Hepatic angio-sarcoma: An unusual source of intra-hepatic bleeding

INTRODUCTION

Hepatic angio-sarcoma represents an uncommon malignant tumor of the liver with a poor prognosis and a high rate of bleeding complications.

PRESENTATION OF CASE

We report a case of hepatic angio-sarcoma with a multi-nodular pattern complicated by intra-hepatic bleeding. The diagnosis was performed by computed tomography (CT). Angiographic procedure was unsuccessfully attempted as a treatment option. Autoptic examination confirmed the vascular nature of the malignant tumor.

DISCUSSION

Hepatic angio-sarcoma represents the most common malignant mesenchymal tumor of the liver. The diagnosis is provided by the histological examination and by specific endothelial markers. However, CT examination allows to recognize the disease and to detect intra-abdominal bleeding occurring in one-fourth of cases. Surgical resection represents the only definitive treatment of hepatic angio-sarcoma. In case of haemoperitoneum, trans-catheter arterial embolization represents the primary procedure used to stop the acute arterial bleeding.

CONCLUSION

CT represents the reference technique for the diagnosis of hepatic angio-sarcoma and allows to recognize the intra-abdominal bleeding which represents its most common complication. This condition always requires an immediate therapeutic approach.     

Impact of infection by verotoxigenic Escherichia coli O157:H7 on the use of surgical services in a children’s hospital

Objectives

To determine the impact of Escherichia coli O157:H7 infection in children on the need for surgical assessment in a pediatric surgical practice and whether clinical and bacteriologic variables might contribute to that need.

Design

Examination of a case series.

Setting

A tertiary-care pediatric hospital.

Patients

Between 1990 and 1994, E. coli O157:H7 gastrointestinal infections were documented among 85 children, 29 of whom suffered from hemolytic-uremic syndrome.

Intervention

Surgical consultation for presumed or proven complications of the infection.

Main Outcome Measures

The frequency of and reasons for surgical consultation, clinical and bacteriologic variables between patients who did or did not require surgical assessment.

Results

Of the 85 children, 17 (20%) were assessed by the surgical service. The majority of these children were inpatients. Two required abdominal surgery. Female gender, older age and progression to hemolytic-uremic syndrome were factors associated in univariate analyses with a likelihood of need for surgical assessment; variation in bacterial genotype was not.

Conclusion

There is the potential for verotoxigenic E. coli O157:H7 infection to have a considerable impact on the utilization of pediatric surgical services.     

The tail of neuroendocrine tumors from lung to pancreas: Two rare case reports

INTRODUCTION

Primary pancreatic neuroendocrine tumors are a well-established disease entity, however, neuroendocrine metastases to the pancreas from other sites have been scarcely documented. Specifically, pancreatic metastases from a pulmonary carcinoid tumor have only previously been described in a single case report.

PRESENTATION OF CASE

We sought to outline our institutional experience of two patients with pulmonary neuroendocrine tumors that developed metastases to the pancreas, confirmed by gross pathology and immunohistochemistry. In both cases, the pancreatic metastases were surgically resected and their pulmonary origin were discovered post-operatively.

DISCUSSION

Our findings should raise awareness to the possibility of metastatic disease when evaluating a pancreatic mass in a patient with a clinical history of pulmonary carcinoid tumor. Expert opinion on immunohistochemically differentiating a primary pancreatic neuroendocrine malignancy from a metastasis should be employed in these cases.

CONCLUSION

Establishing this diagnosis pre-operatively could affect the decision to proceed with surgical resection, given the morbidity of pancreatectomy and the unknown long-term clinical outcome of patients with pulmonary carcinoid tumors metastatic to the pancreas.     

Hepatobiliary rhabdomyosarcoma mimicking choledochal cyst: Lessons learned

INTRODUCTION

The differential diagnosis of hepatic cystic lesions is a challenging process especially in case of hepatic rhabdomyosarcoma (HRMS) presenting as hepatic cyst.

