Phase II trial of capecitabine and cisplatin in advanced, persistent, or recurrent carcinoma of the cervix.

PURPOSE: Platinum-based drugs are the most active agents in cervical carcinoma. The aim of this study was to assess the activity and toxicity of the association of cisplatin and capecitabine as first-line treatment in patients with advanced, persistent, or recurrent carcinoma of the cervix. PATIENTS AND METHODS: Patients with histological proven primary carcinoma, presence of measurable tumors, age 18 years or older, performance status of 2 or less, and adequate bone marrow, renal, and hepatic functions were potentially eligible for this trial. Prior chemotherapy was allowed only in the context of radiosensitization. Treatment consisted of 50 mg/m of intravenous cisplatin on day 1 with 2500 mg/m oral capecitabine daily in 2 divided doses for 14 consecutive days in 21-day cycles. Responses were assessed using response evaluation criteria in solid tumors. RESULTS: Between November 2004 and October 2007, 22 women were entered into the trial. Median age was 51 years (range, 37-70 years). Seventeen patients had prior radiotherapy, and 13 received a radiation sensitizer, whereas 2 patients underwent surgery exclusively and 3 patients had no prior treatment. A median of 5 cycles was administered (range, 2-8 cycles). There were one septic death, one grade 4 neutropenia, and one grade 4 anemia. Grade 3 fatigue, gastrointestinal toxicity, renal toxicity, and hand-foot syndrome were seen in 31.8%, 22.7%, 9%, and 9% of the patients, respectively. There were 1 complete response and 6 partial responses for an overall response rate of 31.8%. Seven patients (31.8%) each had stable disease, and 8 patients showed progression. The median time to progression was 7.6 months, with a median overall survival of 20 months. CONCLUSION: These results seem to suggest that the capecitabine-cisplatin combination is a moderately tolerated and active regimen in advanced, persistent, or recurrent cervical carcinoma patients. Further evaluation of this drug combination may be warranted.

Metastatic collecting duct carcinoma of the kidney treated with sunitinib.

Collecting duct carcinoma (CDC) of the kidney is a rare and aggressive malignant tumor arising from the distal collecting tubules which has been shown to have a poor response to several kinds of systemic therapy. We present a case of metastatic CDC that responded favorably to a multiple tyrosine kinase inhibitor, sunitinib, achieving a partial response in both lung and skeletal metastases. To our knowledge, this is the first report showing therapeutic activity of sunitinib against CDC. Considering these findings, it would be worthwhile prospectively investigating the role of multiple tyrosine kinase inhibitors, particularly sunitinib, in the management of metastatic CDC.

Diagnostic challenge for ovarian malignant melanoma in premenopausal women: primary or metastatic?

BACKGROUND: In the ovary, metastatic malignant melanoma may be confused with primary malignant melanoma and presents a diagnosis challenge. Most cases are associated with disseminated diseases and poor prognosis. We present this case report of a metastatic ovarian malignant melanoma simulating primary ovarian cancer. CASE REPORT: A 45-year-old premenopausal woman was incidentally found to have an abdominal mass, 3 years after removal of a cutaneous melanoma lesion. Ultrasound and CT scan revealed left two solid masses, which were found to be an ovarian tumor at laparotomy. Left oophorectomy was performed. Histopathology and immunohistochemistry showed melanoma metastasis to the ovary. Nine months later, the patient developed epilepsy and confusion. Magnetic Resonance Imaging showed unique Wright frontal lobe lesion. She underwent stereotactic radio surgery and dacarbazine monotherapy. For months later, the patient is died from disseminate disease progression. CONCLUSION: Ovarian metastasis is an unusual presentation of cutaneous melanoma and the prognosis was dismal. As illustrated by this case report, a differential diagnosis of a metastatic malignant melanoma must be considered.

Symptomatic hypopituitarism revealing primary suprasellar lymphoma.

