A case of acquired hemophilia A with massive hemothorax

Acquired hemophilia A (AHA) is an uncommon but potentially life-threatening hemorrhagic disorder caused by the development of an inhibitor against coagulation factor VIII (FVIII). AHA is very rare, affecting approximately 1 in 1 million individuals. However, the incidence may actually be higher, because diagnosis is difficult and the disease can be overlooked. We report a case of an 80-year-old man who presented with sudden onset of severe hemothorax. The patient was diagnosed with presumed AHA based on acute onset of bleeding symptoms and unexplained isolated prolonged activated partial thromboplastin time. Diagnosis was definitely established by demonstrating a decrease in FVIII activity, presence of FVIII inhibitor activity, and normal von Willebrand factor. The patient was successfully treated with recombinant activated coagulation factor VII and transcatheter artery embolization of the intercostal arteries.     

Polymyositis as presenting manifestation of gallbladder carcinoma: A case report

INTRODUCTIONInflammatory myositis as a paraneoplastic presentation of gallbladder cancer is an extremely rare event. In this paper we reported the first case of gallbladder carcinoma presented as polymyositis.PRESENTATION OF CASEA 68-year-old housewife presented with proximal muscles weakness, pain, significant decrease in force of proximal muscles, and globally decreased deep tendon reflexes. Laboratory studies revealed an anemia, increased acute phase reactants and increased serum creatine phosphokinase (CPK) levels. Electromyography (EMG) and nerve conduction velocity test (NCV) demonstrated mild myopathic changes. Muscle biopsy was suggestive for polymyositis. Corticosteroid therapy initiated and a meticulous search for probable underlying malignancy performed concurrently. Malignancy workup finally revealed a gallbladder tumor. Patient candidated for extended cholecystectomy. Pathologic evaluation of gallbladder tumor demonstrated a moderately differentiated carcinoma. Progressive improvement in clinical conditions and complete normalization of laboratory parameters occurred post-operatively. After 8 months of follow-up patient is still alive and in good state of health. There is no evidence of metastatic or local recurrence of tumor. Musculoskeletal complaints subsided completely.DISCUSSIONGallbladder carcinoma is a rare and usually aggressive malignancy. Its primary presentation by paraneoplastic syndromes especially in the form of paraneoplastic neurological syndromes is an extremely rare event. Some believes that increased association between inflammatory myopathy and malignancy is limited to the dermatomyositis; however, presentation of our patient as polymyositis is contrary to this. This is the first reported case of gallbladder cancer who presented with polymyositis.CONCLUSIONGallbladder cancer though rare, should be considered in patients with inflammatory myositis.     

Metaplastic breast carcinoma with osseous differentiation: A rare case report

IntroductionMetaplastic breast carcinoma (MBC) is a rare type of breast cancer. Osseous differentiation is a very rare subtype. Reporting this kind of case is important because its clinical course and line of management are poorly mentioned in the literatures. We present a very rare case of MBC with osseous differentiation. A 48-year-old female presented with painless hard mass of the left breast. Examination and investigations showed MBC with osseous differentiation. She was managed by operation with adjuvant chemotherapy.ConclusionMBC with osseous differentiation is a very rare type of breast carcinoma presenting with hard mass and managed by mastectomy, axillary lymph node sampling and adjuvant chemotherapy.     

Acquired hemophilia A caused by factor VIII inhibitors: report of a case

We report a case of acquired hemophilia A (AHA) after esophageal resection. The patient was an 80-year-old woman whose preoperative activated partial-thromboplastin time (APTT) was well within the normal range, at 34.9 s. She underwent thoracic esophagectomy and gastric pull-up for superficial esophageal cancer (operative time, 315 min; intraoperative blood loss, 245 ml). Intrathoracic and subcutaneous bleeding occurred spontaneously on postoperative day (POD) 39. The APTT was prolonged, at 140 s, and factor VIII inhibitor was 36 Bethesda U/ml. Treatment with recombinant activated factor VII, prednisolone, and cyclophosphamide resulted in remission within 2 months. This case supports an association between surgery and the triggering of factor VIII inhibitors. The diagnosis of AHA requires clinical acumen and must be considered in any patient with bleeding and a prolonged APTT.     

