Clear cell meningioma with anaplastic features: Case report and review of literature

Clear cell meningioma (CCM) is an uncommon variant of meningioma, corresponding to WHO grade II. We present two cases of CCMs with anaplastic features in the intracranial and intraspinal region. The first case is a 65-year-old male who gradually developed changes in behavior over a period of 1 year. The second case is a 35-year-old female who presented with a 7-month history of posterior cervicothoracic pain and dysuria for 1 week. Magnetic resonance imaging revealed an intracranial lesion in the right frontal lobe in the male patient, and an intradural extramedullary lesion at C7 in the female patient. On histological examination, both tumors partly exhibited unusual anaplastic appearances with nuclear pleomorphism, high mitotic activity and necrosis, distinct from classical CCMs. Tumor cells were immunoreactive to epithelial membrane antigen (EMA) and vimentin, with a high MIB-1 index up to 40%. Total excision was performed. The male patient was found to have developed local recurrence and lateral ventricle metastasis 3 months after surgery. A diagnosis of CCM with anaplastic features was made (WHO grade III). Based on its aggressive behavior, we recommend postoperative adjuvant radiotherapy or chemotherapy even if total excision of the tumor has been performed, and MRI scans every 3–6 months during the first period of follow-up.     

A rare case of malignant pediatric ectomesenchymoma arising from the cerebrum

Malignant ectomesenchymoma is a rare tumor that contains both ectodermal and mesenchymal elements. So far, only 7 patients with a manifestation in the cerebrum (with confirmed clinicopathological data) have been reported. A 4-year-old girl was present at our hospital with a 3-week history of intermittent sudden dizzy with no apparent cause. MRI showed an irregular enhanced lesion in the left frontal-parietal lobe and lateral ventricle with peripheral gadolinium-enhancement with a significant surrounding edema. Total removal of the tumor was performed. Histological examination of the resected tumor revealed a mixed astrocytoma and anaplastic ependymoma component with undifferentiated mesenchymal spindle cell component. Generally speaking, the main malignant part in most cases of malignant ectomesenchymoma (MEM) is the mesenchymal component. In the present case, the malignant component was both in the mesenchymal and ectodermal part. In particular, the mesenchymal part was mainly composed of spindle cells, and the ectodermal part primarily consisted of gliomatous component and anaplastic ependymoma component. The patient was then treated with chemotherapy and as regard to the prognosis, there was no evidence of tumor recurrence at the 5 months’ follow-up. The long term follow-up is still in progress.     

MALIGNANT GANGLIOGLIOMA OF THE SPINAL CORD

A case of tumor of the spinal cord with a long clinical history was described. At autopsy, the tumor was composed of a mixture of two elemental cells, i.e. matured but neoplastic ganglionic cells and anaplastic glial cells. The clinical manifestations suggested that the tumor in this case developed in a benign form with malignant evolution occurring during the latter part of its clinical course. The malignant evolution, however, was due to aggressive and anaplastic growth only of the elemental glial cells, while the cytologic features of the elemental ganglionic cells remained always benign.     

Malignant ganglioglioma of the spinal cord

A case of tumor of the spinal cord with a long clinical history was described. At autopsy, the tumor was composed of a mixture of two elemental cells, i.e. matured but neoplastic ganglionic cells and anaplastic glial cells. The clinical manifestations suggested that the tumor in this case developed in a benign form with malignant evolution occurring during the latter part of its clinical course. The malignant evolution, however, was due to aggressive and anaplastic growth only of the elemental glial cells, while the cytologic features of the elemental ganglionic cells remained always benign.     

Gliosarcoma arising from an anaplastic ependymoma: a case report of a rare entity

We report the first case of a gliosarcoma arising from an anaplastic ependymoma and the second case of gliosarcoma arising from any type of ependymal neoplasm. The patient was a 48-year-old woman with a solid and cystic, peripherally enhancing, 7-cm right frontal mass lesion. Histologically, the lesion displayed characteristics of anaplastic ependymoma (grade III, World Health Organization scale). The tumor recurred despite multiple cycles of postoperative radiation and chemotherapy. After the fourth recurrence, the tumor displayed a biphasic pattern of differentiation. The first pattern was similar to the original anaplastic ependymoma, whereas there was a second new sarcomatous pattern resembling fibrosarcoma that was admixed with and overrunning the ependymal component. The spectrum of gliosarcoma is therefore expanded to include not only astrocytic and oligodendroglial components but ependymal components as well.     

