Breast implant-associated anaplastic large cell lymphoma presenting as a breast mass: A case report and literature review.

INTRODUCTION: Breast implant-associated anaplastic large cell lymphoma (BIA-ALCL) is a rare neoplasm most frequently associated with textured implant placement. The classic presentation consists of a persistent periprosthetic seroma. Implant removal and individualized adjuvant treatment are potentially curative interventions. Most BIA-ALCL present with a seroma, not with a breast and/or axillary mass. Knowledge of this presentation and how to manage it allows an adequate diagnosis, and appropriate treatment with excellent results. PRESENTATION OF CASE: A 44-year-old woman presented with a 3-month history of a right breast mass located in the lower medial quadrant, with associated right axillary lymphadenopathy. Medical history was significant for a mastoplasty with textured implants 15 years before the onset of her symptoms. Imaging studies and histological analysis helped to confirm the diagnosis of BIA-ALCL. A bilateral capsulectomy was performed and adjuvant chemotherapy and immunotherapy were administered. With these interventions, the patient had complete resolution of her symptoms, good cosmetic results, and absence of tumor activity detectable by positron emission tomography with fluorodeoxyglucose (PET-CT FDG) at a 2.5-year follow-up. DISCUSSION: This case describes an atypical presentation of BIA-ALCL as a breast mass, as well as lymph node and bone marrow involvement. Knowledge of the different presentation modalities of this pathology is necessary for a correct diagnosis and treatment. Through a multidisciplinary approach, adequate treatment was given with excellent results. CONCLUSION: Anaplastic large cell lymphoma associated with breast implants is a clinicopathological entity still little known in some medical fields. A variety of presentations must be considered, and high clinical suspicion must be maintained in patients with a history of textured breast implant placement to optimize diagnosis and avoid delays in treatment.

Laparoscopic resection of gastric schwannoma: A case report.

INTRODUCTION: Gastric schwannomas are an extremely rare presentation of mesenchymal tumors originating from Schwann cells, accounting for 0.2% of all gastric tumors. Patients are usually asymptomatic, so these tumors are frequently detected incidentally. PRESENTATION OF CASE: 68-year old male patient found to have a 5 cm mass in the lesser curvature of the stomach. After a careful preoperative evaluation, complete laparoscopic resection was performed. Pathology review confirmed a completely resected gastric Schwannoma. The patient's recovery was uneventful. At a one-year follow-up he remains asymptomatic and with no evidence of disease. DISCUSSION: We present the uncommon case of a gastric schwannoma that was appropriately treated with a laparoscopic approach and present a current literature review focusing on diagnostic and treatment methods of these rare tumors. CONCLUSION: Schwannomas should be included in the differential diagnosis of gastric tumors and can be appropriately treated with a laparoscopic approach.

Pulmonary extraskeletal myxoid chondrosarcoma: A case report and literature review.

BACKGROUND: Extraskeletal myxoid chondrosarcoma (EMC) accounts for the 3% of all soft tissue sarcomas and it's categorized as a tumour of uncertain differentiation. This entity has shown to have the recurrent balanced chromosomal translocation t(9;22) (q22;q12.2), which leads to the oncogenic fusion gene EWSR1-NR4A3. This sarcoma usually presents as a slow growing, palpable mass in the extremities. EMC arising from the lung is extremely infrequent. We report one case of pulmonary extraskeletal mixoid chondrosarcoma and a review of the world literature. CASE REPORT: A 69-year-old male patient presented with intermittent hemoptysis for the last 6 months. A PET/CT scan showed a hypermetabolic solid mass with lobulated borders of approximately 29×26mm in the inferior right lobe. We performed a right thoracotomy with inferior lobectomy and lymphadenectomy of levels VII, VIII, X, and XI levels. The neoplasm was constituted by cords of small cells with small round nucleus and scarce cytoplasm immerse in an abundant myxoid matrix. The immunophenotype was positive for MUM-1, CDK4, MDM2, and showed focal expression for S-100 protein and CD56. The final pathology report revealed a pulmonary extraskeletal mixoid chondrosarcoma. No further surgical interventions or adjuvant therapies were needed. CONCLUSION: EMC is an intermediate-grade neoplasm, characterized by a long clinical course with high potential for local recurrence and distant metastasis. Treatment for EMC is surgical and non-surgical treatment is reserved for recurrence or metastatic disease. Pulmonary extraskeletal myxoid chondrosarcoma is a rare neoplasm with only isolated case reports found in the literature.