Severe postoperative hemorrhage caused by antibody-mediated coagulation factor deficiencies: report of two cases

Antibody-mediated coagulation factor deficiencies constitute a rare disorder that may develop in elderly patients without any history of a bleeding diathesis. Patients may present with severe and sometimes catastrophic bleeding. We report two cases of postoperative hemorrhage caused by a coagulation factor deficiency. In Case 1, massive intraabdominal bleeding occurred on day 3 after pancreaticoduodenectomy for bile duct cancer, and was caused by an acquired inhibitor of coagulation factor VIII. Hemostasis was achieved and the factor VIII inhibitor titer decreased to zero with activated prothrombin complex concentrates, prednisolone, and cyclophosphamide. In Case 2, intraabdominal bleeding occurred on day 7 after hepatectomy for hepatocellular carcinoma, and was caused by an acquired inhibitor against factors II (prothrombin) and V. This patient was treated with hemostatic agents containing bovine thrombin during surgery and also with prednisolone. We report these cases to highlight that antibody-mediated coagulation factor deficiencies should be considered when an elderly patient suffers sudden postoperative hemorrhage and to stress the importance of prompt diagnosis because of the risk of potentially life-threatening hemorrhage.     

Unusual collision tumor with infiltrating ductal carcinoma and breast skin squamous cell carcinoma: A case report and literature review

IntroductionBreast cancer is the most common diagnosed cancer among women worldwide. Invasive ductal carcinoma (IDC) is the most common type, on the other hand, squamous cell carcinoma of the skin (SCC) overlying the breast is a rare tumor. The co-presence of two tumor types in one organ is even a rarer entity, termed as collision tumor. Only 3 known cases of collision tumor with breast invasive ductal and skin squamous carcinoma were reported in the literature.Case presentationAn otherwise medically free 91-year-old, postmenopausal, female presented with left breast fungating mass for four months. Pre-operative core tissue biopsy and incisional skin biopsy revealed two distinct tumor subtypes of invasive ductal carcinoma, positive for progesterone, estrogen receptors and negative for human epidermal growth factor receptor 2, as well as skin squamous cell carcinoma, and axillary lymph node metastasis. Patient underwent left breast modified radical mastectomy and split skin grafting for wound closure. The final histopathology was consistent with grade 2 IDC. The nipple and areola complex were involved by moderately differentiated squamous cell carcinoma. Currently patient on adjuvant hormonal treatment. Follow up showed no local recurrence or distal metastasis.ConclusionCollision tumors of the breast with IDC and SCC of the overlying skin is very rare. The surgeon has to be aware of of such entity as the proper peri-operative management should be tailored to target the most aggressive histologic subtype.     

Retroperitoneal unicentric Castleman's disease—A case report and review of literature

Introduction and importanceCastleman's disease is not so commonly diagnosed worldwide due to non-specific symptoms Clinical findings are variable.No definite blood investigation or any biomarkers are established to diagnose this disease. Radiological investigations do not play much role in diagnosing. It can be unicentric or multicentric. Etiological factors are not well understood except predilection of this disease towards immune-compromised persons. Surgery is considered as a prime modality to treat, if resectable.Case presentationPatient had recurrent abdominal pain in left lumbar region which was intermittent for last 6 months, dull aching with no aggravating and relieving factors. There was no history of abnormal bowel habits, urinary complaints, fever, night sweats, weight loss, rashes in body, joint pains and loss of appetite. On examination, there was a single, firm, non-tender, intra-abdominal, retroperitoneal mass of approximately size 10 1 7 cm in left lumbar region encroaching inferiorly towards left inguinal region. Digital rectal examination (DRE) and external genitalia were normal.Clinical discussionAll routine blood investigations along with relevant tumour markers were normal. Magnetic Resonance Imaging (MRI) abdomen showed a well-defined mass suggestive of stromal tumour or retroperitoneal sarcoma. Patient underwent surgery in which complete excision of the mass was done. Postoperative event was uneventful and currently doing well.ConclusionTake away lesson in this case report is that we should not presume all retroperitoneal mass as cancer or sarcoma, we have to think about other rare causes like Castleman's disease, if clinical picture, blood and radiological investigation are discordant.     

An elderly woman with triple primary metachronous malignancy: A case report and review of literature