Primary carcinosarcoma of the vagina

Primary carcinosarcoma of the vagina is a very rare tumor, with only eight cases diagnosed as carcinosarcoma in the literature that we are aware of. We recently encountered a case of primary carcinosarcoma of the vagina in a 75-year-old woman. The patient had a history of hysterectomy and bilateral ovariectomy for uterine corpus cancer at 55 years of age. Recurrence of the cancer was suspected 17 years after the operation and irradiation therapy was performed, but the patient died 3 years after the recurrence. Autopsy revealed a mass lesion in the pelvic cavity that originated in the vagina. Histological examination showed that the tumor contained anaplastic carcinoma, rhabdomyosarcoma, leiomyosarcoma and chondrosarcoma components, and it was diagnosed as carcinosarcoma. The histological diagnosis of the uterine corpus cancer was well-differentiated adenocarcinoma, and there was no sarcomatous component. The carcinosarcoma occurred 17 years after the hysterectomy, and it was concluded to be a primary carcinosarcoma of the vagina. This is the first case of primary vaginal carcinosarcoma in which the epithelial and sarcomatous components were clearly identified histologically and immunohistochemically.     

Posttransplant primary cutaneous Ki-1 (CD30)+/CD56+ anaplastic large cell lymphoma

An anaplastic large cell lymphoma that was negative for Epstein-Barr virus and positive for Ki-1 (CD30) presented as a polypoid scalp mass in a 56-year-old man 16 years after renal transplantation. The lymphoma was of the CD4+ cytotoxic T-cell lineage, and the tumor cells also expressed CD56. Despite reduction in the dose of immunosuppression and localized radiotherapy, the tumor had rapidly progressed to involve the soft tissue of the right hand. Systemic chemotherapy induced complete regression of the soft tissue lesion. This case illustrates that posttransplant primary cutaneous CD30+ anaplastic large cell lymphomas may assume an aggressive clinical course but can still be controlled by systemic chemotherapy.     

Ovarian mucinous tumour with a focus of anaplastic carcinoma: a case report

A 50-yr-old patient presented with abdominal symptoms due to the presence of cystic ovarian tumours. After hysterectomy and bilateral salpingo-oophorectomy, the diagnosis of a mucinous tumour with a focus of anaplastic carcinoma in one ovary and a dermoid cyst in the other was made. Further treatment was refused and the patient died 12 mth after operation with disseminated anaplastic carcinoma. The case is the first reported when adjuvant therapy has not been given and illustrates the poor natural history of this disease.     

Anaplastic carcinoma of the thyroid gland with chondrosarcomatous component

The case of an 83-year-old female with a history of a two months enlarging goiter is presented. Clinically, a firm goiter with fixation to surrounding soft tissues was found. Fine needle aspiration cytology was performed with the conclusion of a sarcomatoid variant of anaplastic carcinoma of the thyroid gland; biopsy was recommended. The patient was operated on and died six months after operation with metastatic dissemination in lymphatic nodes and lungs. Histologically, an invasive high-grade tumor composed of irregular, sometimes bizarre spindled or polygonal cells intermingled with foci of chondroid tissue was seen. The cartilage comprised lobularly arranged chondrocytes with irregular nuclei; bi- or multinucleated chondrocytes were present as well. The diagnosis of thyroid anaplastic carcinoma with chondrosarcomatous component was established. Differential diagnostics of this tumour is discussed.     

A case of primary gastric CD30-positive anaplastic large-cell lymphoma

Gastric CD30-positive anaplastic large-cell lymphoma is a very rare disease. It is sometimes difficult to distinguish it from undifferentiated carcinoma, sarcoma and so on. We report here on a case of primary gastric anaplastic large-cell lymphoma. A 50-yr-old woman complained of epigastric pain and severe chest pain for 1 week. The gastroendoscopic examination revealed geographic mucosal irregularities with shallow ulceration at the antrum. She underwent a total gastrectomy. The gross finding of the resected stomach was an 8 x 4.5 cm sized ulceroinfiltrative lesion at the pyloric antrum along the lesser curvature. The microscopic examination revealed diffuse and solid proliferations of large atypical cells with pleomorphic nuclei. Immunohistochemically, the tumor cells were positive for CD30, vimentin and CD3, and this was a finding compatible with anaplastic large-cell lymphoma. To the best of our knowledge, this is the first such reported case in Korea.     