BACKGROUND: The most common cause of hypopituitarism is pituitary adenoma. However, in the case of suprasellar masses different etiologies are possible. We report an unusual case of primary suprasellar lymphoma presented with hypopituitarism. CASE PRESENTATION: A 26 year old woman presented with amenorrhea, galactorrhea and neurological disorders. Also, the laboratory work-up revealed partial hypopituitarism. The magnetic resonance imaging of the head showed a suprasellar mass. A presumptive diagnosis of granulomatous processes was made and the patient was given steroid therapy. Repeated brain MRI detected new lesions in the brain with regression of the suprasellar mass. Stereotactic biopsy of the paraventricular lesion revealed the diagnosis of B-cell lymphoma. CONCLUSION: This case presentation reports a rare cause of hypopituitarism. Primary suprasellar lymphoma is extremely rare and represented a real diagnostic challenge. Besides, suprasellar masses are varied in aetiology and can present diagnostic problems for a radiologist. Also, because of the increased incidence of PCNSL, lymphoma must be kept in mind in the differential diagnosis of lesions in the suprasellar region.

Granulosa cell tumor of the ovary and antecedent of adjuvant tamoxifen use for breast cancer.

BACKGROUND: Adult granulosa cell tumor associated with antecedent use of tamoxifen as adjuvant hormonotherapy for breast cancer is rare. The pathogenesis of this occurrence remains difficult to explain. The estrogenic effect of tamoxifen can be one such explanation. CASE PRESENTATION: A 47 year-old women was treated with surgery, chemotherapy, radiotherapy and tamoxifen for stage III estrogen receptor positive breast carcinoma. Ten months after stopping tamoxifen, we diagnosed a stage Ic granulosa cell tumor of the ovary. CONCLUSIONS: Use of tamoxifen has been found to be associated with gynecological tumors like endometrial carcinoma. Its association with granulosa cell tumor of the ovary is uncommon. Only two previous cases have been reported in literature.

Metastatic giant basal cell carcinoma: a case report.

Basal cell carcinoma is the most common skin cancer, characterised by a slow growing behavior, metastasis are extremely rare, and it occurs in less than 0, 1% of all cases. Giant basal cell carcinoma is a rare form of basal cell carcinoma, more aggressive and defined as a tumor measuring more than 5 cm at its largest diameter. Only 1% of all basal cell carcinoma develops to a giant basal cell carcinoma, resulting of patient's negligence. Giant basal cell carcinoma is associated with higher potential of metastasis and even death, compared to ordinary basal cell carcinoma. We report a case of giant basal cell carcinoma metastaticin lung occurring in a 79 years old male patient, with a fatal evolution after one course of systemic chemotherapy. Giant basal cell carcinoma is a very rare entity, early detection of these tumors could prevent metastasis occurrence and improve the prognosis of this malignancy.

[Granulosa cell tumors of the ovary]. / Les tumeurs de la granulosa de l'ovaire.

Ovarian granulosa cell tumors (TGO) are rare neoplasms. They arise from sex cord stromal cells of the ovaries. They are characterized by their slow natural history, and their tendency to relapse long time after the initial diagnosis. Complete staging surgery of the disease is the cornerstone of treatment. Chemotherapy is indicated for localized tumors with a high risk of recurrence, and for recurrent or advanced tumors. Prolonged follow-up is recommended.

Malignant peripheral nerve sheath tumor of the small bowel: an unusual presentation with fatal outcome.

Malignant peripheral nerve sheath tumor of the small bowel is an extremely rare disease. Histologic distinction from other types of soft tissue sarcoma especially fibrosarcoma and leiomyosarcoma requires electron microscopy. Complete surgery remains the only curative treatment. However, late diagnosis makes curative surgery more difficult. The contribution of chemotherapy to incomplete surgery has been proved without controlled studies. We report a case of this type of lesion discovered following a small bowel perforation.

Primary immature teratoma of the uterus relapsing as malignant neuroepithelioma: case report and review of the literature.

