Giuseppe Isimbaldi

Malignant epidermoid cyst of the pineal region with lumbar metastasis

This report describes a malignant neoplasia of the pineal region in a 65‐year‐old woman who presented with symptoms of intracranial hypertension and dissemination to CSF with radiologically documented lumbar intradural localizations. The histological examination of the lesion showed fragments of a cystic malignant neoplasia with epithelial elements resembling squamous cell carcinoma. We excluded the possibility of a cerebral metastasis from a squamous cell carcinoma arising in the genital or gastrointestinal tract, lung and skin. We propose this unique report for cyto‐ histological correlation and, as suggested in the American and Japanese literature, we discuss the hypothesis of the development of a squamous cell carcinoma arising from the malignant degeneration of a cerebral epidermoid cyst and we show by cytological examination of the CSF the exceptional presence of malignant cells and by lumbar MRI their intradural localizations.

A CASE OF MALIGNANT PHYLLODES TUMOR WITH MUSCULAR AND FATTY DIFFERENTIATIONS

A 50-year-old female underwent surgical removal of a mammary phyllodes tumor, whose peculiar histologic feature was the coexistence of areas of liposarcoma and leiomyosarcoma. The morphologic differential diagnosis is briefly discussed.

A case of carcinoid of Meckel's diverticulum associated with gastric adenocarcinoma.

Meckels diverticulum is an uncommon gastrointestinal congenital anomaly that occurs in 1-3% of the population. It is sometimes associated with complications related to the presence of ectopic tissue (obstruction, ulceration, hemmorhage, inflammation, perforation, fistula and tumors). Neoplastic degeneration of Meckels diverticulum mucosa is rare, developing in only 1-5% of all diverticula, usually asymptomatic and occasionally discovered. Disease is metastatic, usually to the liver, in 25% of cases. We report a case of asymptomatic unsuspected carcinoid of Meckels diverticulum with ileal, hepatic and mesenteric metastasis discovered during a gastrectomy performed for gastric adenocarcinoma. The patient underwent ileal and Meckel diverticulum resection, excision of mesenterial metastasis and liver bisegmentectomy. Furthermore, total gastrectomy with esophago-jejunal anastomosis was performed. After an 18-month follow-up period, the patient is alive and disease free. Owing to possible neoplastic degeneration, Meckels diverticulum should be resected when occasionally discovered. In the presence of a carcinoid tumor, even if associated with metastatic disease, extended resection is recommended.

Lymph node hyperplasia: clonal chromosomal and genomic rearrangements. Report of two new cases and literature review

Cytogenetic analysis is not routinely performed on lymph node hyperplasia (LH). We describe clonal chromosomal rearrangements in two unrelated cases of LH. Lymph nodes of both patients showed typical morphologic features of benign follicular hyperplasia. Cytogenetic analysis revealed clonal chromosomal rearrangements in both cases. Patient 1 showed interstitial 14q and 6q mosaic deletions, whereas patient 2 showed a terminal 14q mosaic deletion. Fluorescence in situ hybridization with IGH break-apart probes identified a partial deletion of IGH in both cases, but the loss of the LSI IGH in patient 2 and loss of the LSI IGHV in patient 1 were observed on the morphologically normal chromosome 14. In the latter case, the finding of two morphologically normal chromosomes 14 with the IGHV deletion in one of the chromosomes suggested that the first mutational event was the IGH deletion and the second event was the interstitial deletion of chromosome 14 with the IGH intact. Array comparative genomic hybridization performed on both biopsies confirmed the IGH deletion at mosaic, but not the chromosomal deletion. Patient 1 was re-biopsied after 9 months and a marginal zone lymphoma was diagnosed. The finding of clonal cytogenetic abnormalities in LH highlighted the difficulties in interpretation of results and clinical follow-up.

Ganglioglioma of the spinal cord in neurofibromatosis type 1

The oncologic involvement of the spinal cord in neurofibromatosis type 1 (NF1) is not a typical feature of the disease. Here, we present a case of ganglioglioma of the spinal cord in a child with NF1 and try to define if this tumor can be considered coincidental or not. A 4-year-old boy affected by NF1 was diagnosed with a spinal cord-enhancing tumor extending from C4 to D3, with a disappearance in the T2 MRI sequences of the cerebrospinal fluid signal. The patient underwent a subtotal resection. The pathological exam revealed a ganglioglioma. To the best of our knowledge, only 1 other case of spinal cord ganglioglioma has been described in an NF1 patient. We suggest considering ganglioglioma in the differential diagnosis of an NF1 patient with a spinal cord tumor due to its favorable survival rate, especially in relation to the anatomical and surgical issues of this tumor that do not always entail a gross total resection.

