Plasmacytoid Dendritic Cell Leukemia: A Rapidly Evolving Disease Presenting with Skin Lesions Sensitive to Radiotherapy plus Hyperthermia

Plasmacytoid dendritic cell leukemia (pDCL) is a rapidly evolving disease, which frequently presents with skin lesions, particularly nodules and plaques with a typical reddish‐brown or brown color. Treatment of pDCL is based on multiagent chemotherapy followed by allogeneic hematopoietic stem cell transplant, but skin lesions may be refractory to therapy. Here, we report on a 61‐year‐old patient affected by pDCL who first presented with multiple cutaneous nodules and plaques on the trunk. Lesions showed an excellent response to radiotherapy plus hyperthermia. Although this treatment did not avoid the systemic evolution of disease, it resolved skin lesions and prevented their relapse, thus representing a therapeutic option to be used in combination with chemotherapy regimens. The case presentation is followed by a general discussion with an emphasis on the diagnosis and treatment of this rare malignancy.     

Intravascular lymphoma: a role for single-agent rituximab

Intravascular lymphoma is a rare aggressive systemic neoplasm with neurological and cutaneous presentation, which commonly eludes the diagnosis ante mortem. We document a case of intravascular lymphoma in a 65-year-old man who presented with altered mental status, fevers and weight loss. The diagnosis was made by random skin biopsies demonstrating an intravascular, CD20 positive cellular infiltrate. Initial treatment consisted of cyclophosphamide, vincristine, doxorubicin, prednisone and rituximab. Because of disease progression, the therapy was switched to weekly rituximab after the first cycle. A complete response was observed with resolution of symptoms and laboratory abnormalities. The patient remains in remission 3 years after completion of therapy. This is the first case report to describe a sustained remission following single-agent rituximab in the patient with neurologic involvement of intravascular lymphoma who failed antracycline-based therapy. Single-agent rituximab has activity in this disease and may be considered as a treatment option.     

Management of neurocytomas: case report and review of the literature

The purpose of this report is to review the available literature on the presentation, pathology, and treatment of central nervous system (CNS) neurocytomas. A case report of an extraventricular neurocytoma is presented along with a comprehensive literature search of patients with a diagnosis of CNS neurocytoma. CNS neurocytomas are rare neoplasms, with fewer than 240 cases reported in the literature. The majority of neurocytomas are found in the ventricular system of the brain. Immunohistochemistry is frequently used to help distinguish this tumor from other CNS neoplasms. MIB-1 proliferation index is commonly used in an attempt to predict biologic behavior. Little is known about the management of patients with this tumor, because most reports are from the pathologic literature and contain sparse information regarding clinical management. Neurocytomas are rare CNS tumors with varied biologic behavior. MIB-1 index may help direct adjuvant therapy. An excellent prognosis can be expected if a gross total resection is achieved. Postoperative radiation therapy (RT) may be considered after subtotal resection. Otherwise, RT is an option for medically inoperable or recurrent disease.     

Treatment of multicentric Castleman's disease complicated by the development of non-Hodgkin's lymphoma with high-dose chemotherapy and autologous peripheral stem-cell support

Background: Castleman's disease or angiofollicular lymph node hyperplasia is a rare entity with a localized/unicentric or a generalized/multicentric presentation. Whilie surgery is curable for most localized presentations, there is limited information regarding the optimal management of the multicentric type. The latter type is associated with a poor prognoses and can be associated with the development of lymphoma and infections.Patients and methods: In this report we describe a case of multicentric Castleman's disease who failed steroids and chemotherapy and developed a follicular mixed lymphoma. He was treated with high-dose chemotherapy with autologous stem-cell support and remains disease at four years of follow-up.Conclusions: A long-term durable remission may be possible with high dose chemotherapy with stem-cell support. This treatment modality should be considered an option in the management of multicentric Castleman's disease.     