INTRODUCTIONPrevalence of multiple primary malignancies is slowly increasing due to prolonged survival of cancer patients with advances in diagnostic and therapeutic modalities. The reasons may be environmental modifications, genetic predisposition or therapy induced. We describe a case of a 64-year-old woman with three different metachronous primary malignancies managed at our center since 4 years.PRESENTATION OF CASEFirst primary diagnosed in our patient was adenocarcinoma of small intestine which is a rare gastrointestinal malignancy. For this she underwent surgical resection followed by chemotherapy. After 21 months she developed infiltrating duct carcinoma of breast which was managed with modified radical mastectomy and chemotherapy. Again after latent period of 10 months patient had papillary adenocarcinoma of ovary for which she was administered chemotherapy. During follow up tumor was found to be chemoresistant and again she underwent cytoreductive surgery followed by chemotherapy.DISCUSSIONIn present case patient did not have significant risk factors for development of carcinoma of small intestine, breast and ovary. Our patient underwent surgical excision three times and received total 16 chemotherapy cycles of different regimens during management of all three primary malignancies. Development of second and higher order primary malignancy after successful management of previous one should be always kept in mind.CONCLUSIONAwareness, suspicion of multiple primary malignancy and aggressive diagnostic work up plays crucial role in their detection at earlier stage for better outcome. In addition choice of appropriate chemotherapeutic agents and their regimens remains the cornerstone while managing the patients with multiple primary malignancies.     

Bilateral giant inguinoscrotal hernia: A case report

IntroductionBilateral giant inguinoscrotal hernia (GIH) is rare and creates significant challenge in surgical management. The main concern of hernia reduction to abdominal cavity is development of abdominal compartment syndrome (ACS). Different approaches for prevention of ACS after surgery have been suggested.Case presentationWe report a case of 68-year-old male with bilateral inguinoscrotal hernia for 20 years reaching just below midpoint of thigh. He presented with difficulty in micturition and mobility. Preoperative investigations were normal. He underwent bilateral mesh repair without any preoperative or intraoperative adjunct measures. No significant complication occurred in postoperative period.Case discussionBilateral GIH is rare and the patients usually present late. GIH has been classified into three types on the basis of extension. Type I GIH can be managed with simple hernioplasty, in both unilateral and bilateral cases. Measures like resection of hernia contents and measures to enlarge intraabdominal space are warranted in type II and III GIH. Abdominal volume can be increased by utilising techniques like Pre-operative Progressive Pneumoperitoneum (PPP), injection of Botulinum toxin A (BTA) in the anterior abdominal wall, and rotation of viable tissue. The measures can be used either alone or in combination.ConclusionType I GIH can be treated with simple hernioplasty with safety with monitoring for features of ACS and respiratory complications postoperatively. However, additional measures like resection of hernia contents or procedures to enlarge intra-abdominal space are warranted for type II and III GIH.     

Epidermoid cyst of the spleen,a case report

IntroductionSplenic tumors are rare and are either primary or secondary, benign or malignant. Most have none to minimal symptomatology and are found incidentally. Splenic cysts can be infectious, congenital, or traumatic. Epidermoid cysts and parasitic cysts are examples of primary cysts and usually have a classic presentation on imaging. Despite advanced imaging modalities and patient's clinical presentation, it can be difficult to diagnose an epidermoid cyst without histological examination. The purpose of this paper is to discuss typical findings of primary splenic cysts on imaging, but how they may differ in appearance.Presentation of case51 year old female who presented with vague abdominal discomfort and was found to have a large splenic mass with cystic components on imaging which did not demonstrate a typical primary splenic cyst appearance. Patient underwent an uneventful hand-assisted laparoscopic total splenectomy and had an uneventful recovery with histopathology revealing an epidermoid splenic cyst.ConclusionPrimary splenic cysts are difficult to diagnose and differentiate with imaging alone. They have a variable presentation and can present like as a cystic mass. It is important to include them in the differential diagnosis of splenic masses since histopathology is the final determinant of the diagnosis.     

A rare case of breast cancer metastasising to the nasopharynx and paranasal sinuses

INTRODUCTIONMetastatic spread from non-head and neck tumours to the sinonasal region is exceedingly rare. We present a case of breast cancer metastasis to the nasopharynx, ethmoid and sphenoid sinuses. To date there have been only two similar cases in the literature. We discuss the diagnosis and management of such cases and propose how they may be staged.PRESENTATION OF CASEA 75-year-old woman with past medical history of breast carcinoma, presented clinically as having a primary sinonasal malignancy. Magnetic resonance imaging (MRI) demonstrated a lesion involving the spenoid and ethmoid sinuses, nasendoscopy revealed a mass in the nasopharynx. Biopsy from clinic pointed to inverted sinonasal papilloma, however this did not fit with the MRI or the clinical picture. Repeat biopsy under image guidance revealed the lesion to be a breast cancer metastasis.DISCUSSIONAn extensive literature review revealed few cases of spread to the sinonasal region from distant primary malignancy. When such cases do arise, most are from renal tumours. Breast cancer metastases usually present with signs and symptoms of disseminated disease, however our case represents a true isolated metastasis. We discuss the management of our case and suggest the use of the tumour-node-metastasis (TNM) system, in order to stage these rare isolated occurrences.CONCLUSIONIf discovered early, this rare manifestation may be managed by primary surgical resection. Metastases to the region may be more common than previously thought. A high index of suspicion should be employed, especially where there is past medical history of malignancy.     