The plasmablasts in Castleman Disease

To the Editor In their article, Hsi et al1 described the broad spectrum of histopathologic features of Castleman disease (CD). We would like to report one additional peculiar case, rich in human herpesvirus-8 (HHV-8)-infected plasmablasts (PBs), which we recently observed in our institution. The normal PB is a short-lived, very mobile B cell that can secrete antibodies but retains characteristics of an activated and proliferating cell.2 The PB could be considered the precursor of the more mature noncycling plasma cell (PC). Both naive and memory B cells can differentiate into PBs when activated with or without T-cell help, with or without antigen.3 The maturation of PBs in PCs is characterized by the cessation of proliferation and loss of mobility. B-lymphocyte-induced maturation protein 1 (Blimp-1)/PR domain zinc finger protein (PRdm-1) is essential for PC maturation, repressing the expression of genes that m aintain B-cell identity (Pax5) and others that promote B-cell proliferation, such as Bcl6 and MYC.3 The PB is characterized by the following phenotype: CD19+, CD27++, CD38−/+, CD20−/+, cIgM+.4 The PBs are rare in normal lymph nodes; they probably live around the mantle zone of the germinal centers, and then they exit into peripheral blood and may survive for a short period only unless they are recruited into mucosa or bone marrow niches by chemokine receptor expression.5,6 In pathology, PBs characterize only rare human disorders, most notably the multicentric, HHV-8-related variant of CD. HHV-8, which uses host cell division as a means of propagation, targets the rapidly proliferating, antibody-secreting PBs.7 …

Occult monoclonal B-cell disorder of hyoid bone

To the Editor The detection of minimal occult B-cell lymphoproliferative disorders is constantly increasing in the current pathological practice, and these entities need to be distinguished from clear-cut lymphoma by careful clinical correlation [1,2]. The following case presents challenging diagnostic issues in this sense. Because of computed tomography (CT) imaging suggesting a neoplasm (Figure 1A), a 64-year-old man underwent a total laryngectomy, and a histological diagnosis of squamous cell carcinoma was rendered. In the hyoid bone haematopoietic tissue, some focal nonparatrabecular and centrolacunar lymphoid aggregates without interstitial infiltration [2] were incidentally found. The aggregates were com posed by monotonous, small B-lymphocytes (Figure 1B, C), involving about 10–15% of the total cellularity. The immunophenotype of the aggregates was CD20 (highly positive), BCL2, CD5 , CD23 (intensely expressed, Figure 2), CD10 , BCL6 and cyclin D1 . The sample was submitted to PCR-based DNA amplification in order to analyze somatic rearrangements of the immunoglobulin heavy chain gene variable (IGHV) region, and two clonal rearrangements were observed utilizing IGHV3 and IGHV4 gene, respectively [3] (Figure 1D). A FISH analysis investigating the t(14:18) follicular lymphoma related translocation was performed, but it was unsatisfactory for technical reasons. A final diagnosis of incidental atypical lymphoproliferative disorder was rendered. This finding led the patient to sustain further clinical staging procedures, but physical examination and total body CT did not reveal any systemic localization. Moreover, blood cell count (4.59 10/l; lymphocytes 1.44 10/l; 31.4%), flow cytometry (CD3 T-lymphocytes 73%; 60%CD4/40%CD8; CD3 /CD16 /CD56 NK cells 22%; CD19 B lymphocytes: 5% without clonality) and serum biochemistry, including LDH levels, were normal. A successive posterior left iliac crest bone marrow biopsy and aspirate did not show any other localization or sign of lymphoma. During the following months, all blood cell counts performed (one per month) were normal, and the patient did not develop any signs of lymphoproliferative disease. Moreover, 7months after laringectomy, a fineneedle aspiration biopsy revealed a metastatic lymph node of the neck. A complete laterocervical nodal dissection was performed without any evidence of lymphoma at histology. This case raised the question related to the correct clinicalpathological management of this unusual entity. The differential diagnosis includes bone marrow localization of peripheral lymphoma versus primary bone marrow lymphoma (PBML) [1] versus a ‘CD5’ Monoclonal B-cell