Mammary analog secretory carcinoma of the parotid gland: A case report and literature review.

BACKGROUND: Mammary analog secretory carcinoma (MASC) was first described in 2010 by Skálová et al. This entity shares morphologic and immunohistochemical features with the secretory carcinoma (SC) of the breast. MASC usually presents as an asymptomatic mass in the parotid gland and predominantly affects men. This tumor is considered a low-grade carcinoma but has the potential for high-grade transformation. We report one MASC case and a review of world literature. CASE REPORT: A 66-year-old male patient presented because he noticed a mass of approximately 3×3cm on the right pre-auricular region. Physical examination demonstrated a 3×3.5cm, firm, fixed, non-tender mass in the right pre-auricular region. An MRI of the head and neck showed an ovoid heterogeneous lesion, dependent of the right parotid gland of 27×28mm. We performed a superficial parotidectomy with identification and preservation of the facial nerve. The immunophenotype was positive for epithelial membrane antigen (EMA), CK8/18, vimentin, S-100 protein, and mammoglobin. No further surgical interventions or adjuvant therapies were needed. The patient will have a close follow up. CONCLUSION: The presence of t(12;15) (p13;q25) translocation which results in the ETV6-NTRK3 gene fusion or positive immunochemical studies for STAT5, mammoglobin and S100 protein, are necessary to confirm the diagnosis of MASC. MASC treatment should mimic the management of other low-grade malignant salivary gland neoplasms. The inhibition of ETV6-NTRK3 gene fusion could be used as treatment in the future.

Significance of common hepatic artery lymph node metastases during pancreaticoduodenectomy for pancreatic head adenocarcinoma.

BACKGROUND: Common hepatic artery lymph nodes (CHALN) are frequently sampled during pancreaticoduodenectomy for adenocarcinomas of the head of the pancreas. In some institutions, if metastatic disease is detected intraoperatively in these lymph nodes, the tumor is considered unresectable and a curative operation is not performed. No solid data exist to support this practice. METHODS: A retrospective review of a prospectively collected database was conducted of the records of all patients who underwent a pancreaticoduodenectomy for pancreatic adenocarcinoma between September 1991 and April 2005. Clinical and pathologic factors were analyzed to determine their influence on survival. RESULTS: Fifty-five of 175 patients had CHALN separately identified and evaluated; these patients constituted the study population. Thirty-eight patients (69%) had one or more lymph nodes with metastatic involvement; 10 of these had disease in CHALN. The median overall survival for patients with node-negative, node-positive (but CHALN-negative), and CHALN-positive disease were 22.9, 16.1, and 14.7 months, respectively. The 5-year overall survival rates for the respective groups were 22%, 17%, and 0%. CONCLUSIONS: CHALN metastases correlate with poor prognosis and no long-term survival. Further studies examining CHALN status are indicated and could lead to modifications of pancreatic cancer staging and management.

Steroid receptors and their role in the biology and control of breast cancer growth.

Steroid hormone receptors (estrogen receptor [ER] and progesterone receptor [PR]) play a critical role in the development of breast cancer. Most importantly, the expression of ER and/or PR by tumor cells provides important information that is critical for the selection of treatment. Recent studies on ER and PR have provided new insights into the pathogenesis of breast cancer and the mechanisms of resistance to antihormonal therapy. Better understanding of steroid receptors, their ligands, and the mechanisms through which they exert their effects will allow the correct treatment to be targeted to responsive tumors.

