Lhermitte-Duclos disease: A series of six cases.

The Lhermitte-Duclos disease (LDD), also known as dysplastic cerebellar gangliocytoma, is a rare lesion characterized by variable enlargement of cerebellar folia. The pathological basis of LDD has long been debated, as it has overlapping features of both, a neoplasm and hamartoma. Association between LDD and Cowden syndrome (CS) has been established based on the presence of phosphatase and tensin homologue germline mutation in both. We present a series of six cases of LDD: Four females and two males, aged between 16 and 38 years, presenting with headache and imbalance on walking of 1-7 months duration. Histomorphology showed thickening and vacuolation of the molecular layer, loss of Purkinje cells, and replacement of granular cell layer by large dysplastic ganglion cells. Awareness of histological features of this rare entity and a higher level of suspicion is required for the correct diagnosis, which, in turn, should prompt thorough investigations to exclude features of associated CS. LDD is a rare entity, awareness of its histological features and correlating them with radiology is essential, especially in tiny biopsies; to render the correct diagnosis. Diagnosis of LDD warrants further clinical workup and close follow-up for the associated features of CS.

Glioblastoma Occurring as Second Primary in a Treated Case of Diffuse Large B-Cell Lymphoma.

Glioblastoma as second primary malignancy (SPM) has been reported after prostate cancer, meningiomas, Hodgkin's lymphoma. We report an extremely rare case of glioblastoma as SPM, occurring after remission of diffuse large B-cell lymphoma (DLBCL). Fifty-year-old male presented with loss of consciousness followed by right-sided weakness. He was treated with chemotherapy for DLBCL of the cervical lymph nodes, 5 years back. Present scans revealed well-defined intra-axial lesion in the left parietal lobe, suggestive of central nervous system (CNS) involvement by lymphoma. Left parieto-occipital craniotomy was performed and microscopic examination revealed the tumor to be Glioblastoma, WHO Grade IV. The tumor cells were positive for glial fibrillary acid protein and negative for leucocyte common antigen. He was treated by radiotherapy and temozolomide. Pathologic examination is a must for CNS lesions. Had it not been for the biopsy, the patient would have been treated as a recurrence of CNS lymphoma by chemotherapy and would have probably succumbed.

Solitary Plasmacytoma with Amyloid - An Unusual Dural-Based Lesion.

Solitary plasmacytoma of the dura without systemic involvement are extremely rare lesions, with <15 cases reported in the literature. Among these, ours is the second case to show the presence of amyloid. Fifty-year-old male had presented with headache, sudden onset right-sided weakness, and vomiting. Magnetic resonance imaging revealed an extra-axial mass in the left fronto-parietal region measuring 10 cm × 8.7 cm × 3.9 cm, suggestive of meningioma. The left fronto-parietal craniotomy was performed and multiple tissue bits aggregating to 10 cm × 8.5 cm × 2 cm along with thinned out membrane-like bit of calvarium was sent for pathologic examination. H and E stained sections showed sheets of plasmacytoid cells along with amyloid, which showed apple-green birefringence on Congo red staining. On immunohistochemistry, tumor cells were positive for CD38, CD138, showed kappa light chain restriction and were negative for CD45, CD34. Hence, it was diagnosed as a plasma cell neoplasm. Further work-up with whole-body positron-emission tomography scan revealed no systemic involvement. Dural-based lesions can mimic meningioma radiographically as well as intraoperatively. Histopathological examination unveils the diagnosis, to guide appropriate therapeutic regimens.

Meningiomas with Collagenous Rosettes: A Report of Three Cases.

