Molecular Analysis of an Abdominal Wall Cesarean Section Endometrioid Carcinoma.

Malignant transformation of endometriosis is rare, and most cases concern the ovaries, while extraovarian cases are mostly found in the rectovaginal septum. Incisional adenocarcinoma is extremely rare, with only few cases reported in the literature, while their molecular profile remains unknown. Thus, we report on an abdominal wall cesarean section scar endometrioid adenocarcinoma studied by next-generation sequencing and microsatellite instability analysis.

Mesenteric desmoid tumor after Roux-en-Y gastric bypass: a case report

We report the case of a 37-year-old woman investigated for left flank pain 1 year after bariatric surgery (Roux-en-Y gastric bypass). Abdominal computed tomography (CT) revealed a solid intra-abdominal lesion measuring 9.3 × 9.4 × 10.4 cm, compressing adjacent structures with no signs of invasion. Ileocolectomy with partial mesenteric resection was performed. A histopathological and immunohistochemical analysis confirmed the diagnosis of mesenteric desmoid tumor.(AU)

Treatment of consistent BRAF/HRAS gene mutation and MYC amplification radiation-induced abdominal wall angiosarcoma with low-dose apatinib: a case report.

BACKGROUND: An extremely rare condition, radiation-induced angiosarcoma is characterized by a poor prognosis, high recurrence rate and lack of effective treatment. Herein, we present a case report of a 48-year-old female patient with radiation-induced abdominal wall angiosarcoma who showed a dramatic response to low-dose apatinib. CASE PRESENTATION: The patient, who was diagnosed with cervical squamous cell carcinoma 20 years ago, had received radiotherapy and chemotherapy after operation. Angiosarcomas of the abdominal wall appeared 9 years later. After repeated surgical operations and intravenous chemotherapy for the angiosarcomas, the patient developed tumor recurrence and pulmonary metastasis. The abdominal wall tumors showed repeated rupture and bleeding, with poor wound healing. On evaluation, laboratory findings detected the negative serum tumor markers CEA, CA 125, CA 15-3 and CA 19-9. Imaging showed multiple subcutaneous nodules and masses in the abdominal wall, accompanied by suspected small subpleural nodule at the lower lobe of the right lung. Immunohistochemistry of previous surgical pathology indicated that CD31, ERG and Vim were positive. The result of whole exome sequencing suggested the mutations of BRAF and HRAS, and the amplification of MYC. Based on the above results, the patient was clinically diagnosed with radiation-induced angiosarcoma of the abdominal wall with pulmonary metastasis. The patient was treated with low-dose apatinib and rejected reoperation or chemotherapy. RESULTS: At the 6-month follow-up visit, the abdominal wall lesions that had previously ruptured stopped bleeding and showed significant shrinkage. Imaging showed that most of the abdominal wall lesions had partially regressed, and some of the lesions on the abdominal wall and the suspected lesion of subpleural nodule at the lower lobe of the right lung had disappeared. CONCLUSIONS: We described this case and reviewed the literature on radiation-related angiosarcoma. Importantly, this case suggests that apatinib may be an effective and sensitive treatment for radiation-induced angiosarcoma even at the lowest dosage, without aggravating the bleeding of lesions.

Giant abdominal tumor - would you think adrenal?

Adrenal cavernomas are rare benign tumors, and those of giant dimensions are exceptional. Usually, they are symptomless or they induce symptoms and signs due to compression over nearby organs. We present the case of a 68-year-old woman, who complained of abdominal enlargement and abdominal pain in the left part of the abdomen. Imagistic investigations (native and contrast abdominal computed tomography) revealed an inhomogeneous retroperitoneal mass of 210∕182∕200 mm, containing calcifications. Laboratory findings were not relevant, just a slight and non-significant elevation of carcinoembryonic antigen and a slight elevation of C-reactive protein. Diagnosis of cancer of undetermined origin was considered, and surgery was performed. During surgery, a giant encapsulated inhomogeneous tumor of 330 mm, with cystic areas, was removed, without assessing the origin. Primary or secondary tumors (metastasis from breast, intestinal, lung, renal or skin cancer) were taken into account. Only histopathology and immunohistochemistry revealed the diagnosis of adrenal cavernoma. Until this moment, we found only one published article in the medical literature with similar dimensions of an adrenal cavernoma as in our case. Even if rare, hemangioma of the adrenal gland must be considered during the differential diagnosis of an adrenal tumor.