Management of postoperative hemorrhage associated with factor VIII inhibitor: report of a case

This report presents a case that was successfully treated for acquired factor VIII inhibitor after extensive visceral surgery. A 71-year-old male who underwent surgery for bile duct cancer had active bleeding in the abdominal drainage tube on postoperative day (POD) 5, and prolonged activated partial thromboplastin time (aPTT) was detected (83.1 s) on POD 7. An extensive coagulation work-up revealed factor VIII deficiency (1 %), and a diagnosis of an acquired factor VIII deficiency was established when a factor VIII inhibitor of 8 Bethesda units was demonstrated. The patient was treated with activated prothrombin complex concentrate (aPCCs) and bloody discharge was stopped within 3 days. Inhibitor elimination was started using prednisolone on POD 20; rituximab, was administered on POD 74 and 81. Factor VIII inhibitor had disappeared by POD 124, and factor VIII (72 %) and aPTT recovered to 45.9 s. This case report demonstrated the efficacy of aPCCs and rituximab in the treatment of acquired hemophilia associated with visceral surgery.     

Completion pneumonectomy of the residual left lung to treat lung cancer in a patient with hemophilia a: Report of a case

Hemophilia A is a sex-linked recessive hereditary disease that is relatively rare and the number of patients with this disorder who undergo major surgery is limited. Although replenishing coagulation factors can allow hemophiliac patients to undergo similar surgery to that performed for patients without hemophilia, there have been few reports on major surgery and none on the resection of lung cancer in patients with hemophilia A. We recently performed completion pneumonectomy of the left lung in a 70-year-old man with hemophilia A, for squamous cell carcinoma in the residual left lung. The administration of a recombinant DNA coagulation factor VIII preparation allowed this operation to be successfully carried out. This case serves to demonstrate that the recombinant DNA coagulation factor VIII preparation described may enable us to safely perform major surgery on hemophiliac patients, since there is no risk of viral infection or any other adverse effects, such as deterioration of immunocompetence or hemolysis, which are occasionally encountered with human plasma-derived preparations. Received: September 1, 1999 / Accepted: May 30, 2000     

Management of radiation-induced sarcomas in a tertiary referral centre: A review of 25 cases

IntroductionRadiation-induced sarcomas are a rare and late complication of radiotherapy for breast carcinoma which often have poor prognosis.MethodsThis study is a retrospective review of 25 patients referred to a regional sarcoma unit between 1978 and 2009.ResultsRadiation-induced sarcoma following the diagnosis and treatment of breast cancer occurred after a mean period of 156 months. Anatomical sites involved were the breast, chest wall, clavicle, scapula, humerus and axilla. Twenty one patients had wide local excision followed by chest wall reconstruction, latissimus dorsi flap cover or limb amputation. The estimated five years survival following the diagnosis of the radiation-induced sarcoma was 27% and the local recurrence rate 52%.ConclusionRadiation-induced sarcoma following breast cancer has high local recurrence rate and poor prognosis. They should be managed in a multi-disciplinary setting. Long-term follow-up of patients treated with radiotherapy for breast cancer is therefore advisable.     

Quadruple primary urogenital cancers – A case report

IntroductionUrogenital cancers are not an uncommon occurrence in daily practice. Prostate cancer is the second most frequent cancer in men, kidney cancer accounts for 2.4% of all cancers and bladder cancers represent 3.1% of cancers in both men and women [1]. However, the cases of a simultaneous development of all three cancers, including one with a neuroendocrine component, are very few and far between.Presentation of caseOur case report involves a case of a patient with prostate adenocarcinoma, clear-cell renal carcinoma, papillary renal carcinoma and small-cell bladder cancer. The patient was treated as if he had separate pathologies by a multidisciplinary team: surgical and oncological, performing radical cystoprostatectomy with left perifascial nephroureterectomy, right ureterostomy and adjuvant chemotherapy, with excellent outcome even four years after the initial diagnosis.DiscussionThe distinct features of this case are the occurence of four different malignancies of the urogenital system, the family history of colon cancer, the development of small-cell carcinoma of the bladder, which is extremely rare and the good outcome, despite the quadruple malignancies and the aggresivity of the small-cell carcinoma.ConclusionMutiple primary malignancies are a relatively rare pathology, but should be considered as a possibility in patients who already had a second malignancy. Cases of patients with MPMs should be supervised by a multidisciplinary team and should be followed closely.     