Background. Although some mature cases of teratomas have recently been described in the cervix, they are not commonly found in the uterus, especially in immature forms. An immature uterine teratoma relapsing after surgery as malignant neuroepithelioma has never been reported in the literature. Case Presentation. We describe a case of immature teratoma which occurred primarily in the uterus in a 56-year-old female. Treatment consisted of total simple hysterectomy. Three months after surgery, the patient relapsed with voluminous pelvic mass and was treated with bleomycin, etoposide, and cisplatin-containing chemotherapy regimen. Conclusion. In this report and according to the pertinent literature, clinical and pathological features and management of uterine immature teratomas are discussed. The mainstay of treatment is surgery. The prognosis of this unusual disease remains uncertain.

Null phenotype non-Hodgkin lymphoma presenting as a large chest wall mass: case report.

Tumors of chest wall represent a variant entity. Most of them arise from metastasis of malignant tumors or from local invasion by contiguity. However, non-Hodgkin's lymphomas of the chest wall are extremely rare; only a few cases have been reported in the literature. We report a case about a Moroccan woman, with non-Hodgkin null phenotype lymphoma of the chest, treated successfully with CHOP (cyclophosphamide, Doxorubicin, Vincristin and prednisone) followed by local radiotherapy.

Metastatic Ewing's sarcoma/PNET of kidney in 40 year old patient.

INTRODUCTION: Primary renal Ewing sarcoma/PNET is an uncommon and very aggressive tumor. PRESENTATION OF CASE: : We report the case of a young woman who underwent nephrectomy for a renal mass from unknown etiology. Histologic analysis found small tumoral cells in rosette formation, and immunohistochemical staining was positive for CD99, and focally positive for vimentin and Protein S-100. A post operative abdominal computed tomography (CT) scan revealed a residual renal processus with hepatic wounds and abdominal metastatic nodes. After 6 cycles of chemotherapy including Vincristine, Doxorubicin, Cyclophosphamide, the response was considered as good. DISCUSSION: Ewing's sarcoma/PNET of kidney is a member of the family of small round cell tumors and it should be differentiated from Wilms tumor, neuroblastoma, rhabdomyosarcoma and lymphoblastic lymphoma. The principle management of its treatment have been extrapolated from the treatment of osseous Ewing sarcoma of bone. CONCLUSION: Despite aggressive treatment, primary renal Ewing sarcoma/PNET has a poor prognosis. It requires a multidisciplinary approach including oncologists, urologists and radiation oncologists.

Male breast cancer: a report of 127 cases at a Moroccan institution.

BACKGROUND: Male breast cancer (MBC) is a rare disease representing less than 1% of all malignancies in men and only 1% of all incident breast cancers. Our study details clinico-pathological features, treatments and prognostic factors in a large Moroccan cohort. FINDINGS: One hundred and twenty-seven patients were collected from 1985 to 2007 at the National Institute of Oncology in Rabat, Morocco.Median age was 62 years and median time for consultation 28 months. The main clinical complaint was a mass beneath the areola in 93, 5% of the cases. Most patients have an advanced disease. Ninety-one percent of tumors were ductal carcinomas.Management consisted especially of radical mastectomy; followed by adjuvant radiotherapy and hormonal therapy with or without chemotherapy. The median of follow-up was 30 months. The evolution has been characterized by local recurrence; in twenty two cases (17% of all patients). Metastasis occurred in 41 cases (32% of all patients). The site of metastasis was the bone in twenty cases; lung in twelve cases; liver in seven case; liver and skin in one case and pleura and skin in one case. CONCLUSION: Male breast cancer has many similarities to breast cancer in women, but there are distinct features that should be appreciated. Future research for better understanding of this disease at national or international level are needed to improve the management and prognosis of male patients.

[Psychosocial impact of cancer on Moroccan adolescent and young adult: experience of National Institute of Oncology of Rabat]. / Impact psychosocial du cancer sur les adolescents et les jeunes adultes marocains : expérience de l'Institut national d'oncologie de Rabat.

Cancer is an uncommon disease; its imaginary concept is very particularly on adolescent and young adults. It disturbs their lives on the whole. The purpose of this study is to describe the specific psychosocial effects of cancer on adolescent and young adults in Moroccan population in order to help physicians educate and counsel future young patients and their families. During the period from January to July 2009, patients aged between 15 and 30 years with histologically confirmed cancer, were prospectively interviewed by a questionnaire covering socio-epidemiological characteristics, repercussions of disease on physical, psychic, sexual and religious practices. The aim of this study is to determine the impact of cancer, particularly on this young North African population, which is underrepresented in the psychosocial cancer literature.