PRESENTATION OF CASE

We introduce our experience with a case of HRMS in a 3-year-old female patient who was misdiagnosed to have type IV-A choledochal cyst and definitive correct diagnosis was reached after the pathological and immunohistochemical examination of the surgically resected lesion. This case presentation is followed by important practical messages to hepatobiliary surgeons regarding HRMS.

DISCUSSION

HRMS is a rare pediatric tumor. Jaundice is the most common presentation of HRMS followed by abdominal pain and vomiting. Great effort is needed to differentiate the tumor from choledochal cyst and infectious hepatitis. Through evaluation using available imaging studies together with clinical anticipation is mandatory for establishing the correct diagnosis.

CONCLUSION

Differentiation of HRMs from choledochal cyst mandates through evaluation and clinical anticipation. HRMS should be suspected in any child with obstructive jaundice. Once diagnosis is established, multidisciplinary treatment is the best management strategy and it has proved better surgical outcome and long term survival.     

AIR SCORE ASSESSMENT FOR ACUTE APPENDICITIS

Background:

Acute appendicitis is the most common cause of acute abdomen. Approximately 7% of the population will be affected by this condition during full life. The development of AIR score may contribute to diagnosis associating easy clinical criteria and two simple laboratory tests.

Aim:

To evaluate the score AIR (Appendicitis Inflammatory Response score) as a tool for the diagnosis and prediction of severity of acute appendicitis.

Method:

Were evaluated all patients undergoing surgical appendectomy. From 273 patients, 126 were excluded due to exclusion criteria. All patients were submitted o AIR score.

Results:

The value of the C-reactive protein and the percentage of leukocytes segmented blood count showed a direct relationship with the phase of acute appendicitis.

Conclusion:

As for the laboratory criteria, serum C-reactive protein and assessment of the percentage of the polymorphonuclear leukocytes count were important to diagnosis and disease stratification.     

Kienbock��s disease in a varsity football player: a case report and review of the literature

Objective:

To present the diagnostic, clinical features, and management of Kienbock’s disease and create awareness of the differential diagnosis of this condition in patients presenting with insidious, progressive dorsal wrist pain.

Clinical Features:

A 23-year old male varsity football player presented with insidious progressive dorsal sided wrist pain with reduced wrist flexion and extension. A diagnosis of Kienbock’s disease was made based on radiographs and magnetic resonance imaging.

Intervention and Outcome:

A 3mm ulnar-minus variance was found and a joint leveling procedure to shorten the radius was performed. Conservative therapy was provided pre and post surgical management.

Summary:

This case report demonstrates the importance of findings on radiographs, MRI, and clinical examination in the accurate diagnosis and management of a patient with wrist pain.     

Nerve sheath myxoma of the dorsal paravertebral space

INTRODUCTION

Nerve sheath myxomas (NSM) are rare benign soft tissue tumors. The dorsal paravertebral placed NMS diagnosis can be difficult.

PRESENTATION OF CASE

This article presents clinical, radiological findings and treatment of the NSM of the dorsal paravertebral space in a 32-year-old man presented with a right shoulder and back pain for 4 years.

DISCUSSION

NSM is a rare and benign tumor and that most often occurs in the skin of the head, neck or upper limbs of younger patients. Rare locations such as intracranial, spinal canal, trunk, lower limb and oral cavity were also reported. The appropriate treatment of NSM is surgical excision. Diagnosis is difficult in an uncommon presentation.

CONCLUSION

Although the most presented case of NMS are dermal tumors, it may also be found extremely rare locations. We conclude that, the definitive treatment of NSM is surgical excision with safe margins even when it is possible.     

Benign skull lesions

Objective

To document the epidemiologic and clinical features of benign skull lesions.

Design

A case series.

Setting

St. Michael’s Hospital, a tertiary care facility affiliated with the University of Toronto.

Patients

Thirty-one patients who had a neurosurgical consultation and were discharged from hospital after excision of a benign skull lesion during a 10-year period.

