Cholecystitis, cholelithiasis, and ganglioneuromatosis of the gall bladder: an unusual presentation of MEN type 2b.

A 40 year old man with multiple endocrine neoplasia type 2b (MEN 2b) presented with cholecystitis caused by gall stones. Twenty four years earlier, he had had a partial thyroidectomy for a cold nodule. At his initial presentation MEN 2b with medullary carcinoma of the thyroid had not been made. This was diagnosed while investigating his gall bladder symptoms and he was found to have asymptomatic residual medullary thyroid carcinoma and bilateral adrenal phaeochromocytomas. The cholecystectomy specimen contained several mixed calculi and extensive ganglioneuromatosis with large, prominent nerves containing ganglion cells in the gall bladder wall.     

Primary carcinoma of gall bladder--a review of thirty six cases

The study presents the clinicopathological findings in thirty six cases of primary carcinoma of gall bladder diagnosed at SKIMS Srinagar over a period of four years (1983-86). Particular attention was paid to age, sex, presenting symptoms, presence of gall stones and site of metastasis. Majority of patients were found to be middle aged females (24 out of 36 cases). Most common presenting symptom was abdominal pain and duration of symptoms was two months or less in majority of cases. Gall stones were found in 55.5% cases. Diagnosis of primary gall bladder carcinoma was an incidental histopathological finding in 22% patients. Well differentiated adenocarcinoma was found to be the commonest type of malignancy (77.7%). 33% cases had widespread metastatic disease at the time of operation and liver was the commonest site of invasion (47.2%). Follow up records of 21 out of 36 patients available show only two patients to be alive till date with more than two year follow up and in both these cases carcinoma was an incidental histopathological diagnosis at cholecystectomy. Rest of the patients had died within 5-6 months after surgery. This reinforces the poor outcome for primary gall bladder carcinoma patients irrespective of treatment unless diagnosed and treated at a very early stage. Long term survival can be expected in incidentally found carcinoma at cholecystectomy.     

Metastatic renal cell carcinoma of the gallbladder

Metastatic renal cell carcinoma is renowned for its potency to spread to almost any organ of the body; however metastasis to the gall bladder is very rare. We present a case of a 48 year old man who initially demonstrated renal cell carcinoma, and in who gallbladder metastasis was later detected. A review of the literature revealed only a small number of cases of renal cell carcinoma metastasizing to the gallbladder, and these were primary found upon necropsy. Gallbladder metastasis in this case was detected clinically.     

S100P as a marker for poor survival and advanced stage in gallbladder carcinoma

AimsGallbladder carcinomas usually present in advanced stages and has a dismal prognosis despite modern imaging techniques and aggressive surgical intervention. Identification of biologic markers for early diagnosis and improved therapeutic strategies is thus of paramount importance. S100P has been identified in a variety of malignant neoplasms of the gastrointestinal and pancreaticobiliary systems, but it is not yet known if S100P expression is associated with clinically-relevant characteristics of gall bladder carcinoma. The aims of the present study were: 1) to investigate the relationship between S100P expression and histological type, grade, tumor-node-metastasis stage, presence of vascular invasion, perineural invasion and necrosis; and 2) to evaluate for any S100P-defined difference in the risk for tumor recurrence or death.MethodImmunostains for S100P were performed on 4 tissue microarray blocks containing 91 cases of gall bladder carcinoma.ResultThe intensity of S100P staining was significantly associated with pathological T stage 4 (p = 0. 0238). Staining intensity ≥3 in ≥25% tumor cells was associated with pathological T stage 4 (p = 0.0005). A higher S100P immunoreactivity score (IRS) was significantly associated with higher TNM stage (p = 0.0341). Age (p = 0.0485), presence of vascular invasion (p = 0.0359), pathological T stage (p = 0.0291) and TNM stage (p = 0.0153) were significantly associated with tumor recurrence. Intense S100P reactivity was associated with decreased overall survival [hazard ratio = 9.614; 95% confidence interval (CI), 1.873–49.338; p = 0.0067].ConclusionOur findings indicate that S100P over-expression is a potential prognostic marker for gall bladder carcinoma and is significantly associated with advanced tumor stage and poorer survival.     

