[A Case of Primary Intrasellar Chondroid Chordoma].

OBJECTIVES: Intracranial chordomas are thought to arise from remnants of the notochord and usually occur at the parasellar region. We present a case of a primary intrasellar chondroid chordoma in a patient who was initially diagnosed with a pituitary adenoma. CASE: A 77-year-old woman had a history of two surgeries for a tumor in the sella turcica(17 months after the 1st surgery). On initial MRI, the intrasellar mass showed low signal intensity on T1WI, very high signal intensity on T2WI, and inhomogeneous enhancement. On bone reconstructive CT, the sellar floor was thin, and no abnormalities were observed at the top of the clivus. Transsphenoidal surgery was performed. The pathological diagnosis was pituitary adenoma in both cases. Seventy-two months after the 1st(31 months after the 2nd)surgery, she developed right-sided oculomotor and abducent nerve palsies again. Since recurrence occurred during the relatively short period, the surgical specimens obtained from the 1st and 2nd surgeries were reexamined. Reexamination of the previously obtained specimen demonstrated areas of chondroid tissue that were embedded in a mucoid stroma and tumor cells that were composed of round or pleomorphic nuclei with vacuolated cytoplasm(physaliphorus cells)that were compatible with chondroid chordoma. The third surgery was performed. Postoperatively, her symptoms improved, and cyber knife therapy was administered for the residual part of the tumor. CONCLUSIONS: Although intrasellar chondroid chordomas are extremely rare, they should be considered in the differential diagnosis of tumors located in the sella turcica.

Nuclear inverse polarity papillary lesions lacking myoepithelial cells: A report of two cases.

Here, cases of a 68- (Case 1) and a 44-year-old (Case 2) female are presented. They had an abnormality in the breast, and came to our hospital for further examination and treatment. Radiologically, malignancy could not completely excluded so breast excision was performed. Histologically, both cases revealed papillary neoplastic lesions lined by fibrovascular core and nuclear inverse polarity without atypia. Loss of myoepithelial cells was observed by HE, p63, and calponin. Previous report indicate CK5/6, ER, p63 and MUC3 are important for distinguishing between papillary lesions according to the differential index (based on Allred score) of ([ER total score] + [MUC3 total score])/([CK5/6 total score] + [p63 total score] + 1). Based on this analysis, our two cases had benign lesions. However, based on immunopositivity for cell-cycle marker Cyclin-D1, Case 1 was negative, and Case 2 was about 70% positive. Additionally, the Ki-67 index was <1% in both cases, and no evidence of disease was observed after a maximum 62 months of follow-up in both cases, despite lack of additional treatment. Thus, we propose that lack of myoepithelial cells in papillary lesions do not necessarily indicate malignancy and are thought to be, at the most, uncertain malignant potential.

[A Case of Primary Extradural Meningioma Located at the High Convexity:A Case Report and Review of the Literature].

OBJECTIVES: Primary meningiomas arising outside the intracranial component are rather rare and have been termed primary extradural meningiomas(PEMs). We present a case of an intraosseous-type PEM occurring at a high convexity location and discuss the clinical characteristics of PEMs. CASE: An 80-year-old woman presented with a soft and painless subcutaneous mass of approximately 10 cm in diameter in the right parietal region, which had appeared 1 year previously. Mild cognitive dysfunction and left hemiparesis were observed upon admission. A skull radiograph and a bone window computed tomography scan revealed an osteolytic lesion at the above-mentioned site. Magnetic resonance imaging indicated that the mass was inhomogeneously enhanced and seemed to extend through the skull defect both intra- and extra-cranially. The preoperative diagnosis was a metastatic skull bone tumor due to the patient's history of breast cancer. During surgery, the tumor was found to be solid and had expanded through both the inner and outer tables of the skull, destroyed the inner table at one location, and perforated into the subdural space via the thinned, but not infiltrated, dura mater. The tumor was removed along with a wide margin of surrounding healthy bone and a cranioplasty was performed using a titanium plate. The histopathological diagnosis was atypical meningioma(World Health Organization grade II). Postoperatively, no adjuvant therapies(radiation and/or chemotherapy)were administered and the patient was reported to be well at 8 months post-surgery with no evidence of tumor recurrence. CONCLUSIONS: Although PEMs are rather rare, clinicians should consider the differential diagnosis of osteolytic skull vault tumors.

[De Novo Cavernous Angioma Secondary to a Developmental Venous Anomaly:A Case Report and Review of the Literature].

OBJECTIVE: Previously, cavernous angiomas(CAs)have been thought to be only congenital in origin. Recently, however, a few cases of de novo CAs have been reported in the literature. We present a case of a de novo CA and discuss the etiology of the newly appeared CA. CASE REPORT: A 29-year-old man was presented to a local clinic because of hypersomnia. MRI demonstrated a heterogeneous mass peripherally located, which was in contact with a developmental venous anomaly(DVA)at the left thalamus. Six years before the presentation, he visited the same clinic because of faintness, and MRI results indicated no abnormality except for the DVA. Three weeks later, he suddenly experienced difficulty in speech, and the MRI revealed an increase in the size of the mass. Subsequently, he was admitted at our institution, and neurological examination revealed aphasia and right hemiparesis. A left carotid angiogram on venous phase showed a narrowing of the DVA, which was seen as it entered the internal cerebral vein. The diagnosis of a de novo CA was made. The mass was completely resected through the transcallosal transventricular approach to avoid injuring the DVA. The DVA could not be found during surgery. The pathological diagnosis was in line with the findings of CA. Postoperatively, the patient continued having difficulty in speech and was transferred to another institution for speech rehabilitation. CONCLUSIONS: Although the association of CA and DVA has been described with increasing frequencies recently, the etiology of de novo CA in the case of this association has been a matter of debate. In the present case, it was speculated that a narrowing of the DVA resulted in increased venous pressure and caused the development of de novo CA.