Main outcome measures

Patient demographics, clinical signs and symptoms, radiographic and pathological tumour characteristics, surgical procedure, length of hospital stay, outcome and follow-up.

Results

The 31 patients (6 men, 25 women) had 32 lesions excised. The mean age of the patients was 41.9 years. Osteomas accounted for 63% of the tumours. The most frequent location was the parietal bone. Neurologic symptoms were absent in the majority of calvarial tumours. Useful diagnostic studies included plain skull radiography and computed tomography. Nuclear bone scanning was done in 7 patients. All patients underwent craniectomy, with cranioplasty in most cases. Three patients had new neurologic symptoms postoperatively and 1 patient had incomplete resolution of symptoms.

Conclusions

Benign skull lesions are infrequent, but they require neurosurgical intervention. When necessary, surgical excision can serve to confirm the diagnosis, improve cosmesis and retard the progression of neurologic dysfunction. Of primary importance is the recognition of such lesions by primary care physicians and referral to the surgeon so that an appropriate treatment plan can be made.     

Pilomatricoma: Experience of The Hospital for Sick Children

BACKGROUND:

Pilomatricoma (calcifying epithelioma of Malherbe) is a common skin neoplasm in the pediatric population that is often misdiagnosed as other skin conditions or tumours.

OBJECTIVES:

The objective of the present retrospective study was to review the clinical and histopathological presentation of this neoplasm in children.

METHODS:

The records of the pathology department at The Hospital for Sick Children, Toronto, Ontario, were searched for all cases of pilomatricoma between 2001 and 2006. The records of these patients were reviewed to determine sex, age, location and size of the tumour, pathological features and recurrence rate. All patients underwent surgical excision of the lesions.

RESULTS:

A total of 93 lesions in 85 patients were identified. The median age was 8.7 years. Of the 85 patients diagnosed with pilomatricoma, 44 (52%) were female. In all cases, the initial presentation was an asymptomatic, slow growing, superficial hard mass with bluish discolouration. The most common sites of occurrence were the face (48%), neck (21%) and upper limbs (18%). The size of the surgical specimens collected ranged from 0.1 cm to 2.6 cm. The diagnosis was confirmed by histopathological examination in all cases. Ghost cells and basaloid cells were described in most of the cases (83%). There were no recurrences in this series.

CONCLUSIONS:

This entity should be considered with other benign or malignant conditions in the clinical differential diagnosis of solitary firm skin nodules, especially those on the face, neck and upper limbs. The diagnosis can generally be made by clinical examination. The treatment of choice is surgical excision, and the recurrence rate is very low.     

Inflammatory myofibroblastic tumor of the small-bowel mesentery: A case report of nonspecific clinical presentation and a review of the literature

INTRODUCTION

Inflammatory myofibroblastic tumor (IMT) is a rare neoplasm of various anatomical sites, which is histopathologically characterized by spindle-shaped cells with myofibroblastic proliferation and inflammatory infiltration.

PRESENTATION OF CASE

In this case report, a 37-year-old man presented with nonspecific systemic symptoms, including abdominal pain and weakness, which was diagnosed by multislice computed tomography and ultrasonography. An 8 cm × 5 cm × 5 cm nodular gray-white firm noninfiltrative mass, which was well localized in the mesentery tissues of small bowel, was observed and the patient underwent surgical resection.

DISCUSSION

A review of the literature on IMT of the small-bowel mesentery yielded a small number of previously described cases. This tumor most frequently involves the lungs and arises most commonly in extrapulmonary locations such as the mesentery and omentum. The etiopathogenesis and the clinical course of the disease are unclear. The histological and clinical differential diagnosis of IMT also includes reactive processes and mesenchymal tumors of the gastrointestinal tract. Follow-up after surgical removal documented local recurrence and metastasis.

CONCLUSION

The preferred primary treatment is complete surgical excision, and patients require close clinicoradiological follow-up. In general, cases treated with complete surgical resection have a good prognosis.     