Hepatobiliary cystadenocarcinoma with cystadenoma elements of the gall bladder in an old man

Hepatobiliary cystadenoma and cystadenocarcinoma of the gall bladder have rarely been reported. An 88-year-old Japanese man was admitted to our clinic because of hypochondralgia and jaundice. Imaging techniques revealed hemobilia and a multilocular cystic tumor in the fundus of the gall bladder, and cholecystectomy was performed. Grossly, the tumor (3.5 x 3 x 3 cm) was multicystic, containing seromucous fluid. The tumor was located in the fibromuscular layer and subserosa of the gall bladder fundus, and protruded into the serosal surface, not into gall bladder lumen. The mucosa appeared free of tumor involvement, and no gall stones were recognized. Microscopically, the tumor was located in the fibromuscular layer, subserosa and tiny focus of the mucosal surface. The tumor consisted of mucin-rich benign columnar cells, dysplastic mucous cells, malignant papillotubular cells and invasive carcinoma cells. Malignant and atypical tumor cells were located in the center of the tumor and in the tiny area of the mucosal surface, while benign tumor cells were located in the peripheral portions of the tumor and in the serosal side. Neither ovarian stroma-like mesenchymal stroma nor an oncocytic change in tumor cells was recognized. Non-tumorous gall bladder showed chronic cholecystitis. Immunohistochemically, benign and carcinoma cells were positive for cytokeratins, epithelial membrane antigen, CA19-9, MUC1, MUC5AC and MUC6, and carcinoma cells were also positive for carcinoembryonic antigen and p53 protein. The present case indicates that hepatobiliary cystadenocarcinoma without mesenchymal stroma may occur in the gall bladder of old men, and suggests that hepatobiliary cystadenoma without mesenchymal stroma may transform into hepatobiliary cystadenocarcinoma in the gall bladder.     

Ceruminous gland adenoid cystic carcinoma with contralateral metastasis to the brain.

We present the case of a 38-year-old man with an adenoid cystic carcinoma originating from the ceruminous glands of the external ear canal. The patient subsequently presented with a contralateral brain mass that was also diagnosed as adenoid cystic carcinoma. To our knowledge, contralateral metastasis to the brain of a patient with an adenoid cystic carcinoma of the ceruminous glands has not been reported previously. This rare neoplasm should be considered in the differential diagnosis of poorly differentiated carcinomas metastatic to the central nervous system in patients with occult malignant neoplasms.     

Inappropriate mucin production in gall bladder metaplasia and neoplasia–an immunohistological study

This study demonstrates the presence of three antigens in glandular metaplasia occurring in patients with cholecystitis and cholelithiasis: specifically carcinoembryonic antigen (CEA), large intestinal mucin antigen (LIMA) and small intestinal mucin antigen (SIMA). These antigens could not be detected in normal gall bladder mucosa or in squamous metaplasia of the gall bladder. The occurrence of the three intestine-associated antigens in three carcinomas was irregular. In one mucinous carcinoma, only SIMA could be demonstrated. In one adenocarcinoma, SIMA was present in small areas of mucinous change, whilst CEA was present in the nonmucinous malignant tissue. In a mixed mucinous and non-mucinous adenocarcinoma with widespread dissemination, the three antigens were present both in the primary tumour and the metastases. These observations suggest that all forms of glandular metaplasia of the gall bladder are intestinal in nature and at least a proportion of gall bladder carcinomas are of an intestinal type. Finally they provide further immunological evidence that glandular metaplasia of the gall bladder should be considered a pre-malignant condition.     

CT预测肌层浸润性膀胱尿路上皮癌盆腔淋巴结转移的回顾性研究

目的　探讨CT预测肌层浸润性膀胱尿路上皮癌（muscle-invasive bladder urothelial carcinoma,MIBUC）伴盆腔淋巴结转移的可行性。 方法　回顾性分析接受根治性全膀胱切除术加盆腔淋巴结清扫术的40例MIBUC患者的临床和影像学资料,对比分析CT检查的盆腔淋巴结情况与病理诊断结果之间的相关性。 结果　（1）以病理诊断结果为标准,CT检查发现MIBUC伴盆腔淋巴结转移的准确率为90%,灵敏度为75%,特异度为93.8%;（2）临床分期为T1、T2、T3、T4期的MIBUC患者,其盆腔淋巴结转移的发生率分别为0、9.1%、45.5%、100%。 结论　（1）CT影像学检查可较好地预测MIBUC是否伴盆腔淋巴结转移;（2）随着MIBUC临床分期的进展,盆腔淋巴结转移的发生率相应增高（P<0.05）。     

CT预测肌层浸润性膀胱尿路上皮癌盆腔淋巴结转移的回顾性研究

目的　探讨CT预测肌层浸润性膀胱尿路上皮癌（muscle-invasive bladder urothelial carcinoma，MIBUC）伴盆腔淋巴结转移的可行性。 方法　回顾性分析接受根治性全膀胱切除术加盆腔淋巴结清扫术的40例MIBUC患者的临床和影像学资料，对比分析CT检查的盆腔淋巴结情况与病理诊断结果之间的相关性。 结果　（1）以病理诊断结果为标准，CT检查发现MIBUC伴盆腔淋巴结转移的准确率为90%，灵敏度为75%，特异度为93.8%；（2）临床分期为T1、T2、T3、T4期的MIBUC患者，其盆腔淋巴结转移的发生率分别为0、9.1%、45.5%、100%。 结论　（1）CT影像学检查可较好地预测MIBUC是否伴盆腔淋巴结转移；（2）随着MIBUC临床分期的进展，盆腔淋巴结转移的发生率相应增高（P<0.05）。     

Primary malignant melanoma of the gall bladder.