Emerging principles for the development of resistance to antihormonal therapy: implications for the clinical utility of fulvestrant.

We seek to evaluate the clinical consequences of resistance to antihormonal therapy by studying analogous animal xenograft models. Two approaches were taken: (1) MCF-7 tumors were serially transplanted into selective estrogen receptor modulator (SERM)-treated immunocompromised mice to mimic 5 years of SERM treatment. The studies in vivo were designed to replicate the development of acquired resistance to SERMs over years of clinical exposure. (2) MCF-7 cells were cultured long-term under SERM-treated or estrogen withdrawn conditions (to mimic aromatase inhibitors), and then injected into mice to generate endocrine-resistant xenografts. These tumor models have allowed us to define Phase I and Phase II antihormonal resistance according to their responses to E(2) and fulvestrant. Phase I SERM-resistant tumors were growth stimulated in response to estradiol (E(2)), but paradoxically, Phase II SERM and estrogen withdrawn-resistant tumors were growth inhibited by E(2). Fulvestrant did not support growth of Phases I and II SERM-resistant tumors, but did allow growth of Phase II estrogen withdrawn-resistant tumors. Importantly, fulvestrant plus E(2) in Phase II antihormone-resistant tumors reversed the E(2)-induced inhibition and instead resulted in growth stimulation. These data have important clinical implications. Based on these and prior laboratory findings, we propose a clinical strategy for optimal third-line therapy: patients who have responded to and then failed at least two antihormonal treatments may respond favorably to short-term low-dose estrogen due to E(2)-induced apoptosis, followed by treatment with fulvestrant plus an aromatase inhibitor to maintain low tumor burden and avoid a negative interaction between physiologic E(2) and fulvestrant.

Estrogen receptors as therapeutic targets in breast cancer.

The estrogen receptor alpha (ERalpha) has proven to be the single most important target in breast cancer over the last 30 years. The use of the selective ER modulator (SERM) tamoxifen for the treatment and prevention of breast cancer has changed therapeutics. The SERM raloxifene, approved for the treatment of osteoporosis, lacks tamoxifen's increased risk for endometrial cancer and is being evaluated for the prevention of breast cancer. Other SERMs approved or under development for use against breast cancer or osteoporosis include toremifene, GW5638, GW7604 (the active metabolite of GW5638), idoxifene, lasofoxifene, arzoxifene, bazedoxifene, EM-800 and acolbifene (the active metabolite of EM-800). Aromatase inhibitors (AIs) have recently proven to be more efficacious than tamoxifen as first-line therapy, efficacious for second-line therapy (e.g. against tamoxifen-resistant disease), and useful for extended adjuvant therapy after tamoxifen. The AIs include the non-steroidal agents letrozole and anastrole, and the steroidal agent exemestane. The pure antiestrogen fulvestrant has proven to be just as effective as AIs. Other pure antiestrogens, ZK-703, ZK-253, RU 58668 and TAS-108 show great promise. The development of resistance to endocrine therapy remains a clinically important problem, and laboratory models based on human breast cancer cells grown as tumors in immune-compromised mice have led to important insights into this problem. Progesterone receptor-negative status of ER-positive breast cancers may reflect altered growth factor receptor signaling, and helps to explain why this subclass of tumors exhibits lower response rates to tamoxifen compared to cancers typed progesterone receptor-positive. Crosstalk among plasma membrane-localized ER, growth factor receptor signaling, and nuclear-localized ER provide further insights into antihormonal-resistant breast cancer.

Hepatic resection for noncolorectal, nonneuroendocrine metastases.