Mixed endometrioid and clear cell carcinoma arising from laparoscopic trocar site endometriosis.

Laparoscopic port site endometriosis is less common in abdominal wall endometriosis, and malignant transformation of abdominal wall endometriosis is rare. We reported a case of mixed endometrioid and clear cell carcinoma arising from port site endometriosis. The patient was a 49-year-old woman with a history of laparoscopic excision of ovarian endometrioma. Physical examination revealed a subcutaneous solid tumor around the laparoscopic surgical scar. Imaging showed a suspicious malignancy. She underwent radical marginal resection of the abdominal wall tumor, flap reconstruction of the abdominal wall, hysterectomy, bilateral salpingo-oophorectomy and omental biopsy. Histological examination revealed mixed endometrioid and clear cell carcinoma. Computed tomography scan showed no evidence of recurrence after six cycles of chemotherapy. This is the first case of malignant transformation from laparoscopic trocar site endometriosis.

[Surgical treatment of neurofibromatosis type I followed by retroperitoneal tumor (in Russian only)]. / Khirurgicheskoe lechenie neirofibromatoza I tipa, assotsiirovannogo s opukholevym porazheniem zabriushinnogo prostranstva.

AIM: To determine the optimal surgical approach in patients with abdominal and retroperitoneal manifestations of Recklinghausen's disease. MATERIAL AND METHODS: There were 4 patients (3 women and 1 man) with neurofibromatosis type I who were treated at Vishnevsky National Medical Research Center of Surgery. RESULTS: There were 3 robot-assisted procedures: excision of retroperitoneal tumors (plexiform neurofibroma and schwannoma) in 2 cases and right adrenal pheochromocytoma in 1 patient. We also included 1 clinical case of conventional surgery for neurofibromatosis type I followed by multiple gastrointestinal stromal duodenal and intestinal tumors. In one case, postoperative period was complicated by fluid accumulation in the bed of previously removed tumor that required US-assisted drainage. Postoperative period was uneventful in other cases. CONCLUSION: Robot-assisted surgery is safe and effective in patients with Recklinghausen's disease followed by single abdominal and retroperitoneal tumors. It is more expedient to choose conventional technique for multiple tumors located in different parts of retroperitoneal space or abdominal cavity.

Langerhans cell sarcoma arising from antecedent langerhans cell histiocytosis: A case report.

RATIONALE: Langerhans cell sarcoma (LCS) is a rare, high-grade neoplasm characterized by overtly malignant cytologic features and a poor prognosis. Herein, we present a rare case of langerhans cell histiocytosis (LCH) that later transformed into langerhans cell sarcoma 11 months after the benign mass was excised from soft tissue in the right groin. PATIENT CONCERNS: A 41-year-old patient who presented with a mass in the right groin for 3 years earlier after being bitten by ants. DIAGNOSES: The patient was diagnosed with langerhans cell sarcoma arising from antecedent langerhans cell histiocytosis. INTERVENTIONS: The patient underwent with 6 cycles of a modified etoposide, cyclophosphamide, vindesine, dexamethasone (E-CHOP) regimen. OUTCOMES: The patient is currently receiving follow-up care. LESSONS: LCH transformed into LCS is a rare case. E-CHOP as an effective first-line therapy to treat LCS cases, but, the mechanism is unclear. Due to their rarity, further data on clinical outcomes are necessary to establish the optimal treatment strategy for LCS.

Renal Transplant Failure Due to Infiltrating Kaposi Sarcoma.

Kaposi sarcoma is one of the most common malignancies seen during the posttransplant period, and it usually manifests in its cutaneous form. Renal transplant involvement is rare, whereas renal transplant parenchymal involvement causing transplant dysfunction is exceptionally rare. We report a case of visceral Kaposi sarcoma that led to renal transplant failure due to neoplastic infiltration of the renal allograft.

[Aggresive angiomyxoma after renal transplantation]. / Angiomixoma agresivo postrasplante renal.

