both of which are fibrosis-stimulating cytokines that act on fibroblasts to promote fibrosis. However, there are few reports based on human clinical pathology studies. We conducted an immunohistochemical study on paraffin-embedded formalin-fixed specimens obtained from 104 patients with various pathologic conditions (acute leukemia, malignant lymphoma, inflammation, cancer, etc.). The association of tissue fibrosis with positive immunohistochemistry for TGF- beta1 and/or TNF-alpha1, TGF-beta1 was found to be strongly associated with tissue fibrosis, and in cases with positive immunohistochemistry for TGF-beta1, the odds ratio for fibrosis was 12.8, which was significantly high. Combined positivity for TGF-beta1 and TNF-alpha1 was also associated with a significant odds ratio for fibrosis of 3.4, suggesting that TGF-beta1 expression is an important prerequisite. TGF-beta1 has been suggested as playing a relatively important role in tissue fibrosis. Future clinical application of these cytokines for both diagnosis and treatment is expected.

Possible association between minor head injury and intratumoral hemorrhage: A metastatic brain tumor from thyroid carcinoma.

A 78-year-old woman presented after a fall and injury in the left forehead. She had undergone surgery for papillary thyroid carcinoma 14 years prior and breast carcinoma 7 years prior. The patient had exhibited uneventful postoperative courses without relapse or metastasis. Anticoagulants or antiplatelet agents were not prescribed her. At presentation, the patient exhibited no focal neurological deficits. Computed tomography revealed a 19 × 20 mm hemorrhagic lesion in the right temporal lobe. On cerebral magnetic resonance imaging, the center of the lesion exhibited inhomogeneous intensity on both T1- and T2-weighted sequences with heterogeneous enhancement. In contrast, the perilesional hemorrhagic regions, appearing hyperintense on both T1- and T2-weighted sequences, showed temporary regression followed by marked enlargement over the subsequent 123 days. The patient underwent total tumor resection. The microscopic findings of the resected specimens were consistent with papillary thyroid carcinoma. Minor head injuries may trigger intratumoral hemorrhage in metastatic brain tumors. Metastasis should be assumed when patients with a history of thyroid carcinoma present with a solitary parenchymal lesion with the appearance of cerebral cavernous malformation, even if they have been disease free for a long period.

Calvarial angiomatous meningioma developed in the diploe.

Background: Angiomatous meningioma is a rare subtype of meningiomas. To the best of our knowledge, there have been no reports of intradiploic angiomatous meningioma. Case Description: A 53-year-old previously healthy woman was diagnosed with a calvarial lesion during a brain checkup. Cerebral magnetic resonance imaging showed an intradiploic tumor, 11 × 14 × 12 mm, in the right parietal bone. It was an enhancing, lobular tumor presenting as isointensity on T1- and hyperintensity on T2-weighted sequences, with an intense enhancement of the adjacent dura mater. Computed tomography revealed bone erosion at the tumor site, extending predominantly into the inner side, and sclerotic changes in the surrounding bone. Total resection was performed. Microscopically, the tumor tissue comprised cells with low-grade meningioma and intervening prominent vasculatures, consistent with angiomatous meningioma. Conclusion: Angiomatous meningioma should be considered as a differential diagnosis when an intradiploic tumor shows a lobular structure, intense enhancement of the adjacent dura mater, and sclerotic changes in the surrounding skull. These findings can support prompt tumor resection.

Calvarial Langerhans cell histiocytosis in an adult presenting rapid growth.

Background: Langerhans cell histiocytosis (LCH) arising in the skull is rare in adulthood. Case Description: A 58-year-old woman experienced a durable headache. Cerebral magnetic resonance imaging (MRI) at presentation showed no abnormalities; however, MRI and computed tomography (CT) performed 6 weeks later revealed the emergence of a well-demarcated, heterogeneously enhancing calvarial tumor accompanied by irregular-shaped bone erosion. On MRI, the temporalis muscle and subcutaneous tissue adjacent to the tumor were extensively swollen and enhanced. The patient underwent en bloc resection. The microscopic appearance of the tumor was consistent with that of LCH. Postoperative systemic 18F-fluorodeoxyglucose positron emission tomography/CT did not detect any abnormal accumulation. Conclusion: LCH may develop within a short period. It should be considered as a differential diagnosis when a rapidly growing calvarial tumor is encountered, even when the patient is an adult. Prompt histological verification is recommended in such cases.