Rare recurrence of a rare ovarian stromal tumor with luteinized cells: a case report.

INTRODUCTION: Sex cord-stromal tumors of the ovary are uncommon. They behave unpredictably and often have a late recurrence, making counseling, management, and prediction of prognosis challenging. CASE PRESENTATION: A 52-year-old Moroccan woman with an sex cord-stromal tumors underwent a bilateral oophorectomy. The histology was unusual but was likely to be a luteinized thecoma with suspicious features for invasion. Seven years later, after a gastrointestinal bleed, a metastasis within the small bowel mucosa was detected. This represents probable isolated hematogenous or lymphatic spread, which is highly unusual, especially in the absence of concurrent peritoneal disease. CONCLUSIONS: To the best of our knowledge, this is the second reported case of an sex cord-stromal tumors recurring in small bowel mucosa and mimicking a primary colorectal tumor. This highlights the diverse nature and behavior of these tumors.

Systemic treatment and targeted therapy in patients with advanced hepatocellular carcinoma.

BACKGROUND: ADVANCED HEPATOCELLULAR CARCINOMA (HCC) IS A MALIGNANCY OF GLOBAL IMPORTANCE: it is the sixth most common cancer and the third most common cause of cancer-related mortality worldwide. Despite decades of efforts by many investigators, systemic chemotherapy or hormone therapy has failed to demonstrate improved survival in patients with HCC.. Ongoing studies are evaluating the efficacy and tolerability of combining Sorafenib with erlotinib and other targeted agents or chemotherapy. AIMS: On the basis of placebo-controlled, randomized phase III trials, Sorafenib has shown improved survival benefits in advanced HCC and has set a new standard for future clinical trials. The successful clinical development of Sorafenib in HCC has ushered in the era of molecularly targeted agents in this disease, which is discussed in this educational review. MATERIAL AND METHODS: Many molecularly targeted agents that inhibit angiogenesis, epidermal growth factor receptor, and mammalian target of rapamycin are at different stages of clinical development in advanced HCC. Future research should continue to unravel the mechanism of hepatocarcinogenesis and to identify key relevant molecular targets for therapeutic intervention. Identification and validation of potential surrogate and predictive biomarkers hold promise to individualize patients' treatment to maximize clinical benefit and minimize the toxicity and cost of targeted agents. RESULTS: Systemic therapy with various classes of agents, including hormone and cytotoxic agents, has provided no or marginal benefits. Improved understanding of the mechanism of hepatocarcinogenesis, coupled with the arrival of many newly developed molecularly targeted agents, has provided the unique opportunity to study some of these novel agents in advanced HCC. CONCLUSIONS: The demonstration of improved survival benefits by Sorafenib in advanced HCC has ushered in the era of molecular-targeted therapy in this disease, with many agents undergoing active clinical development.

Biotherapy in the Adjuvant Treatment of Colorectal Cancer.

The use of adjuvant chemotherapy has improved survival in early-stage colon cancer. Ongoing adjuvant clinical trials are evaluating the addition of targeted therapies to standard chemotherapy regimen. Preliminary results with bevacizumab were disappointing. Also, cetuximab added to chemotherapy does not seem to be better than chemotherapy alone, even in selected wild-type KRAS populations. A better understanding of mechanisms of action of drugs, tumor biology, and predictive biomarkers are needed to design future adjuvant trials.

Hepatocellular Carcinoma and High Grade Neuroendocrine Carcinoma: A Case Report and Review of the Literature.