Laparoscopic Resection of a Small Bowel Lipoma with Incidental Intussusception

Background:

Small bowel tumors are rare entities that often present with nonspecific symptoms. The diagnosis is more likely in patients with occult gastrointestinal bleeding of unknown origin or in adults with small bowel intussusception. Even with exhaustive diagnostic testing, small bowel tumors are often not diagnosed preoperatively. Because 60% to 70% of small bowel tumors are malignant, surgical excision is always recommended.

Methods:

We report the case of a 73-year-old man with occult gastrointestinal bleeding. A small bowel tumor was discovered only after video capsule endoscopy, computed tomography, and multiple endoscopies were performed.

Results:

The patient underwent laparoscopic exploration. An incidental intussusception made the tumor simple to identify. By extending the umbilical port, the tumor was easily removed. The final pathology demonstrated a submucosal lipoma.

Conclusions:

Small bowel lipomas can cause intussusception and gastrointestinal bleeding. When diagnosed preoperatively, laparoscopic resection is feasible.     

A giant adrenal cyst difficult to diagnose except by surgery

Introduction

Adrenal cysts represent rare clinical entities. Although surgical indications are well defined, pitfalls arise from the failure to establish an accurate preoperative diagnosis. Cystic lesions of other abdominal organs especially the pancreas complicate the diagnostic field.

Presentation of case

We present the case of a giant adrenal cyst in a young female causing diagnostic dilemma. Imaging studies revealed a large cystic lesion of uncertain origin located between the spleen and the tail of the pancreas. It was decided to perform a laparotomy which confirmed the presence of an adrenal cyst and enucleation of the cyst was performed. Examination at one year confirmed no complications.

Discussion

Adrenal cysts should always be included in the differential diagnosis of cystic abdominal lesions.

Conclusion

When the preoperative diagnosis is uncertain, surgical intervention can be both diagnostic and therapeutic.     

The effectiveness of patient verbalization on informed consent

Objective

To determine if preoperative patient verbalization of the risks and benefits of anterior cruciate ligament (ACL) reconstruction enhances understanding of the risks and benefits of that procedure.

Design

A randomized clinical trial.

Setting

A referral-based outpatient sport medicine clinic.

Subjects

Twenty patients from the general population with clinically diagnosed ACL tears requiring elective reconstruction surgery were randomly assigned to 2 groups. Twelve patients who made up a control group received a standard surgical consultation, consisting of knee models, diagrams, open dialogue and informed consent to surgery. Eight patients in the experimental group were exposed to the same surgical consultation and were required to accurately verbalize the associated risks and benefits before operation. One month after informed consent was obtained, patients answered 3 questions about the risks and benefits of ACL reconstruction.

Intervention

ACL reconstruction.

Main outcome measures

A 3-question questionnaire, addressing 2 risks and 1 benefit of ACL reconstruction.

Main results

Patients in the experimental group were able to answer all 3 questions correctly. In the control group, 4 patients answered all 3 questions correctly, but 1 patient answered all 3 questions incorrectly, and 7 patients answered 1 question incorrectly. There was a statistically significant difference (p = 0.03) between the control group and the experimental group.

Conclusion

Patients who verbalized the risks and benefits during their surgical consultation demonstrated a significantly greater understanding of the risks and benefits of an ACL reconstruction procedure.     

Mandibular Metastasis of a Signet Ring Cell Carcinoma of the Breast in a Patient who Underwent Bilateral Mastectomy more than 25 Years Earlier

Summary

Background

Metastatic tumors account for less than 1% of all malignant tumors occurring in the oral cavity.

Case Report

The clinical case of a 94-year-old patient with a mandibular tumor is reported here. The patient had undergone bilateral mastectomy more than 25 years before. An immunohistochemical study found hormone receptors in signet ring cells, suggesting a diagnosis of breast cancer metastasis.