The condition of immunosuppressed increases the risk of cancer in kidney transplant patients, as compared to the general population. The best survival of inmunosupressed patients in recent years has turned both neoplasms and cardiovascular diseases into the main causes of morbidity and mortality. We present the case of a renal transplanted patient who developed an unusual form of mesenchymal tumor such as the aggressive angiomyxoma, four years after the implant and requiring wide surgical resection.

Risk of tumor implantation in percutaneous endoscopic gastrostomy in the upper aerodigestive tumors.

Percutaneous endoscopic gastrostomy (PEG) has become a mainstay in providing enteral access for patients with obstructive head, neck and esophageal tumors. Tumor cell implantation is a rare complication in patients with aerodigestive cancers, who have undergone PEG tube placement. The objective of this review is to determine the incidence and contributing risk factors leading to the implantation of metastases into the abdominal wall following PEG placement. A comprehensive review of the literature in PUBMED (2008-2018) was performed.  The literature search revealed reports of more than 50 cases of abdominal wall metastases after PEG placement. As most of these studies were case reports, the exact rate of metastasis remains unknown. Generally pharyngoesophageal location of primary cancer (100%), squamous cell histology (98%), poorly differentiated tumor cells (92%), advanced pathological stage (97%), and large primary cancer size were identified as strong risk factors for the development of stomal metastasis. Abdominal wall metastases following PEG placement are a rare but serious complication in patients with head and neck malignancy.

Malignant transformation of abdominal wall endometriosis to clear cell carcinoma: case report

ABSTRACT BACKGROUND: Malignant transformation of endometriosis in the abdominal wall is a rare and still poorly understood event. Less than 30 cases have been reported in the worldwide literature. Most cases of solid tumors are report in a previous abdominal scar with malignant transformation of a focus of endometriosis. Presence of lymph node metastases in nearby chains is frequent and is associated with poor prognosis. CASE REPORT: We report a case of a 42-year-old woman with a history of abdominal surgery (Pfannenstiel) to resect abdominal wall endometriosis. Physical examination revealed a solid mass of approximately 10 cm x 6 cm in the anterior wall of the abdomen. Computed tomography (CT) of the abdomen and pelvis showed a heterogeneous, predominantly hypoattenuating expansive formation measuring 10.6 cm x 4.7 cm x 8.3 cm. The patient underwent exploratory incisional laparotomy, block resection of the abdominal mass and lymphadenectomy of the external and inguinal iliac chains. The abdominal wall was reconstructed using a semi-absorbable tissue-separating screen to reconstitute the defect caused by resection of the tumor. Histological evaluation revealed infiltration by malignant epithelioid neoplasia, thus confirming the immunohistochemical profile of adenocarcinoma with clear cell components. Lymphadenectomy showed metastatic involvement of an external iliac chain lymph node. CONCLUSION: Resection of the mass along with the abdominal wall, with wall margins, is the most effective treatment. Reconstruction is a challenge for surgeons. The patient has been followed up postoperatively for eight months, without any evidence of disease to date.

Angiomixoma agresivo postrasplante renal / Aggresive angiomyxoma after renal transplantation

La condición de inmunosuprimido aumenta el riesgo de cáncer en trasplantados renales, en comparación a la población general. La mejor supervivencia de esta población en los últimos años ha convertido a las neoplasias y a la enfermedad cardiovascular en las principales causas de morbi-mortalidad. Presentamos el caso de un paciente trasplantado renal que desarrolló cuatro años después del trasplante una forma inusual de tumor mesenquimatoso, el angiomixoma agresivo, que requirió resección quirúrgica amplia.

The condition of immunosuppressed increases the risk of cancer in kidney transplant patients, as compared to the general population. The best survival of inmunosupressed patients in recent years has turned both neoplasms and cardiovascular diseases into the main causes of morbidity and mortality. We present the case of a renal transplanted patient who developed an unusual form of mesenchymal tumor such as the aggressive angiomyxoma, four years after the implant and requiring wide surgical resection.

The changing clinical pattern of endemic Burkitt lymphoma in Western Africa: Experience from a tertiary center in Ghana.