Resection of certain hepatic metastases of noncolorectal, nonneuroendocrine (NCNNE) origin provides actual long-term (>5 years) survival. We conducted a retrospective outcome study at a single tertiary referral institution. Between January 1988 and October 1998, 64 consecutive patients underwent resection of hepatic metastases from NCNNE primary tumors. Overall and disease-free survival rates were correlated to clinicopathologic factors and operative morbidity and mortality. Thirteen patients underwent a right hepatectomy, 6 underwent a left hepatectomy, 3 had extended right and 2 extended left hepatectomy, 2 patients had segmentectomy, 24 underwent wedge resections, and 14 underwent a combination of these forms of resection. R0 resection was achieved in 56 patients (87.5%). The operative mortality was 1.5% (1 of 64). Actual 1-, 3-, and 5-year survivals were 81%, 43%, and 30%, respectively. The factor adversely associated with overall and disease-free survival was uniformly related to the interval between primary tumor resection and the development of hepatic metastases. A 1.5% operative mortality and an actual 5-year survival of 30% justifies hepatic resection, including major hepatic resection, for certain NCNNE metastases. The factor affecting prognosis in this highly select group of patients was the biological behavior of the tumor, with tumors that metastasize earlier having poorer survival rates.

Type IV thoracoabdominal aortic aneurysm with lymphoplasmacytic aortitis and cystic medial degeneration in a 32-year-old patient with Marfan syndrome.

Aortitis identified in approximately 12% of all thoracoabdominal aneurysms. The most common subtype of inflammatory aortitis is giant cell aortitis, followed by lymphoplasmacytic aortitis. Inflammatory aortitis may occur in isolation or as part of a systemic inflammatory disorder such as Takayasu arteritis, systemic lupus erythematosus, rheumatoid arthritis, and giant cell arteritis. Aortitis has not been described in patients with Marfan syndrome. We report the case of a 32-year-old man with Marfan syndrome and a strong family history of aneurysmal disease who presented with an asymptomatic Crawford type IV thoracoabdominal aneurysm. His aneurysm had no associated dissection, and surgical pathology revealed severe medial degeneration and lymphoplasmacytic aortitis. To our knowledge, this is the first report of such a finding in a patient with Marfan syndrome.

Unsatisfactory weight loss after vertical banded gastroplasty: is conversion to Roux-en-Y gastric bypass successful?

BACKGROUND: In 1991, the National Institutes of Health sanctioned 2 operations for treatment of morbid obesity: vertical banded gastroplasty (VBG) and Roux-en-Y gastric bypass (RYGB). Long-term results with VBG are disappointing. We wondered whether patients who had "adapted" to the VBG anatomy and had regained weight would lose weight after conversion to RYGB. METHODS: We reviewed data on patients undergoing conversion of VBG to RGYB. RESULTS: Fifty-four patients (mean body mass index [BMI] of 46 kg/m2 [range, 36-66]) underwent standard (48 patients) or distal (malabsorptive) (6 patients) RYGB. There were no perioperative deaths; postoperative morbidity delaying discharge occurred in 7 patients (13%). Follow-up (complete in 51 patients, x=6.1 years) was obtained by mail questionnaires and patient contact. Mean BMI decreased to 35 kg/m 2 (range, 22-47), and 59% of the patients with >1 year follow-up had a BMI <35 kg/m2 . The number of patients requiring positive pressure oxygen for sleep apnea decreased by half; most patients discontinued or decreased the number of medications treating weight-related comorbidities. At last follow-up, 90% of patients were satisfied subjectively with the results. CONCLUSIONS: Conversion of VBG to RYGB is safe and provides weight loss, improved quality of life, and reversal of weight related comorbidities.

Androgen-secreting adrenal tumors.