Conclusion

Immunohistochemical diagnosis and antineoplastic hormone therapy is the cornerstone in the management of this clinical case.Key Words: Mandible, Metastasis, Breast cancer, Signet ring cells     

Outcome after Surgical Treatment of Pelvic Sarcomas

Background

We present here the oncological and functional outcomes of limb salvage with or without reconstruction for primary sarcomas in the pelvic bone.

Methods

Forty-four patients who underwent pelvic resection for primary sarcomas involving the pelvis were reviewed. The average follow-up period was 39 months (range, 0 to 146 months). Chondrosarcoma (n = 17) and osteosarcoma (n = 10) were the most common diagnoses. Various clinicopathologic factors were analyzed in relation to the oncological outcomes of overall survival and local recurrence. The Musculoskeletal Tumor Society functional scores and complications were compared according to the tumor location, pelvic continuity and the type of resection.

Results

The overall 5-year survival rate was 40%. Metastasis at the time of diagnosis, the surgical margin and the histologic grade were the independent prognostic factors for survival. The surgical margin was an independent prognostic factor for local recurrence. An ischiopubic location of the tumor, restoration of pelvic continuity and hip joint reconstruction with total hip replacement arthroplasty were related with higher functional scores. Complications occurred in 50% of the patients and the complications varied according to the tumor location with infection being the most common complication.

Conclusions

Achieving an adequate surgical margin is necessary for improving the oncological outcome of pelvic sarcomas. Restoration of the pelvic continuity and the hip joint improves the functional outcome. However, complications are common and so careful selection of the reconstruction method is needed.     

Mid common bile duct inflammatory pseudotumor mimicking cholangiocarcinoma. A case report and literature review

INTRODUCTION

Biliary inflammatory pseudotumors (IPTs) represent an exceptional benign cause of obstructive jaundice. These lesions are often mistaken for cholangiocarcinomas and are treated with major resections, because their final diagnosis can be achieved only after formal pathological examination of the resected specimen. Consequently, biliary IPTs are usually managed with unnecessary major resections.

PRESENTATION OF CASE

A 71-year-old female patient underwent an extra-hepatic bile duct resection en-bloc with the gallbladder and regional lymph nodes for an obstructing intraluminal growing tumor of the mid common bile duct (CBD). Limited resection was decided intraoperatively because of negative for malignancy fast frozen sections analysis in addition to the benign macroscopic features of the lesion. Histologically the tumor proved an IPT, arising from the bile duct epithelium, composed of inflammatory cells and reactive mesenchymal tissues.

DISCUSSION

The present case underlines the value of intraoperative reassessment of patients undergoing surgical resection for histopathologically undiagnosed biliary occupying lesions, in order to optimize their surgical management.

CONCLUSION

The probability of benign lesions mimicking cholangiocarcinoma should always be considered to avoid unnecessary major surgical resections, especially in fragile and/or elderly patients.     

Bilateral elastofibroma dorsi: A case report

INTRODUCTION

Elastofibroma dorsi(EFD) is slow-growing, benign, soft tissue tumor of unclear pathogenesis, typically located at the subscapular region of elderly people. It may be unilateral or bilateral. As it exhibits benign behavior, it should be surgically removed only in symptomatic patients. Magnetic resonance imaging (MRI) is a useful tool for assessment of EFD and can potentially help avoid the need for unnecessary biopsy and surgery.

PRESENTATION OF CASE

A 62-year-old woman presented with 2 years complaint of back pain, particularly aggravated with shoulder movements and swelling with bilateral elastofibroma dorsi. Both masses were totally excised with bilateral posterolateral subscapular incision. Symptoms were completely controlled and significant discomfort from the surgical procedure was completely resolved with in a few weeks interval from the operation.

DISCUSSION

The pathogenesis of the EFD still remains unclear. Elastofibroma dorsi has an unspecific clinical presentation and can be confused with other tumors of the periscapular region Imaging studies are useful for diagnosis.

CONCLUSION

Elastofibroma dorsi is benign soft tissue tumor mostly observed in the subscapular regions of elderly female patients. The surgery is indicated in symptomatic cases and must be confined to simple excision of the lesion.     