Unusual case of cavitary lung metastasis of esophageal cancer: A case report

Introduction and importanceCavitary lung metastases are rare. We experienced lung metastasis of esophageal cancer with a cavity, which is extremely rare.Case presentationA 69-year-old female diagnosed with thoracic esophageal cancer underwent radical esophagectomy. Pathological diagnosis was T3N0M0-pStageII. Cavitary lesion appeared in the lower lobe of the right lung 12 months after surgery. We suspected an inflammatory change and followed up strictly. On Computed tomography (CT) image 18 months after surgery, the cavitary lesion slightly increased in size, showing wall thickening and fluid inside. Fluid collection disappeared after antibiotic treatment. The patient was followed for 4 weeks without antibiotics and fluid collection reappeared. Cavitary lesion resection for the purpose of diagnosis was planned because malignancy could not be excluded, although the readministration of antibiotics was also considered. Thoracoscopic partial resection of the right lower lobe of the lung was performed. Histopathological examination showed squamous cell carcinoma which was similar in morphology to esophageal cancer. The final diagnosis was lung metastasis of esophageal cancer.Clinical discussionMetastatic lung cancer with a cavity is rare, accounting for approximately 4% of all cases. Moreover, Lung metastasis of esophageal cancer with a cavity is extremely rare.ConclusionsThe specimen collection, including total biopsy, should be aggressively performed when diagnosing cavitary lung disease in patients with a history of neoplastic disease.     

Broad ligament hernia: Two contrasting ways to a common goal – Two case reports with review of literature

IntroductionBroad ligament hernia (BLH) is a rare but potentially life threatening condition. One of the two cases described here is the only reported case of BLH in recent literature, where marsupialisation was done. These two case reports comprise the only reported side by side pictorial comparison of the two laparoscopic surgical therapeutic options for BLH.Presentation of casesBoth patients presented with classical symptoms and signs of acute intestinal obstruction. Imaging confirmed obstructed left BLH in case 2 and indicated a complete small bowel obstruction in case 1. Both cases were successfully managed laparoscopically. Both patients had an uneventful immediate postoperative recovery and have not had recurrence over a mean follow up period of 34.5 months.DiscussionBLH is rare among all types of internal herniae. It accounts for only 4% of internal herniae and is a difficult condition to diagnose. The advent of computed tomography has increased chances of accurate preoperative diagnosis.ConclusionBLH can be successfully managed by minimally invasive surgery, even in the acute setting. When tightly entrapped bowel is unyielding; it is better not to risk injury to it by aggressive attempts at its reduction. It is safer to attempt widening of the defect into which it is entrapped, whenever feasible.     

Adrenal failure due to bilateral adrenal metastasis of rectal cancer: A case report

IntroductionIt is rare for a patient to present with adrenal insufficiency secondary to bilateral adrenal metastases from a malignant colorectal tumor.Case presentationAn 82-year-old Japanese man presented to our hospital with high fever and malaise. He was receiving oral chemotherapy for the treatment of rectal cancer with multiple metastases. Computed tomography showed new bilateral adrenal gland metastases. A rapid adrenocorticotropic hormone (ACTH) test showed adrenal insufficiency. Treatment with hydrocortisone provided immediate symptom improvement.DiscussionAdrenal insufficiency secondary to bilateral adrenal metastases from rectal cancer is rare. A rapid ACTH test is useful to diagnose adrenal insufficiency.ConclusionThe incidence of adrenal insufficiency may be underestimated in patients with multiple metastasis. Appropriate therapy with adrenal corticosteroid hormone supplementation may lead to a significant improvement in the patient’s symptoms and quality of life.     

Non-muscle invasive transitional cell carcinoma of the distal ureter and bladder with lung metastasis: A case report and literature review

INTRODUCTIONNon-muscle invasive transtitional cell carcinoma (TCC) with metastases is exceedingly rare.PRESENTATION OF CASEWe report the case of a 78-year old man with an incidental finding of a non-muscle invasive TCC of the left distal ureter during treatment for Duke's B Colon cancer. Following laparoscopic nephro-ureterectomy (LNU) he had two further superficial recurrences in the bladder over a 14-month period which underwent transurethral resection (TUR). On surveillance imaging for his colorectal cancer follow-up a lung nodule was detected and video-assisted thoracic surgical (VATS) resection of the lesion showed it to be TCC in origin. He was referred to oncology for chemotherapy and remains clinically well.DISCUSSIONA Literature search found only three other such cases and the ureteric TCC is the most likely source of the metastasis.CONCLUSIONThis occurrence is exceedingly rare.