BACKGROUND: Adrenal tumors that secrete androgens exclusively are extraordinarily rare. The aim of this study was to characterize patients with pure androgen-secreting adrenal tumors. METHODS: A retrospective chart review from January 1946 through November 2002 identified 11 female patients with pure androgen-secreting adrenal tumors. RESULTS: The mean age was 23.4 years (range, 1-52). The most common presenting symptoms were hirsutism, acne, and clitoral enlargement. Elevated 17-ketosteroids were found in seven of nine tested patients. Computed tomogram, ultrasound, or both localized tumors in six of seven patients. All tumors were surgically resected, one laparoscopically, all without complications. Five of the 11 tumors were malignant. Mean weight and mean maximal diameter for benign and malignant tumors were 44 g and 4.2 cm and 232 g and 9.8 cm, respectively. Mean hospital stay was 8.5 days, with excess androgen production resolved in all patients. Recurrence and disease-related death occurred in only one patient who had pulmonary metastases at diagnosis. The remaining patients had no recurrence of tumor at mean follow-up of 11.7 years (range, 0.5-32 years). CONCLUSIONS: Pure androgen-producing tumors are extremely rare. Approximately 50% are benign, and surgical resection provides excellent treatment if the tumors are not metastatic at the time of diagnosis.

Open versus laparoscopic splenectomy for idiopathic thrombocytopenic purpura: clinical and economic analysis.

BACKGROUND: Since 1991, laparoscopic splenectomy (LS) has gained acceptance in the treatment of hematologic disorders, including idiopathic thrombocytopenic purpura (ITP). Several studies suggest that LS provides benefits over open splenectomy (OS). However, study design flaws hinder formal technology assessment. METHODS: We retrospectively reviewed medical and administrative records of patients who underwent splenectomy for ITP between January 1995 and December 2000 to compare clinical and economic outcomes associated with LS and OS. RESULTS: Eighty-six patients were identified; 42 underwent an attempted LS and 44 had OS. Preoperative patient characteristics were similar between groups. Mean operative and anesthesia times for LS and OS were 167 and 201 minutes and 119 and 151 minutes, respectively (P <.001). Overall transfusion and postoperative complication rates were similar between groups. On average, LS patients required 1.2 fewer days of parenteral analgesia and were able to tolerate a general diet 1.7 days earlier. Mean postoperative stay was 2 days lower for LS patients and mean total direct costs did not differ by surgical method (US dollars 8134 vs US dollars 8200). CONCLUSIONS: This observational study shows that LS is safe and offers advantages over OS: less postoperative pain, earlier general diet tolerance, and shorter hospital stay. These benefits are obtained at no significant additional cost.

Dehidroepiandrosterona en el manejo del lupus eritematoso sistémico / Dehydroepiandrosterone in the management of systemic lupus erythematosus

Los glucocorticoides son base de la terapéutica del Lupus Eritematoso Sistémico (LES). En ocasiones el control de la enfermedad requiere dosis altas que resultan inconvenientes. Los pacientes con LES tienen defectos hormonales que resultan en bajos niveles séricos de andrógenos, principalmente dehidroepiandrosterona (DHEA). En estudios recientes la administración de DHEA permitió reducir la dosis de glucocorticoides necesaria para controlar la enfermedad. Objetivo. Corroborar el papel coadyuvante de la DHEA en el tratamiento de LES. Métodos. Diez mujeres con LES de leve a moderada actividad y múltiples manifestaciones clínicas recibieron 200 mg/día de DHEA por vía oral así como otros medicamentos clínicamente indicados. Las pacientes fueron evaluadas mensualmente a través de MEX-SLEDAI, un cuestionario, una escala visual analógica y estudios de laboratorio. Resultados. Ocho pacientes completaron el estudio. Después de 3 meses de tratamiento con DHEA mejoraron los índices de actividad de LES incluyendo la puntuación MEX-SLEDAI y la sensación de bienestar de los pacientes (78.4 por ciento y 24.7 por ciento respectivamente). La DHEA fue bien tolerada; los efectos adversos observados fueron menores y reversibles al suspender el fármaco.Conclusión. La DHEA parece ser un coadyuvante útil en el tratamiento de LES.