Are bone erosion and peripheral feeding vessels hallmarks of intracranial solitary fibrous tumor/hemangiopericytoma?

An 86-year-old man sustained progressive motor weakness in the left lower extremity for 1 month. Cranial computed tomography (CT) revealed an isodense mass in the right parietal lobe, with a smooth-contoured focal erosion in the adjacent parietal bone. The extra-axial tumor appeared isointense on T1- and hyperintense on T2-weighted magnetic resonance imaging with intense enhancement. On three-dimensional CT angiography, the ectatic left occipital artery coursed into the right parietal foramina and connected with a dilated meningeal vessel supplying the tumor. The focal erosion formed in the inner parietal bone was located adjacent to the feeding vessel. A total tumor resection was achieved. The microscopic findings of the resected specimen were consistent with a World Health Organization grade III hemangiopericytoma (HPC). Bone erosion and peripheral feeding vessels may be characteristic findings of intracranial solitary fibrous tumor (SFT)/HPC. Careful interpretation of neuroimages could help detect clues for distinguishing an SFT/HPC masquerading as a meningioma from a true meningioma.

Atypical meningioma originating from the spinal accessory nerve.

Background: Atypical meningiomas rarely originate from the spinal accessory nerve at the C1-C2 level. Case Description: A 57-year-old female presented with a 1-month history of headache. The cervical MR revealed a well-demarcated intradural/extramedullary tumor compressing the spinal cord at the C1-C2 level that measured 12 mm × 10 mm × 25 mm. She underwent microsurgical tumor resection. Intraoperatively, the tumor was adherent to the spinal accessory nerve, rather than the dura mater. Gross total tumor resection was performed, and the pathology was consistent with an atypical meningioma. Conclusion: Atypical meningiomas rarely originate from the spinal accessory nerve. Gross total resection is the procedures of choice to mitigate the risk of tumor recurrence.

Multiple cerebral infarction diagnosed as Eosinophilic Granulomatosis with Polyangiitis by autopsy.

BACKGROUND: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis of unknown cause involving the brain and accompanied by prominent eosinophilia. Intracardiac thrombosis is a major cardiac complication of EGPA that may cause thromboembolism. CASE PRESENTATION: A 53-year-old man presenting with abulia (consciousness disturbance) and left upper limb paralysis was admitted to our hospital. His case was complicated by penetrating branches, small vessel infarcts, and endocardial thrombosis in the right and left ventricle. Cardiomyopathy was also observed. Sixteen days after admission, the patient died from intracranial hemorrhage. Brain autopsy revealed two major findings: 1) large hemorrhagic infarction caused by cardiac embolism; and 2) granuloma and eosinophil infiltration. Vasculitis was accompanied by eosinophil infiltration in the cortical blood vessels and granuloma. CONCLUSIONS: In this case study, we report autopsy findings of brain infarction in a patient with EGPA and endocardial thrombosis. The brain infarction was caused by the cardiac embolisms and vasculitis.

A Case Report: Ovarian Sertoli-Leydig Cell Tumor With Hyperestrogenism and Endometrial Hyperplasia in a Postmenopausal Woman.

The patient was a 66-year-old woman, G2P2. The patient presented a chief complaint of irregular postmenopausal bleeding 1 month ago. A transvaginal ultrasonography showed that bilateral ovaries were not enlarged and uterine endometrium was thickened, measuring at 9 mm. As a result of endometrial curettage, the simple endometrial hyperplasia was revealed. A blood examination showed an elevated estradiol level of 67 pg/mL, an elevated level of testosterone 0.64 ng/mL, and a slightly suppressed follicle-stimulating hormone (FSH) level of 34.86 mIU/mL. We conducted laparoscopic hysterectomy and bilateral salpingo-oophorectomy because the patient strongly suggested less invasive surgery. The result of pathological diagnosis was Sertoli-Leydig cell tumor (SLCT) in moderately differentiation. A blood examination after a month postoperatively revealed an elevated FSH level of 85.59 mIU/mL, depressed estradiol level of less than 10 pg/mL, and testosterone level of less than 0.03 ng/mL. There was no evidence of recurrence in the first year of follow-up.