BACKGROUND: Burkitt lymphoma (BL) is the most common childhood cancer in Ghana, where the endemic variant is the predominant subtype and historically presents as a highly chemo-sensitive jaw tumor. This study aimed to update the current epidemiological characteristics of childhood BL in our institution. PROCEDURE: Patient data for all children diagnosed with BL and seen at Korle Bu Teaching Hospital between January 2007 and December 2012 were retrospectively analyzed. RESULTS: BL was diagnosed in 173 children (<13 years) during the study period, with the abdomen as the most common tumor site (46%) followed by the jaw (31%). Abdominal tumors were associated with advanced/disseminated disease (P = 0.002), and were more likely to occur in females irrespective of tumor stage (relative risk = 1.56 [95% CI; 1.1-12.3]). Twenty-five percent (43/173) of the study cohort died and mortality was influenced by increasing age (P = 0.02) and advanced disease (P = 0.03). Treatment delay was experienced by nine in ten patients primarily due to familial financial constraint (75%). Treatment abandonment was observed as a first event in 94% of patients and two thirds of children in the study were eventually lost to follow-up. CONCLUSION: The predominance of primary abdominal tumors in our study cohort may indicate a changing epidemiological pattern of BL in Ghana. High rates of treatment delay and abandonment were evident and are likely to be contributing factors to the poor childhood cancer survival outcomes seen in resource-limited countries in Africa.

Not so mass effect? Finding of a remarkable 'incidentaloma' in a teenager with neurofibromatosis.

A 13-year-old boy with neurofibromatosis type 1 presented to the emergency department twice in a fortnight with moderate intermittent abdominal pain, radiating to the back and associated with nausea and vomiting. He examined as a well child with a soft abdomen and minimal tenderness. A history of constipation was identified but he failed to respond to a trial of laxatives. Subsequent ultrasound abdomen demonstrated a large mass surrounding the porta hepatis. MRI further characterised a focal, non-aggressive lesion extending from his liver, encapsulating his pancreas, portal vessels and laterally displacing his spleen and left kidney. Biopsy performed at a specialist cancer treatment hospital of our reference later confirmed this to be a benign neurofibroma of a size not previously reported in the literature. He will be managed conservatively with surveillance imaging and the potential for chemotherapy should the lesion continue to grow.

Urological sequelae of desmoids associated with familial adenomatous polyposis.

The aim of this retrospective cohort study was to review urological complication rates arising from familial adenomatous polyposis associated desmoid tumours and their management. All patients over a 35-year period were identified from a prospectively maintained polyposis registry database and had an intra-abdominal desmoid tumour. Those without ureteric complications (n = 118, group A) were compared to those that developed ureteric obstruction (n = 40, group B) for demographics, treatment interventions and survival outcomes. 158 (56% female) patients were identified. Median age at diagnosis was 31 years and desmoids typically occurred 3.6 years after colectomy for familial adenomatous polyposis. Ureteric obstruction secondary to tumour growth occurred in 25% of cases. There was no significant difference in gender distribution or overall age at desmoid diagnosis between the two groups. In group B, the median age at desmoid diagnosis was significantly younger in women compared to men (25 and 43 years, respectively) (p = 0.01). Thirty-eight percent of patients already had ureteric obstruction at desmoid diagnosis, the remainder occurred after 48.6 months, but 20 years in two cases. Seventy-three percent (29/40) had ureteric stenting, a long-term requirement for most. Permanent renal injury occurred in six cases but survival between the two groups was not significantly different. Ureteric obstruction occurs frequently in patients with familial adenomatous polyposis and an intra-abdominal desmoid tumour. Those most at risk are the young following colectomy. Clinicians should actively survey the renal tract at regular intervals after a diagnosis of an intra-abdominal desmoid tumour as complications can arise insidiously, at any stage.

Malignant transformation of abdominal wall endometriosis to clear cell carcinoma: case report.

BACKGROUND: Malignant transformation of endometriosis in the abdominal wall is a rare and still poorly understood event. Less than 30 cases have been reported in the worldwide literature. Most cases of solid tumors are report in a previous abdominal scar with malignant transformation of a focus of endometriosis. Presence of lymph node metastases in nearby chains is frequent and is associated with poor prognosis. CASE REPORT: We report a case of a 42-year-old woman with a history of abdominal surgery (Pfannenstiel) to resect abdominal wall endometriosis. Physical examination revealed a solid mass of approximately 10 cm x 6 cm in the anterior wall of the abdomen. Computed tomography (CT) of the abdomen and pelvis showed a heterogeneous, predominantly hypoattenuating expansive formation measuring 10.6 cm x 4.7 cm x 8.3 cm. The patient underwent exploratory incisional laparotomy, block resection of the abdominal mass and lymphadenectomy of the external and inguinal iliac chains. The abdominal wall was reconstructed using a semi-absorbable tissue-separating screen to reconstitute the defect caused by resection of the tumor. Histological evaluation revealed infiltration by malignant epithelioid neoplasia, thus confirming the immunohistochemical profile of adenocarcinoma with clear cell components. Lymphadenectomy showed metastatic involvement of an external iliac chain lymph node. CONCLUSION: Resection of the mass along with the abdominal wall, with wall margins, is the most effective treatment. Reconstruction is a challenge for surgeons. The patient has been followed up postoperatively for eight months, without any evidence of disease to date.