MALIGNANT LYMPHOEPITHELIAL LESION

A case of undifferentiated carcinoma arising from benign lymphoepithelial lesion (BLEL) of the parotid gland was studied by light and electron microscopy. Histopathologically, the carcinoma was composed of pleomorphic anaplastic cells showing an undifferentiated type among abundant lymphoid tissue forming germinal center. Among the prominent lymphoid tissue, epithelial hyperplasia, dysplasia, and squamous metaplasia of the duct epithelium were found. Dysplastic epithelium revealed a transition with carcinomatous component in some areas. On the electron microscopic observation, the tumor cells were poorly differentiated, possessing desmosomes and intracytoplasmic filaments. The patient is alive and well 2 months after resection of the tumor, but has a high titer of serum Epstein-Barr virus capsid antigen in IgG. Eighty five cases of the malignant lymphoepithelial lesion (MLEL) including the present case are summarized.     

Malignant lymphoepithelial lesion

A case of undifferentiated carcinoma arising from benign lymphoepithelial lesion (BLEL) of the parotid gland was studied by light and electron microscopy. Histopathologically, the carcinoma was composed of pleomorphic anaplastic cells showing an undifferentiated type among abundant lymphoid tissue forming germinal center. Among the prominent lymphoid tissue, epithelial hyperplasia, dysplasia, and squamous metaplasia of the duct epithelium were found. Dysplastic epithelium revealed a transition with carcinomatous component in some areas. On the electron microscopic observation, the tumor cells were poorly differentiated, possessing desmosomes and intracytoplasmic filaments. The patient is alive and well 2 months after resection of the tumor, but has a high titer of serum Epstein-Barr virus capsid antigen in IgG. Eighty five cases of the malignant lymphoepithelial lesion (MLEL) including the present case are summarized.     

Encapsulated anaplastic thyroid carcinoma with three-year disease-free survival

This is a report of an unusual anaplastic thyroid carcinoma case with capsule and long disease-free survival. This 74-year-old male, with an asymptomatic nodule in the mid-line neck for 5 years, was treated with total thyroidectomy. Light microscopy and immunohistochemistry showed an anaplastic carcinoma of the thyroid isthmus. There were no signs of carcinoma elsewhere either at the time of surgery or 35 months later, when the patient was last seen.     

Mucinous tumor of the gallbladder with a separate nodule of anaplastic carcinoma

A case of mucinous tumor of the gallbladder with a separate nodule of anaplastic carcinoma is reported. The patient was an 83-year-old Japanese man who underwent cholecystectomy under the preoperative diagnosis of a mucus-producing gallbladder tumor. A mucinous tumor was found in the neck and distal body of the gallbladder, associated with a separate nodule in the fundus. The latter nodule was initially diagnosed as a benign xanthogranulomatous lesion. However, the immunohistochemical study revealed that the atypical cells in the superficial part of the nodule were positive for cytokeratin and epithelial membrane antigen, confirming the diagnosis of anaplastic carcinoma. Although the occurrence of mural nodules in mucinous cystic tumors of the ovary and pancreas is well reported, to our knowledge, this is the first report on the occurrence of a mucinous tumor with a nodule of anaplastic carcinoma in the gallbladder.     

Cerebellar pleomorphic xanthoastrocytoma in a patient with neurofibromatosis type 1: a case report and literature review

Pleomorphic xanthoastrocytoma (PXA) is an uncommon tumor of young adults that typically occurs supratentorially. It is generally considered to be a low-grade, circumscribed tumor that when treated by surgical resection has a relatively favorable outcome. Cases of cerebellar PXA are rare, and those associated with neurofibromatosis type 1 (NF1) are even less common, with only 2 cases reported to date. We present herein a third case of PXA-NF1 with unusual features. A 33-year-old woman presented with a history of headache. Her medical and family history was significant for NF1. Brain MRI revealed a 3.4 cm ill-defined lesion with a gyriform enhancing pattern in the left cerebellum, superficially mimicking Lhermitte-Duclos disease. The patient underwent a gross total resection of the lesion and had an unremarkable postoperative course. While the lesion had histological features typical of “pure” PXA (WHO grade II) it had an unusual growth pattern with thickening of the superficial cerebellar folia and predominant leptomeningeal involvement. No BRAF, IDH-1, or IDH-2 mutation was identified. Three months after surgery, local recurrence was detected, and the patient was treated with radiation therapy. One year after the first surgery, she underwent surgical resection of the recurrent/residual tumor. Histologically, the recurrent tumor showed very similar features to the initially resected tumor, with no anaplastic features. Most cerebellar PXAs have an indolent clinical behavior as do most cerebral PXAs. Whether co-existence of NF1 was a factor in altering the clinical course and biologic behavior of this patient’s tumor is currently unknown.     