A case of primary malignant melanoma of the gall bladder is reported, in which a 29 year old man presented with acute cholecystitis which led to perforation of the gall bladder and biliary peritonitis. To help in the differentiation between primary and secondary malignant melanoma in the gall bladder and to overcome some of the difficulties posed by the clinical identification of what is often a small or relatively inaccessible primary tumour, it is suggested that certain criteria should be fulfilled before primary melanoma is diagnosed. (i) Tumours must be solitary and arise from the mucosal surface of the gall bladder; (ii) they must either be papillary or polypoid; (iii) they must either display junctional activity or have any other primary sites excluded by history taking, examination, and investigation. If these criteria are applied to the published case reports of primary malignant melanoma, only six cases, including the present one, can be regarded as true primary tumours.     

Micropapillary transitional cell carcinoma of the urinary bladder: report of two cases

Micropapillary carcinoma is an uncommon variant of urothelial carcinoma with apparent high metastatic potential. The reported cases in the literature were associated with high grade and stage of disease at presentation and a poor prognosis. Micropapillary carcinoma is considered a tumor with an aggressive behavior, even though the morphology may be deceptive. The presence of a micropapillary carcinoma component in bladder biopsies should alert the urologists to the potential of higher stage disease and deep biopsies should be obtained. Two cases of micropapillary carcinoma of the urinary bladder were presented. A 71-year-old woman and a 68-year-old man presented with urinary symptoms. Cystoscopy revealed a papillary tumor on the bladder wall in both cases. Pathologic examination of transurethral resection specimen showed an invasive micropapillary carcinoma; small solid nests lying in small clear spaces that were not stained with antibody CD34. Thus, the lacunar histological pattern did not appear to represent invasion of vascular spaces. Only one case showed an association with urothelial carcinoma. No case showed muscle invasion. No recurrence or metastasis were observed after the initial diagnosis in the two cases.     

An unusual case of thyroid papillary carcinoma with solitary cerebral metastasis presenting with neurological symptoms

Papillary carcinoma of the thyroid is well known for metastasis to regional lymph nodes resulting in early detection and overall favorable prognosis. Solitary metastasis of papillary carcinoma of the thyroid in the cerebrum is extremely rare with only a few cases reported in the literature. Here, we report an unusual case of a 40-year-old female patient who presented with neurological symptoms and a radiological diagnosis of meningioma was made. Subsequent histopathology from the resected brain tumor revealed a solitary cerebral metastasis from papillary carcinoma of the thyroid. There was no involvement of regional lymph nodes and the radiological findings were indicative of a primary brain tumor.     

Secondary tumors of the pancreas: Clinicopathological study of 103 autopsy cases of Japanese patients

To investigate the clinicopathological features of patients with secondary tumors of the pancreas, we reviewed autopsy records and pathological features of 103 cases with pancreatic secondary tumors from 690 cases of malignant tumors (excluding cases of primary pancreatic cancer) over a 10-year period. There were 67 men and 36 women in the study, ranging in age from 2 to 94 years (mean: 61 years). The incidence of pancreatic secondary tumors was 15% in the autopsy cases of malignant tumors, and the majority of the secondary tumors were carcinomas. The stomach was the most common primary tumor site (20%), followed by the lung (18%) and extrahepatic bile duct (13%). Because the total number of each primary carcinoma differed, we paid specific attention to the incidence of pancreatic metastasis in each primary carcinoma. We found that carcinoma of the papilla of Vater showed the highest rate of incidence (75%) of pancreatic metastasis in each type of primary carcinoma. Approximately half of the metastatic lesions were solitary, but the metastatic lesions in the pancreas could not be identified macroscopically in 34 cases (33%). Histologically, the most common carcinoma was adenocarcinoma, followed by large cell carcinoma, small cell carcinoma and neuroendocrine carcinoma. The most common non-epithelial tumor was leukemia, followed by malignant lymphoma. Undifferentiated carcinoma and neuroendocrine carcinoma were often found in cases of extrahepatic bile duct or urinary bladder carcinoma with pancreatic metastasis. As for the microscopic infiltration patterns of tumor cells, 73% of cases showed an interlobular and intralobular infiltration. Fat necrosis was most frequently seen as an associated pathological finding (19%). Our study indicates that secondary tumors of the pancreas can be found in approximately one out of six to seven autopsy cases of malignant tumors, and in Japan, the most common of these is adenocarcinoma of the stomach.     