Diagnosis and surgical treatment of solid pseudopapillary neoplasm of the pancreas: analysis of 24 cases

Background

Our aim was to summarize our experience with the diagnosis and surgical treatment of solid pseudopapillary neoplasm (SPN) of the pancreas to provide a reference for the management of this rare condition.

Methods

We collected and analyzed retrospective data on the clinical presentation, laboratory investigations, radiologic imaging, pathology and operative details of patients with SPN of the pancreas diagnosed between February 2001 and December 2009.

Results

In all, 23 of 24 patients were women, and the mean age of all patients was 31 years. The most common clinical presentation was vague abdominal pain. Abdominal imaging showed solid or solid cystic masses in the pancreas, mostly in the tail or head of the gland. All patients were treated surgically. There were no postoperative deaths. After follow-up ranging from 4 to 109 months (median 68 mo), 20 of 22 patients who underwent curative resection were alive with no evidence of disease recurrence. Of the 2 patients with R1 resections, 1 died 42 months after surgery, whereas the other underwent a second operation and was alive after 36 months’ follow-up.

Conclusion

Solid pseudopapillary neoplasm of the pancreas is a relatively indolent tumour. The initial diagnosis of SPN of the pancreas is suggested by radiologic imaging findings but should be considered in the context of clinical and histopathologic characteristics. We advocate for complete surgical resection once SPN is diagnosed.     

Laparoscopic Resection of Retroperitoneal Neural Tumors

Purpose

Retroperitoneal neural tumor (RNT) is rarely excised laparoscopically, and the laparoscopic management of RNT remains controversial. We herein report 4 cases of laparoscopic excision of RNT that resulted in diverse clinical outcomes.

Patients and Methods

Between August 2005 and January 2011, we performed laparoscopic excision of RNT in 4 patients. The mean tumor size was 4.5 cm. The mean operative time was 297 minutes and the mean amount of blood loss was 55 ml. The surgeries were uneventful, with no operative complications or evidence of intra-abdominal bleeding. However, 2 patients required reoperation for delayed hemorrhage and urinoma formation, respectively.

Results

The postoperative pathological diagnoses were schwannoma in 3 patients and ganglioneuroblastoma in 1 patient. All patients were well with no signs of peripheral neuropathy or radiculopathy, and CT and/or ¹⁸F-FDG PET/CT performed during follow-up indicated no evidence of disease.

Conclusions

Obtaining extensive preoperative knowledge of the source neural and vascular anatomy of the tumor is important for the surgical planning of laparoscopic resection of RNT. When a great deal of care is taken to divide the tumor and the source nerves and vital vessels, safe execution of RNT can be achieved for minimal postoperative mortality and morbidity.Key Words: Laparoscopy, Retroperitoneal neural tumor, Schwannoma, Ganglioneuroblastoma     

Surgical strategy in abnormally increased Fluorine-18 fluorodeoxyglucose uptake in an asymptomatic lower esophageal submucosal tumor – Report of a case

INTRODUCTION

Leiomyoma is the most common benign tumor of the esophagus (67–80%), it represents 0.4–1% of all esophageal tumors.

PRESENTATION OF CASE

An incidentally discovered gastro-esophageal submucosal tumor was found to have increased fluorine-18-fluorodeoxyglucose (FDG) uptake on positron emission computed tomography (PET/CT). After laparoscopic surgical exploration and local enucleation the tumor turned out to be a benign esophageal leiomyoma.

DISCUSSION

There are few reports of esophageal leiomyomas with a positive uptake on (PET/CT) and even fewer adopting our combination of a minimally invasive approach and frozen section examination as a management plan. Our approach avoided excessive morbid surgical resections and underestimation of a malignant disease.

CONCLUSION

We report this case hoping to expand the existing literature on the topic and to highlight the limitations of PET/CT in guiding the diagnosis and subsequently the management of esophageal submucosal tumors.