Atypical adenoma of the thyroid diagnosed as anaplastic cancer by cytopathology

Atypical adenoma of the thyroid is a rare form of tumor, and its accurate diagnosis prior to surgical resection is difficult as the histological and pathological morphologies are very similar to those of anaplastic thyroid carcinoma (ATC), and its anaplastic transformation remains to be elucidated. We reported a case of a 75‐year‐old female with a thyroid isthmus nodule diagnosed repeatedly by FNAC as anaplastic carcinoma. Both the first and second FNAC specimen slides showed a large number of scattered or aggregated atypical cells consisting of large, pleomorphic nuclei with irregular membranes, chromatin clumps and prominent nucleoli. The morphology of the surgical specimen was similar to that of an anaplastic carcinoma and although it showed signs of transition from a normal follicular epithelium, there was no invasive growth or mitosis. This lesion was diagnosed as an atypical adenoma, and a papillary carcinoma was also present in the right lobe of the thyroid. Here we evaluate the molecular features of atypical adenomas in comparison with 9 ATC samples, and discuss whether or not atypical adenomas represent a form of premalignant lesion. Ki‐67 expression was found to be very low in atypical adenomas whereas all ATC samples showed high levels of Ki‐67 expression. Epithelial–mesenchymal transition (EMT) marker expression suggested that atypical adenomas maintain their epithelial phenotype to a higher degree than do ATCs. Differential diagnosis between ATC and atypical adenoma is difficult by cytological and histological methods alone, and Ki‐67 and EMT marker expression may support the diagnosis.     

Pigmented papillary epithelial neoplasm of the pituitary fossa: a distinct lesion of uncertain histogenesis

Primary pigmented intracranial neoplasms are strikingly uncommon. The differential diagnosis is limited and includes both epithelial and nonepithelial tumors, most of which arise within or near the ventricular system. The authors describe a 42-year-old man who presented with a pigmented papillary epithelial lesion that arose within the sella and exhibited suprasellar extension and bony erosion. Following external beam radiotherapy and multiple surgical resections, tumor growth became rapid, necessitating additional debulking procedures. Pathologic evaluation of subsequent lesional tissue samples revealed an anaplastic lesion with malignant epithelial and spindle cell components. Occasional epithelial cells showed features reminiscent of the original papillary lesion, whereas others exhibited oncocytic morphologic features. This case represents the only report, to our knowledge, of a pigmented papillary epithelial neoplasm arising within the pituitary fossa. Although the histogenesis of this tumor is enigmatic, this appears to be a distinct lesion characterized by aggressive growth and the capacity for anaplastic progression.     

Primary anaplastic large cell lymphoma of central nervous system--a case report

Central nervous system (CNS) involvement is extremely rare in anaplastic large cell lymphoma (ALCL). Primary ALCL of CNS on radiology is often misdiagnosed as tuberculosis. We report a fatal case of primary ALCL of CNS in a 17 year old male. He came with history of headache and left partial seizures. MRI showed a well- circumscribed lesion in the right fronto-parietal lobe eroding the skull bone. Biopsy showed large pleomorphic cells. Immunohistochemical stains showed positivity for CD30, CD43, EMA and ALK-1. In spite of radiotherapy and steroids, patient expired. Hence a high level of suspicion is essential for early diagnosis and for instituting appropriate treatment.     

Warthin tumor-like variant of papillary thyroid carcinoma: A case with dedifferentiation (anaplastic changes) and aggressive biological behavior

Warthin tumor-like variant of papillary thyroid carcinoma is uncommon and approx 80 cases have been reported in the literature. This tumor is often associated with a favorable prognosis. In this report, a Warthin tumor-like variant of the papillary thyroid carcinoma, 5-cm in maximum dimension, underwent anaplastic changes in a 74-yr-old woman. The tumor was positive for CD15 and EMA, and a high proliferative index was noted in the anaplastic area. The patient developed distant metastases after operation and died of the disease 18 mo after the operation. The present case is the first reported case of Warthin tumor-like variant of papillary thyroid carcinoma with anaplastic changes.