Role of Radiotherapy in Post-transplant Lymphoproliferative Disorders: Three Case Reports and Review of the Literature

Post-transplant lymphoproliferative disorder (PTLD) is an aggressive malignancy that occurs in patients who have undergone solid organ transplantation or hematopoietic stem cell transplantation. It develops as the result of uncontrolled cell proliferations owing to reduced immunological surveillance. PTLD may occur with a various spectrum of clinical presentations, including both localized and extensive disease. Management can be significantly variable according both to the clinical presentation and to the histologic features. The most important systemic treatment strategies are reduction of immunosuppressive therapy, chemotherapy, anti B-cell antibodies, especially rituximab and cytokine-based therapies. The localized form of PTLD could be efficiently treated, and potentially cured, with surgery or radiotherapy (RT). Involved site RT may be a feasible effective option for the treatment of patients with PTLD, given the excellent radio-sensitivity of lymphoid disorders. In this report, we describe 3 adult patients with PTLD treated with moderate-dose RT (24-36 Gy) having a good local control with negligible toxicity. We also review the literature data on the role of radiation therapy in this particular setting.     

Endoprosthetic reconstruction for giant cell tumors of the distal tibia: A short term review

Custom‐made endoprosthetic reconstruction for distal tibia tumors is a viable option of treatment in carefully selected patients. It maintains satisfactory function and provides good pain relief. We report four cases of giant cell tumors of the distal tibia successfully treated by endoprosthetic reconstruction. This is a feasible option in cases of this nature and offers a better function than the other available options.     

Non-Hodgkin's lymphoma of prostate: a rare site of primary extranodal presentation (a report of two cases)

We report two cases of primary extranodal lymphoma of the prostate, an unusual site for extranodal presentation. The clinical presentation and treatment is discussed.     

Giant metastatic myxoid liposarcoma causing cardiac tamponade: a case report

We report a case of a rapidly progressing isolated giant metastatic myxoid liposarcoma to the heart in a 54-year-old man, who presented with acute symptoms of cardiac tamponade. Surgery remains the best treatment option for this rare condition.     

Complete atrioventricular block induced by rituximab in monotherapy in an aged patient with non-Hodgkin’s diffuse large B-cell lymphoma

Rituximab is a treatment option to non-Hodg kin's diffuse large B-cell lymphoma (NHDLBCL) in advanced stage and comorbility. It is known the cardiotoxicity effect of this drug, but there is no previous report describing a complete atrioventricular block (CAVB) secundary to treatment with Rituximab. We present an elderly woman treated with monotherapy with Rituximab who experienced a CAVB after administration of the fifth dose of this drug.     

Percutaneous cystic duct stent placement in the treatment of acute cholecystitis

Percutaneous cholecystostomy is well established as a temporising treatment option in selected patients presenting with acute cholecystitis. However, some patients who undergo cholecystostomy will have persistent discharge, which precludes catheter removal, or may not be medically suitable for future cholecystectomy. In these circumstances, percutaneous cystic duct stenting isa novel treatment option. It may delay or avoid the need for cholecystectomy, and thereby provide definitive treatment in a subset of patients who have acute cholecystitis and a high anaesthetic risk or limited life expectancy. Current application has been limited largely to patients with pre-existing malignant common bile duct strictures, but there is potential for the application to be broadened to include other subsets of patients. In this paper, we describe the technique used for percutaneous cystic duct stenting in a patient and report on its effectiveness. We also explore the technical considerations and consider the application of the procedure on other groups of patients.     

Parathyroid carcinoma secondary to prolonged hyperplasia in chronic renal failure and in coeliac disease.

The presentation of parathyroid carcinoma in patients with chronic renal failure is rare, although with improvements in life expectancy associated with this condition there have now been 12 reported cases, including the first case we report here. It has been proposed that in these cases there has been a malignant transformation of benign parathyroid hyperplastic tissue. We also report the first case of parathyroid carcinoma associated with coeliac disease and suggest that the same mechanism may be responsible. We review the presentation, diagnosis, treatment and natural history of the disease.     

Blunt renal vascular trauma resulting in arterial avulsion injury with a nephron‐sparing outcome

Blunt renal vascular traumatic injury is uncommon, and most injuries can be managed conservatively in a patient who is clinically stable. Pseudoaneurysm or active bleeding at presentation is rare and in an unstable patient, endovascular techniques offer a low morbidity option for rapid treatment. We present an unusual case of avulsion of a second order renal artery from the main renal artery, with active bleeding at presentation treated by excluding the bleed with a stent graft. This was complicated by delayed pseudoaneurysm formation, treated with a larger stent graft. This resulted in preservation of renal parenchyma and renal function in a young patient.     