Seeding of Abdomen with Primary Intracranial Hemangiopericytoma by Ventriculoperitoneal Shunt: Case Report.

BACKGROUND: Ventriculoperitoneal shunt (VPS) placement has been implicated in extraneural metastasis of many primary central nervous system tumors. Reported cases include, but are not limited to, medulloblastoma, germ cell tumor, astrocytoma, oligodendroglioma, lymphoma, ependymoma, melanoma, and choroid plexus tumors. However, a literature review reveals no reported cases of extraneural metastasis of solitary fibrous tumor/hemangiopericytoma (SFT/HPC). CASE DESCRIPTION: Here we report the case of a 34-year-old man with recurrent intracranial malignant SFT/HPC who had undergone surgical tumor resection and subsequent placement of a VPS for obstructive hydrocephalus in 2004. Subsequently, the patient presented in 2011 and again in 2013 with abdominal SFT/HPC metastasis likely caused by the presence of the VPS. CONCLUSION: The case raises concern regarding placement of a VPS in patients with obstructive hydrocephalus caused by SFT/HPC. To avoid spread of SFT/HPC to the abdomen, we propose that patients with intracranial SFT/HPC and obstructive hydrocephalus be treated primarily by endoscopic third ventriculostomy.

Incidence of abdominal wall metastases following percutaneous endoscopic gastrostomy placement in patients with head and neck cancer.

INTRODUCTION: Percutaneous endoscopic gastrostomy (PEG) tubes are an effective modality for enteral nutrition in patients with head and neck cancer; however, there have been documented case reports of "seeding" of the abdominal wall by the theoretic risk of dragging the tube along the tumor during PEG placement. The objective of this study is to determine the incidence and contributing risk factors leading to metastasis to the abdominal wall following PEG placement in patients with head and neck cancer. METHODS: A retrospective chart review was performed on patients diagnosed with head and neck malignancy who underwent PEG placement between 1/5/2009 and 12/22/2014. Variables collected included development of abdominal wall metastases, type of malignancy and tumor characteristics, smoking history, PEG placement technique, and survival following recurrence. Data were then analyzed for overall trends. RESULTS: Out of 777 patients analyzed, a total of five patients with head and neck malignancy were identified with abdominal wall metastasis following PEG tube placement with an overall incidence of 0.64% over an average follow-up of 27.55 months. All of these patients underwent PEG tube insertion via a Pull technique. One patient was found to have a clinically evident and symptomatic stomal metastasis, while the other four patients had radiologically detected metastases either on CT or PET scan. All of the identified patients were found to have stage IV oral cancer at time of initial diagnosis of their head and neck malignancy, followed by widespread distant metastatic disease at time of presentation with their PEG site stomal metastasis. CONCLUSION: Abdominal wall metastases following PEG placement are a rare but serious complication in patients with head and neck malignancy.

[18F-FDG PET/CT contributes to diagnostics and therapy monitoring of radiation-induced angiosarcoma].

Angiosarcomas are rare, aggressive malignant mesenchymal tumours with a poor prognosis. Radiation therapy is an independent risk factor for the development of secondary angiosarcoma. The onset of angiosarcoma may resemble benign lesions, leading to delayed diagnosis. It has been suggested that 18F-fluorodeoxyglucose (FDG) PET/CT scan may be useful in the early diagnosis in differentiating angiosarcoma from benign lesions and in therapy monitoring. We report the utility of 18F-FDG PET/CT scan in the diagnosis and follow-up of radiation-induced angiosarcoma in a patient previously treated for uterine cancer.