Clinical experience with biweekly CHOP plus rituximab chemoimmunotherapy for the treatment of aggressive B-cell non-Hodgkin lymphoma.

BACKGROUND: The results of CHOP-21 (cyclophosphamide, doxorubicin, vincristine and prednisone given every 21 days) for the treatment of aggressive B-cell lymphoma have recently been improved by the addition of rituximab and by increasing the dose density. R-CHOP-14 combines these two approaches. PATIENTS AND METHODS: We present our experience with R-CHOP-14 in a retrospective single-centre review of 50 patients consecutively treated for aggressive B-cell lymphoma. RESULTS: The median age was 59 years and 48% of patients were >60 years. Stage III-IV was present in 62% of the patients and international prognostic index was high-to-intermediate risk or high risk in 32% of the patients. Toxicity was mainly haematological, with grade 3-4 neutropenia observed in 32% and febrile neutropenia in 18%. Other relevant toxicities were peripheral neuropathy in 45% (grade 3 in 4%) and cardiac dysfunction grade 3 in 7.5%. After therapy, 82% of the patients achieved complete response or unproved complete response. With a median follow-up of 30 months, 3-year event-free survival and overall survival were 67% and 82% respectively. CONCLUSIONS: In our experience the combination of RCHOP- 14 is highly effective in patients with aggressive B-cell lymphoma. However special attention must be paid to the control of early and late toxicities.

Hypoglycemic coma secondary to big insulin-like growth factor II secretion by a giant phyllodes tumor of the breast.

Nonislet cell tumor hypoglycemia (NICTH) is uncommon, and has been related to the production of insulin-like growth factor II or its high-molecular-weight precursor (big IGF-II) by the tumor. Cases of NICTH have been related to different tumors, mainly sarcomas. However, only three cases secondary to a phyllodes tumor of the breast (PTB) have been previously reported. We present the clinical course of a woman with a giant PTB, who developed an irreversible hypoglycemic coma secondary to big IGF-II production by the tumor. A brief review of the literature and possible mechanisms of this infrequent condition are also commented.

Primary cutaneous marginal zone b-cell lymphoma (pcmzl-malt)

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Primary Hodgkin's lymphoma of the caecum.

Extra-nodal Hodgkin's lymphoma (HL) represents 15% of all Hodgkin's lymphomas; the primary intestinal site accounts for 1% and with involvement of the ascending colon being rare. We present the case of a patient of 62 years of age diagnosed as having acute appendicitis. Anatomopathology on the excised appendectomy tissue indicated nodular lymphocytic predominant Hodgkin's lymphoma (NLPHL). The morphology indicated isolated L&H (lymphocytic or histiocytic) cells or in groups, surrounded by T lymphocytes, in an environment of germinal centres together with phenomena that would be interpreted as progressive transformation. Immunohistochemistry staining of the HL cells were positive for CD45, CD20, Bc16, EMA and MUM1 and negative for CD15 and CD30. No complementary treatment was administered. Following a literature search, the present case would appear to be the first of its kind.

Angiosarcoma of the abdominal wall after dermolipectomy in a morbidly obese man. A rare form of presentation of Stewart-Treves syndrome.

Angiosarcoma is a rare malignant tumor, with a predilection for skin in the head and neck region, although it has been described in many other locations. Its association with chronic lymphedema is well known, mainly in the setting of postmastectomy lymphedema of the arm in breast cancer patients (termed Stewart-Treves syndrome). However, angiosarcoma can appear in lower limbs with chronic lymphedema and rarely in other locations such as the abdominal wall. Herein, we present a unique case of angiosarcoma developing in the abdominal wall of a morbidly obese patient after extensive dermolipectomy.

Epirubicin, cyclophosphamide and weekly paclitaxel as neoadjuvant chemotherapy for stage II and III breast cancer.

PURPOSE: To assess the efficacy and the toxicity of a new combination of epirubicin, cyclophosphamide and paclitaxel as neoadjuvant chemotherapy for breast cancer. METHODS: Patients with stage II and III breast cancer received 3-4 cycles of epirubicin 75 mg/m(2) plus cyclophosphamide 600 mg/m(2) on day 1, and paclitaxel at a dose of 100 mg on days 1, 8, 15 and 22 on a 28-day cycle. RESULTS: Forty-nine patients were enrolled in the study. Stage II was present in 16 patients (32.7%) and stage III in 33 patients (67.3%). Relevant toxicities were nausea/vomiting grades III-IV in 6 patients (12.2%) and neutropenia grade III-IV in 33 patients (67.3%). The overall clinical response rate was 83.7%. Partial response was observed in 25 patients (51%), complete response in 16 patients (32.7%), stable disease in 7 patients (14.3%) and progression in 1 patient. Thirty-three non-inflammatory breast cancer patients underwent surgery, 29 with breast-conserving surgery (87.9%). Pathological complete response was found in 5 patients (15.1%). CONCLUSIONS: The combination of epirubicin, cyclophosphamide and weekly paclitaxel as given in this study is safe and shows good activity in the neoadjuvant setting of stage II and III breast cancer patients.

Lumbar region intra-spinal primitive neuroectodermal tumour (PNET) combined with neurofibromatosis type 1.

Primitive neuroectodermal tumours (PNET) are aggressive neoplasias that are diagnosed, usually, in infancy. Their appearance in adulthood is rare and, exceptionally, in association with neurofibromatosis type I (NF-1). We present a case of a 37 year-old man with NF-1 combined with PNET in the intra-arachidial lumbar region. Diagnosis was by Nuclear Magnetic Resonance (NMR) and biopsy of soft tissue mass which showed a PNET with undifferentiated round cells and immunohistochemically positive for CD99, neurone-specific enolase, synaptophys in and LEU-7. Surgery was performed with spine decompression and resection of 80% of the tumour, with symptoms improvement. Radiotherapy was administered on the lumbosacral column, but only up to 30 Gy because of severe actinic enteritis and pan-cytopenia grade III. Six months later, the patient was hospitalized with deterioration in his overall clinical status with multi-organ involvement. The patient died and an autopsy was performed. The initial treatment of the PNET is surgery and, if possible, the radical extirpation of the tumour. Administration of radiotherapy and chemotherapy appears to increase survival. We comment on the clinical, histological, cytological and immunohistochemical aspects together with a review of the literature. To the best of our knowledge this is the first documentation of such a case.

Lumbar region intra-spinal primitive neuroectodermal tumour (PNET) combined with neurofibromatosis type 1

Primitive neuroectodermal tumours (PNET) are aggressive neoplasias that are diagnosed, usually, in infancy. Their appearance in adulthood is rare and, exceptionally, in association with neurofibromatosis type I (NF-1). We present a case of a 37 year-old man with NF-1 combined with PNET in the intra-arachidial lumbar region. Diagnosis was by Nuclear Magnetic Resonance (NMR) and biopsy of soft tissue mass which showed a PNET with undifferentiated round cells and immunohistochemically positive for CD99, neurone-specific enolase, synaptophysin and LEU-7. Surgery was performed with spine decompression and resection of 80% of the tumour, with symptoms improvement. Radiotherapy was administered on the lumbosacral column, but only up to 30 Gy because of severe actinic enteritis and pan-cytopenia grade III. Six months later, the patient was hospitalized with deterioration in his overall clinical status with multi-organ involvement. The patient died and an autopsy was performed. The initial treatment of the PNET is surgery and, if possible, the radical extirpation of the tumour. Administration of radiotherapy and chemotherapy appears to increase survival. We comment on the clinical, histological, cytological and immunohistochemical aspects together with a review of the literature. To the best of our knowledge this is the first documentation of such a case