We describe a rare hepatic collision tumor composed of a hepatocellular carcinoma and a high-grade neuroendocrine carcinoma. The patient, a 68-year-old man, underwent a partial hepatectomy because of a 4.0 cm mass. The tumor had two distinctive patterns. The majority of the tumor was a high-grade neuroendocrine carcinoma with features of a small cell carcinoma that was positive for chromogranin, synaptophysin, and cytokeratin 19 and negative for hepatocellular antigen and alpha-fetoprotein (AFP). The second component was a moderately differentiated hepatocellular carcinoma that was positive for hepatocellular antigen and AFP and negative for neuroendocrine markers. The two tumors were separated by fibrous bands. In areas where they collided, there was no transition or intermingling of cells between the two components, thus, it is different from the combined type of tumors. After removal of the tumor, the patient underwent four courses of chemotherappy which included etoposide and cisplatin with a follow-up period of 28 months.

Advanced treatments in non-clear renal cell carcinoma.

PURPOSE: To focus on the use of targeted therapies against the non-clear histologic subtypes of renal cell carcinoma (RCC); papillary I and II, chromophobe, and collecting duct. The unique genetic and molecular profiles of each distinct non-clear kidney cancer subtype will be described, as these differences are integral to the development and effectiveness of the novel agents used to treat them. MATERIALS AND METHODS: On the basis of MEDLINE database searches, we assessed all aspects of targeted therapy in non-clear cell RCC between 2000 and 2010. Trials focusing on non-clear RCC or those that treated clear cell tumors along with significant numbers of non-clear subtypes will be discussed. The role of cytoreductive nephrectomy and the use of neoadjuvant and adjuvant targeted therapy will be reviewed. Lastly, areas of future research will be highlighted. RESULTS: The majority of clinical trials testing novel targeted therapies have excluded non-clear subtypes, providing limited therapeutic options for patients with these diagnoses and their oncologists. CONCLUSION: Patients presenting with advanced non-clear pathology should undergo a thorough metastatic evaluation and, if appropriate, surgical evaluation to determine if nephrectomy, lymphadenectomy, and/or metastectomy are warranted. Aggressive surgical extirpation is often recommended. Sunitinib also is adequately tolerated and oncologically active in subjects with non-clear histology.

Successful management of primary non Hodgkins lymphoma of the cranial vault.

Primary bone lymphoma (PBL) is a relatively uncommon entity. However, involvement of the cranial vault is an unusual manifestation of aggressive non-Hodgkin's lymphoma. We report the case of a 42-year old immunocompetent woman who presented with an enlarging mass involving the right parietal bone. Magnetic resonance imaging (MRI) of the brain revealed an expansive tumor that affects the right parietal bone. Computed tomographic (CT) scans of the abdomen, chest and pelvis were negative for lymphadenopathy or organomegaly. Biopsy of the mass showed diffuse large B-cell non-Hodgkin's lymphoma confirmed by immunohistochemical study. The patient had a complete response after 4 cycles of chemotherapy followed by external beam radiotherapy. After a follow-up of more than 9 months the patient is still in good local control without distant metastasis. The aim of our work is to report a case of Primary bone lymphoma of the cranial vault with good response to treatment combining sequential chemotherapy and radiotherapy.

Metastatic ameloblastoma responding to combination chemotherapy: case report and review of the literature.

BACKGROUND: Ameloblastoma is a rare benign odontogenic tumor with locally aggressive behavior and a high recurrence rate. When metastases occur, which are uncommon, lungs constitute the most frequent site involved. Malignant ameloblastomas are different from ameloblastic carcinomas. Malignant ameloblastomas are tumors considered metastatic despite the appearance of well-differentiated or benign histology, while ameloblastic carcinomas are histologically malignant in both primary and metastatic sites. CASE PRESENTATION: A 24-year-old Moroccan man presented a malignant ameloblastoma of the mandible. The tumor was entirely resected. Five years later, a local recurrence occurred. Our patient was treated by exclusive radiotherapy with persistence of a residual disease. After two years he developed multiple lung metastases. Our patient received a combination chemotherapy using doxorubicin and cisplatin. CONCLUSION: Less than 50 cases of ameloblastoma with metastases have been reported. There is still no standard treatment for metastatic ameloblastoma. Only through continuous reporting of such cases will clinicians be able to draw an optimal strategy for management of this pathology.