Efficacy of the tyrosine kinase inhibitor gefitinib in a patient with metastatic small cell lung cancer

Gefitinib (Iressa, ZD1839) is an orally active inhibitor selective for the epidermal growth factor receptor tyrosine kinase and has shown promise in the treatment of non-small cell lung cancer (NSCLC). There has been no report to date of the effect of gefitinib treatment in patients with small-cell lung cancer (SCLC). Here we report a case of metastatic SCLC that was successfully treated with gefitinib. This case is the first reported of objective clinical response after gefitinib treatment in a patient with SCLC. Our report suggests that treatment with gefitinib is a novel option for a subset of patients with SCLC.     

Therapeutic approach guided by genetic alteration: Use of MTOR inhibitor in renal medullary carcinoma with loss of PTEN expression

Renal Medullary Cancer (RMC) is a rare and aggressive type of renal cell cancer that presents predominantly in patients with sickle cell hemoglobinopathies, and is typically metastatic at the time of presentation. Although platinum based chemotherapeutic regimens have recently emerged as the best option for producing a clinically significant response as reported in various case series, the response is far from satisfactory, as most RMC patients still succumb to their disease within a year of diagnosis. There is currently no standard of care for treatment of this disease. We report, to our knowledge, the first case of RMC where in molecular characterization of the tumor was used to guide therapy. In our patient, molecular analysis identified a decreased expression of Ribonucleotide Reductase M1(RRM1) and phosphatase and tensin homolog (PTEN). Based on these results of PTEN deficiency, we started our patient on everolimus (an MTOR inhibitor) maintenance after treating him with an induction chemotherapy regimen of Paclitaxel-Cisplatin-Gemcitabine (PCG). His tumor responded to induction therapy and he went into complete remission and remained in remission for 7 months. He is now alive about 14 months from his diagnosis and is asymptomatic with minimal disease. The rarity of RMC makes it very difficult to do any meaningful clinical trials in this group of patients. The overall prognosis for RMC remains very poor and knowledge about driver mutations may help in guiding therapy to improve survival in this select group of patients, where there is dearth of available therapies.     

Long-term efficacy of oral alendronate therapy in an elderly patient with polyostotic fibrous dysplasia: A case report

Polyostotic fibrous dysplasia (PFD) is a high- turnover bone disease that frequently entails chronic bone pain, pathological fractures and severe deformities. Recently, bisphosphonates have shown effective antiresorptive properties in the treatment of children or adults with PFD. We report on a 79-year-old female with PFD, who had severe lower limb deformity and chronic bone pain in multiple sites of her extremities for more than 55 years. The patient experienced significant decrease in bone pain and bone turnover markers following long-term (8.5 years) treatment with a low-dose oral alendronate treatment (5 mg/day). To the best of our knowledge, this is the first report of a long-term follow-up of a postmenopausal elderly patient with long-standing symptomatic PFD following continuous low-dose oral alendronate therapy. This case report indicates that long-term daily administration of low-dose alendronate alone is a potential treatment option for elderly patients with PFD, particularly those with long-standing bone pain.     

Treatment sequencing of metastatic colorectal cancer based on primary tumor location

Colorectal cancer is a heterogeneous disease with various clinical, molecular, and embryological differences related to the origin of the tumor from the right or left colon. Recent studies have demonstrated that tumor sidedness has both a prognostic and predictive value in metastatic colorectal cancer . Patients whose primary tumor originates from the left side of the colon and whose tumor's genome encodes wild-type RAS and BRAF should be offered cetuximab or panitumumab in the first-line treatment of metastatic disease or in subsequent lines. For tumors originating from the right side of the colon, anti-angiogenic treatment, particularly bevacizumab, is an option for this poor prognostic group until better options become available. Specifically, an aggressive initial approach with FOLFOXIRI plus bevacizumab is a treatment option in right-sided tumors under investigation. This report reviews the available data for the treatment of metastatic colorectal cancer according to the location of the primary tumor and proposes the optimal treatment sequencing strategy incorporating the site of origin of the tumor and molecular information into the decision-making process.     