Formation of rosettes is very rarely encountered in meningiomas. The 2016 WHO classification of central nervous system tumors mentions it as a rare pattern secondarily encountered in different variants. We report three cases of meningiomas forming collagenous rosettes. Case 1 was a 60-year-old male with a right frontoparietal mass lesion. Excisional biopsy showed features of atypical meningioma (WHO Grade II) with diffusely scattered collagenous rosettes. Case 2 was a 48-year-old male with right frontoparietal space-occupying lesion. Microscopy revealed a papillary variant of meningioma (WHO Grade III) with prominent diffusely scattered collagenous rosettes. Case 3 was a 75-year-old female with left parietal convexity tumor. Microscopy revealed a clear-cell meningioma (WHO Grade II) with cerebral invasion. Focal collagenous rosettes and fibrosclerotic whorls were noted. In all three cases, Masson's trichrome was used to confirm collagenous nature of the rosettes. All three tumors were positive for epithelial membrane antigen and vimentin.

Chondrosarcoma of the dorsal spine - A rare case.

Chondrosarcomas of the spine are rare tumors and represent <10% of all chondrosarcomas. In the spine, they may arise from vertebral bodies or posterior elements. They may occur in patients ranging from 13 to 78 years of age. Here, we present a case of a 25-year-old female who presented with complaints of lower backache, stiffness in both lower limbs, and tingling sensation in the right lower limb. On examination, both power and sensations were decreased below waist. Magnetic resonance imaging spine revealed an extradural lesion at D5 vertebral body level with severe cord compression. We received the mass in multiple fragments which were grayish-white and firm to hard in consistency. Microscopically, a chondroid tumor was seen with cells arranged in lobules in abundant myxoid matrix. The neoplastic chondrocytes were large in size and had bizarre hyperchromatic nuclei. Few binucleate and multinucleate forms were also seen along with occasional atypical mitoses. There was permeation and destruction of the host bone, and the tumor was seen invading the marrow spaces. Few foci showed high cellularity. No osteoid formation was seen by the tumor. The tumor was diagnosed as Chondrosarcoma - Grade II.

Primary Pineal Rhabdomyosarcoma: A Rare Case.

Primary pineal rhabdomyosarcoma (RMS) is extremely rare, and only three cases have been reported so far. Here, we report a case of 12-year-old male who presented with complaints of diplopia and diminution of vision since 15 days. He also had left-sided facial paresis. Magnetic resonance imaging brain revealed a space-occupying lesion in the region of pineal gland. The patient underwent midline suboccipital craniectomy with excision of tumor. Microscopic examination revealed a highly cellular tumor with areas showing small round cells admixed with cells having abundant eosinophilic cytoplasm resembling rhabdomyoblasts and multinucleated giant cells. Differential diagnoses of pineal anlage tumor and primary RMS were considered. The tumor cells were positive for desmin while being negative for synaptophysin and glial fibrillary acidic protein. Myogenin was used to confirm the diagnosis of RMS, which showed focal nuclear positivity. INI1 was retained. All the markers for germ cell tumors were negative.

Angiocentric Glioma: Report of a Rare Case Presenting with Psychosis.

Angiocentric glioma (AG), first described in 2005, was included as a distinct entity in the 2007 World Health Organization Classification of Tumors of the Central Nervous System. It is a very rare cerebrocortical tumor mainly affecting children and young adults with a history of intractable partial seizures. The histopathological features of this entity are perivascular arrangement of monomorphic, bipolar spindled cells with subpial aggregation of tumor cells and variable neuroparenchymal colonization. Of uncertain histogenesis, this is a stable/slowly growing tumor. Prognosis following total surgical resection is favorable. We describe an AG in a 16-year-old, intellectually disabled, male patient, with psychosis. This is a rare presentation with only one such case in literature. Patient's symptoms ameliorated following surgery.

Isolated Intraventricular Rosai-Dorfman Disease.

Rosai-Dorfman disease (RDD) is a benign histioproliferative disorder characterized by generalized lymphadenopathy, weakness, anemia, and rarely extranodal involvement. The disease affects the central nervous system (CNS) very rarely and at a relatively older age. Isolated intracranial involvement is very rare. RDD tends to present as dural masses mimicking meningioma, though very rare case reports describe isolated parenchymal involvement in the CNS. Only one case of intraventricular RDD has been described so far to the best of our knowledge. We present a very rare presentation of this disease as an isolated intraventricular presentation in the region of the atria of the lateral ventricles.