Lung metastases from cutaneous adenoid cystic carcinoma 23 years after initial treatment.

Cutaneous adenoid cystic carcinoma (CACC) is an extremely rare neoplasm of head and neck region, and is characterized by propensity for local recurrence and perineural invasion. Late distant metastases occur usually to lungs. Although patients with lung metastases from CACC cannot be cured, long-term survival may be possible due to its slow-growing malignancy. We report a case of a 69-year-old female with lung metastases from CACC 23 years after initial surgery of scalp nodule.

Cotyledonoid dissecting leiomyoma of the uterus: report of two cases.

BACKGROUND: Cotyledonoid dissecting leiomyoma (Sternberg tumor) is a rare variant of the uterine smooth muscle tumor. Although this tumor is a benign tumor clinically and pathologically, the appearance and growth pattern is unusual, so it may be misdiagnosed as malignancy. CASE REPORTS: We report two cases of cotyledonoid dissecting leiomyoma of the uterus that occurred in two 44- and 31-year-old women, respectively. Total hysterectomy and bilateral salpingo-oophorectomy were performed in one of the patients, and myomectomy was done in the other one. Macroscopically, both tumors were grape-like exophytic masses resembling placental tissue. The patients were well after surgery, and one patient gave birth. To our knowledge, this is the first case report of a successful delivery after myomectomy of this tumor. CONCLUSION: To prevent aggressive surgery it is important to recognize that this tumor is a benign and unusual appearing variant of leiomyoma. A fertility-sparing surgical procedure should be considered if the patient wishes to preserve her fertility.

The utility of serum N-ERC/mesothelin as a biomarker of ovarian carcinoma.

Ovarian carcinoma has been difficult to diagnose at an early stage. Recently, it has been recognized that the measurement of blood N-ERC/mesothelin levels aids early detection in and postoperative therapeutic monitoring of patients with mesothelioma, who have been exposed to asbestos. ERC/mesothelin has also been reported to be expressed in ovarian carcinoma. We determined serum N-ERC/mesothelin levels in patients with ovarian carcinoma using an enzyme-linked immunosorbent assay (ELISA). In addition, we immunohistochemically evaluated surgically resected specimens for C-ERC/mesothelin expression. As a result, of the 32 patients with ovarian tumors (18 carcinoma, 2 borderline tumors), one patient with serous adenocarcinoma showed increased N-ERC/ mesothelin levels. Immunohistochemically, of the 20 ovarian tumor (carcinoma and borderline tumor) specimens evaluated for serum N-ERC/mesothelin, 9 (45.0%) were positive for C-ERC/mesothelin. The C-ERC/mesothelin-positive specimens were found to be serous and clear cell adenocarcinomas. If serum N-ERC/mesothelin, which is considered useful for early detection in and therapeutic monitoring of patients with mesothelioma, may also be used for ovarian carcinoma monitoring, it may be a valuable serum tumor marker for the early detection of ovarian carcinoma.

[The significance of the combined usage of cytology and the HPV test in cervical cancer screening].

BACKGROUND: In the United States, the combined usage of cytology and the HPV test is employed in cervical cancer screening, but, in Japan, only cytology is performed in most cases. We conducted a comparative analysis of histopathological diagnoses by colposcopy, cytology, and the HPV test, and evaluated the effectiveness of cytology and the significance of its combined use with the HPV test. METHODS: We compared the detection rates using the test for high-risk HPV and cytology, which were conducted in cases with various histological diagnoses. The hybrid capture method was used in the HPV test. The blind test was conducted by 3 cytologists in 14 cases in which the cytological differed from the histological diagnosis by more than 2 classes. RESULTS: There were 89 cases above class IIIb or CIN 3, and 26 cases were below class IIIa. A total of 107 cases were positive on the HPV-DNA test, and 8 cases were negative. CONCLUSION: Cytology is essential as a morphological diagnostic method in cancer screening. In contrast, the HPV test detects the cause of cervical cancer. Therefore, the combined usage of cytology and the HPV test as initial screening for cervical cancer would facilitate the diagnosis of a premalignant lesion or early-stage cervical cancer more promptly and accurately.