[Dural cavernous angioma:a case report and review of the literature].

Here, we report a case of dural cavernous angioma. A 54-year-old man presented with headache in his right frontal area for the previous few weeks. Computed tomography (CT) and magnetic resonance imaging revealed a well-demarcated extramedullary mass, 3 x 2.5cm in size, within the subdural space at the right frontal region. The mass was not enhanced on a contrast-enhanced CT scan, and heterogeneously enhanced after administration of Gd-DTPA on magnetic resonance images. Cerebral angiography revealed an avascular mass. Right frontal craniotomy was performed. On surgery, the mass was found to be mainly extramedullar, and partially intramedullar. No adhesion between the mass and the overlying dura was observed. It was removed in an en bloc fashion. The pathological diagnosis was cavernous angioma. The patient's headache was resolved soon after surgery. Cavernous angiomas are usually intramedullar in the subcortical white matter of the cerebral hemispheres;extramedullary cavernous angiomas are rare. Extramedullary dural cavernous angiomas located in the subdural space at the cerebral convexity are extremely rare. They usually present with headache or mass signs, and resemble meningioma radiologically. During surgery they are easily resected with minimal blood loss. Dural cavernous angiomas should be considered in the differential diagnosis of intradural extramedullary mass at the cerebral convexity.

Knowledge of mucin immunostaining status of "nuclear inverse polarity papillary lesions lacking myoepithelial cells" may prevent unnecessary breast surgery: experience of two cases.

We previously reported on two women with breast lesions in whom radiological examination could not exclude malignancy. In both cases, mastectomy was performed, and histological analyses revealed papillary lesions lined by fibrovascular stroma and nuclear inverse polarity. Hematoxylin-eosin, p63, and calponin staining indicated an absence of myoepithelial cells. However, it was concluded that the lesions had been non-malignant. These women have now been under long-term surveillance (74 months for one case and 62 months for the other) and have had no disease recurrence. Mucin (MUC)1, MUC2, MUC4, MUC5AC, MUC5B, and MUC6 immunostaining has also been performed in these women to investigate further whether their tumors were malignant or benign. In both cases, the tumors were only positive for MUC1 in apical luminal apical cells, as in normal breast tissue. MUC5B immunostaining, even when weak, can detect early breast cancer but was completely negative in our two cases. Therefore, both tumors were considered benign. Our findings in these cases suggest that nuclear inverse polarity papillary lesions lacking myoepithelial cells are benign. This knowledge should decrease the number of unnecessary operations performed for this tumor and their negative impact on patients' quality of life.

A collision between vascular adrenal cyst and adrenocortical adenoma.

We report a patient with sigmoid colon cancer who revealed a unique collision of hemorrhagic vascular adrenal cyst and adrenocortical adenoma with myelolipomatous changes. Two months before referral to our hospital, anticoagulant therapy was started for acute myocardial infarction. The components of the adrenocortical adenoma demonstrated a typical signal drop in opposed-phase magnetic resonance (MR) images although macroscopic fat was also depicted both on CT and MR images. The components of the vascular adrenal cyst demonstrated peripheral nodular enhancement with progressive enhancement on dynamic contrast-enhanced CT and a hemorrhagic change in the central region, which showed hyper intensity on T1-weighted images (T1WI) and hypo intensity on T2-weighted images (T2WI). Microscopically, the cyst was filled with foci of hemorrhage, fibrin, fibrosis, and hemosiderin. Furthermore, a white thrombus was found that corresponded to the central low signal intensity depicted on T2WI. Dilated vascular channels that were immunohistochemically positive for CD31 and CD34 were identified within the cyst. They were consistent with the pathological findings of hemorrhagic vascular adrenal cyst. Radiologists should be aware that the diagnosis of adrenal vascular cyst could be challenging since image findings may resemble hemangiomas, pheochromocytomas, and malignancy.

Recurrent hyperparathyroidism due to parathyroid and pulmonary tumors showing features of parathyroid adenoma.

We report on a case of recurrent primary hyperparathyroidism possibly caused by parathyroid adenocarcinoma metastasizing to the lung. A 46-year-old woman with a history of parathyroid adenoma, which was extirpated 8 years ago, presented with symptoms of primary hyperparathyroidism, and was found to have a parathyroid and a lung nodule in radiographic assessments. Resections of the tumors in the parathyroid gland as well as the lung were required to improve her condition, and in pathology, both tumors demonstrated benign features consistent with adenoma. However, from the perspective of the clinical course and location of the tumors, we deduced that the tumors were malignant despite being identified as benign by conventional pathological examination. The integration of information based on clinical status and imaging studies is essential to evaluate the malignant potential of tumors if a patient with hyperparathyroidism has tumors located both inside and outside of the parathyroid gland.

Primary cardiac rhabdomyosarcoma following a uterine leiomyosarcoma: double primary sarcomas.

Primary cardiac rhabdomyosarcoma is rare. A dismal prognosis is usually given due to its high propensity for distant metastases, with survival rarely exceeding 2 years. We report a case that was initially considered to be a cardiac metastatic tumor from a primary uterine leiomyosarcoma, but after morphological and immunohistochemical examinations was shown to be a primary cardiac rhabdomyosarcoma. The right atrium was filled with a mass that oscillated between the right atrium and ventricle through the tricuspid valve. To reduce the risk of sudden death from tumor embolism into the pulmonary arteries, the tumor was resected. Even though the surgery was not curative, it eliminated the risk of sudden death due to tumor embolism into the pulmonary arteries and improved short-term survival.