Blastoid mantle cell lymphoma occurring in a patient in complete remission of chronic myelogenous leukemia.

The development of a de novo lymphoma in patients affected by chronic myelogenous leukemia (CML) is a rare event. The introduction of new molecular cytogenetic techniques, such as fluorescence in situ hybridization (FISH), allows a correct differential diagnosis between lymphoid blastic crisis and a blastoid variant of mantle cell lymphoma (MCL), which shows an aggressive behavior and some molecular characteristics detectable by cytogenetics and immunohistochemistry. We report a case of a blastoid variant of MCL that developed in a patient with CML who achieved complete cytogenetic and molecular response to imatinib mesylate treatment.

Bilateral upper and lower lid fatty herniation: an unusual presentation of non-Hodgkin's lymphoma.

We report the case of a patient presenting with eyelid hernias who required bilateral upper and lower blepharoplasty; histological examination of the excised fat revealed B-cell non-Hodgkin's lymphoma. At diagnosis, the disease was already systemically advanced, but the patient was asymptomatic. No sign of disease had been detected in the preoperative tests. The bilateral orbital presentation of a systemic lymphoma is very rare, and is usually accompanied and revealed by exophthalmos, increased tear secretion, diplopia and decreased visual acuity. To our knowledge, this is the first case in which lid hernias were the first and only clinical sign of such a systemic disease.

Adenocarcinoma of the urachus: radical or conservative surgery? A report of a case and a review of the literature.

AIMS: To identify the most appropriate surgical strategy for carcinoma of the urachus. METHODS: Analysis of a case of adenocarcinoma of the urachus and an examination of the current literature were carried out. CONCLUSIONS: Partial cystectomy is considered the most appropriate surgical strategy but the need for close follow-up is underlined.

Surgical treatment of clival chordomas: the transsphenoidal approach revisited.

This is a report of 12 cases of clival chordomas that were surgically treated at the Catholic University Medical School, Rome, Italy, over a 7-year period. The study emphasizes the role of the transsphenoidal approach. The study group included seven men and five women whose ages ranged from 26 to 80 years (mean 49.8 years). Diplopia was the most common presenting symptom (eight cases). The tumor involved the upper and middle clivus in five cases, the middle clivus in five, and the lower clivus in two cases. One patient developed spinal metastasis. On histological examination, eight cases proved to be typical chordomas, three cases had a chondroid component, and one case of chordoma had atypical features. Immunohistological staining for vimentin and epithelial membrane antigen was positive in all cases. Follow-up periods ranged from 14 to 86 months (mean 40.2 months). The primary treatment consisted of surgery. Ten patients with chordomas of the upper and middle clivus underwent a total of 13 transsphenoidal procedures. Total tumor removal was achieved in seven cases, subtotal removal in two, and partial removal in one case. In the two cases of lower clival chordomas, total removal was accomplished in one and partial removal in the other. After total removal, no recurrence was noted at 14 to 86 months (mean 37.5 months). In the cases undergoing operation via a transsphenoidal approach, there was zero morbidity and one cerebrospinal fluid fistula that resolved without surgery. The tumor recurred in two patients after subtotal and partial removal, respectively. The authors opted to reoperate in cases of recurrence. Postoperative radiotherapy was administered in only two cases in which further surgery was not indicated because of medical reasons or because such a procedure was contrary to the patient's wishes. When mortality and morbidity rates of this group are compared to those of chordoma patients who were treated with extensive skull-base surgery, the results prompt a reappraisal of the transsphenoidal approach in the treatment of clival chordomas.

Rhinocerebral zygomycosis in acute lymphoblastic leukaemia.

We describe a patient with acute lymphoblastic leukaemia who developed rhinocerebral zygomycosis during the aplastic phase induced by antineoplastic chemotherapy. The patient was treated with fluconazole intravenously (400 mg daily) for 30 days and underwent surgical debridement. As a result of this treatment a complete remission of the zygomycosis-associated symptoms was observed. The possibility of treating zygomycosis with fluconazole is discussed.

Granuloblastic masses in the breast: report of two cases.

Two patients with granuloblastic masses of the breast, onsetting at diagnosis and as first relapse site of acute myeloid leukemia are reported. The first case had been diagnosed initially as a carcinoma, and the patient underwent bilateral mastectomy. Acute leukemia was only revealed by histology of the surgical specimen. In the second case a second neoplasia, occurring during acute leukemia, was suspected: a fine needle aspirate of the breast mass showed a leukemic infiltrate. Involvement of breast during acute myeloid leukemia is a rare event and, in our experience, of bad prognostic significance. Mammary glands may be a sanctuary for leukemic cells, contributing to unresponsiveness to chemotherapeutic agents.

Lymph node blast crisis in chronic myeloid leukemia mimicking T-immunoblastic lymphoma.

BACKGROUND: Chronic myeloid leukemia arises from a somatic mutation in a pluripotent stem cell. It generally terminates with a blastic crisis (BC). One third of BC are lymphoid, and most have a pre-B phenotype. Few cases of T-lymphoid BC have been reported. Here we describe a lymph node blast crisis mimicking T-immunoblastic lymphoma. METHODS: Bone marrow and lymph nodes were histologically examined by standard methods and by an immunoperoxidase technique. Cytogenetic studies were also performed on lymph node and blood cells. Analysis of T-cell receptor genes and BCR rearrangements were performed on DNA extracted from both frozen bone marrow and lymph-node cells. RESULTS: Lymph-node histology showed an infiltration by large lymphoid blasts, consistent with a diagnosis of immunoblastic lymphoma. Blast cells were CD2, CD7, TDT positive, and negative for myeloid and mature lymphoid antigens. The Ph1 chromosome was found in both bone marrow and lymph-node cells. BCR rearrangement was found in the DNA from both bone marrow and lymph-node cells. TCR genes were not rearranged. DISCUSSION: The present study provides strong evidence that the lymph-node blast crisis of CML can assume the morphological appearance of immunoblastic lymphoma and may retain the immunological phenotype and genetic features of early T cells with BCR rearrangements.

Episodic angioedema with eosinophilia (Gleich syndrome).

A case of episodic angioedema with hypereosinophilia or otherwise called Gleich's syndrome is reported. The patient was a young woman with a six-year history of recurrent angioedema, itchy urticaria, rapid weight gain and hypereosinophilia. The main clinical, laboratory, and immunological findings included systemic angioedema, urticaria, 12% weight gain and leucocytosis (69.150 WBC/cu mm) with eosinophils of 75.6%. Circulating IgM, IgE, and T-helper lymphocyte were elevated. Skin biopsy showed an important perivascular eosinophil infiltration. The absence of concomitant allergic, malignant and connective disorders, and a dramatic clinical improvement with a fall of the eosinophil count to normal levels following corticosteroid therapy was the basis for our diagnosis.