Diffuse cystic angiomatosis of the breast

Vascular tumors of the breast are extremely rare, and the majority are malignant. In this report, the presentation of a diffuse, benign, cystic, vascular tumor that histologically was angiomatosis of the breast is described. The clinical presentation, magnetic resonance imaging (MRI) appearance, and histologic features of this rare lesion are reviewed. This case is of particular interest because the tumor recurred during pregnancy and underwent massive enlargement despite negative hormone receptor values. Angiomatosis is prone to local recurrence, so complete excision with histologically negative margins is the treatment of choice.     

Evaluation and definitive management of medically inoperable early stage non-small-cell lung cancer. Part 2: newer treatment modalities

Lung cancer is estimated to be the second most commonly diagnosed cancer in both men and women in 2006, and the leading causes of cancer mortality. Non-small-cell lung cancer represents the majority of such cases. Most of these patients have locally advanced disease at presentation and are not eligible for curative resection. For the minority of patients who are technically resectable at presentation, lobectomy or pneumonectomy and pathologic mediastinal nodal staging offer the best overall survival. The high rate of comorbid medical illness and poor baseline pulmonary function in this population, however, make many such early-stage patients medically inoperable. For these patients, conventional single-modality radiotherapy has been the primary definitive treatment option, as discussed in part 1 of this article, which appeared in last month's issue. Numerous retrospective reports demonstrate long-term disease-free and overall survival data that are modestly superior to that expected after observation, but both local and distant failure continue to be significant risks. Investigation of radiotherapy dose escalation is ongoing, in an effort to improve local control while maintaining minimal toxicity. Additionally, emerging evidence suggests that new modalities, such as stereotactic radiosurgery and radiofrequency ablation, may also be potentially curative treatment alternatives. These modalities are addressed in part 2.     

Evaluation and definitive management of medically inoperable early-stage non-small-cell lung cancer. Part 1: Assessment and conventional radiotherapy

Lung cancer is estimated to be the second most commonly diagnosed cancer in both men and women in 2006, and the leading cause of cancer mortality. Non-small-cell lung cancer represents the majority of such cases. Most of these patients have locally advanced disease at presentation and are not eligible for curative resection. For the minority of patients who are technically resectable at presentation, lobectomy or pneumonectomy and pathologic mediastinal nodal staging offer the best overall survival. The high rate of comorbid medical illness and poor baseline pulmonary function in this population, however, make many such early-stage patients medically inoperable. For these patients, conventional single-modality radiotherapy has been the primary definitive treatment option, as discussed in part 1 of this two-part article. Numerous retrospective reports demonstrate long-term disease-free and overall survival data that are modestly superior to that expected after observation, but both local and distant failure continue to be significant risks. Investigation of radiotherapy dose escalation is ongoing, in an effort to improve local control while maintaining minimal toxicity. Additionally, emerging evidence suggests that new modalities, such as stereotactic radiosurgery and radiofrequency ablation, may also be potentially curative treatment alternatives. These modalities will be addressed in part 2.     

Chemotherapy-induced Complete Regression of Choroidal Metastases and Subsequent Isolated Leptomeningeal Carcinomatosis in Advanced Breast Cancer: A Case Report and Literature Review

Choroidal metastases from breast cancer represent an unusual metastatic presentation that has been traditionally treated with radiation therapy.Herein, we report a case of metastatic breast cancer presenting with pulmonary, cutaneous, lymph node and symptomatic choroidal metastases treated with systemic combination chemotherapy incorporating docetaxel and mitoxantrone without induction or consolidation radiation therapy to control visual symptoms from choroidal metastases. The patient experienced a durable complete remission in all metastatic sites that was maintained for 21 months since the initiation of chemotherapy, afterwhich she developed isolated leptomeningeal carcinomatosis managed successfully with intensive intrathecal methotrexate and whole brain irradiation leading to a new complete remission maintained until this report; 11 months after its presentation. This is the first case to our knowledge reporting complete regression of choroidal metastases with docetaxel-based chemotherapy as the only treatment modality and subsequent isolated leptomeningeal carcinomatosis recurrence.