Pathological fracture of the patella as the first presentation of renal cell carcinoma

The patella is a rare site for a metastasis to occur. We present a patient whose presenting symptom of renal cell carcinoma was a pathological fracture of the patella.     

Micropapillary urothelial carcinoma: Clinico-pathologic review

Micropapillary carcinoma is a rare distinct variant of high grade urothelial carcinoma, which has specific morphological characteristics and is almost always associated with muscularis propria and vascular invasion. No currently defined imaging techniques can reliably diagnose some types of deeply invasive urothelial carcinoma of urinary bladder, in particular its micropapillary variant. Therefore, the pathological findings are crucial in making the diagnosis. Micropapillary carcinoma (MPC) is a tumor with an aggressive clinical course, an advanced stage of disease at the time of presentation, and usually a poor outcome. Metastatic micropapillary carcinoma to bladder should always be included in the differential diagnosis. Correct histological diagnosis of this aggressive neoplasm would allow timely, albeit intense, radical treatment of the disease. The current most generally favored treatment option for all patients who present with MPC is immediate radical cystectomy.     

Management of Ramsay Hunt Syndrome in an Acute Palliative Care Setting

Introduction:

The Ramsay Hunt syndrome is characterized by combination of herpes infection and lower motor neuron type of facial nerve palsy. The disease is caused by a reactivation of Varicella Zoster virus and can be unrepresentative since the herpetic lesions may not be always be present (zoster sine herpete) and might mimic other severe neurological illnesses.

Case Report:

A 63-year-old man known case of carcinoma of gall bladder with liver metastases, post surgery and chemotherapy with no scope for further disease modifying treatment, was referred to palliative care unit for best supportive care. He was on regular analgesics and other supportive treatment. He presented to Palliative Medicine outpatient with 3 days history of ipsilateral facial pain of neuropathic character, otalgia, diffuse vesciculo-papular rash over ophthalmic and maxillary divisions of left trigeminal nerve distribution of face and ear, and was associated with secondary bacterial infection and unilateral facial edema. He was clinically diagnosed to have Herpes Zoster with superadded bacterial infection. He was treated with tablet Valacyclovir 500 mg four times a day, Acyclovir cream for local application, Acyclovir eye ointment for prophylactic treatment of Herpetic Keratitis, low dose of Prednisolone, oral Amoxicillin and Clindamycin for 7 days, and Pregabalin 150 mg per day. After 7 days of treatment, the rash and vesicles had completely resolved and good improvement of pain and other symptoms were noted.

Conclusion:

Management of acute infections and its associated complications in an acute palliative care setting improves both quality and length of life.     

Primary multiple clear cell variant urothelial carcinomas of urinary bladder: a rare case report

Clear cell variant urothelial carcinoma of urinary bladder was very rare. There were only 6 report articles included by Pubmed and total 8 cases had been described till now. All of the past reports described single tumor of urinary bladder, but multiple carcinomas had not been reported. Here we reported a 65-years-old Chinese man who complained of intermittent gross hematuria and odynuria for more than 2 months in January 2013. Only one cauliflower-like tumor was detected approximately in the left wall of the urinary bladder with cystoscopy and the biopsy specimen was diagnosed as “urothelial carcinoma, high grade”. However, three tumors were found in anterior wall (×2) near neck of urinary bladder and posterior wall (×1) of the urinary bladder during transurethral resection of the bladder tumor. Typical urothelial carcinoma with partial clear cell appearance made it difficult to make a precise pathological diagnosis and immunohistochemical stain helped to diagnose the case as clear cell variant urothelial carcinoma, but not metastasis of the renal cell carcinoma. Finally, computerized tomographic scanning confirmed that there was no primary tumor in the kidney. The clinical and pathological characteristic had not been identified for the limited reports. More work should be done to know this kind of tumor well for guiding clinical therapy.     

Metastatic extraocular sebaceous carcinoma with an occult primary

The cytological diagnosis of sebaceous carcinoma is based on the aspiration of tumor lobules with extensive sebaceous differentiation. However, these tumors may show a spectrum of cells ranging from basaloid to well-differentiated sebaceous. Extraocular sebaceous carcinoma is an uncommon tumor. An occult primary at initial presentation has not been described previously. A 60-yr old woman was referred to the fine-needle aspiration (FNA) clinic with a submandibular mass. The cytological diagnosis was mucoepidermoid carcinoma. Histopathologically, it was a sebaceous carcinoma metastatic to the submandibular lymph node. No primary tumor was identified. Six months later, she presented with a nodule at the angle of her mouth and a recurrence of the submandibular swelling. FNA cytology (FNAC) and histopathologic examination revealed a primary cutaneous sebaceous carcinoma with submandibular lymph node metastasis. A metastatic sebaceous carcinoma in the submandibular region with limited sebaceous differentiation and occult primary is another pitfall in the cytomorphological diagnosis of mucoepidermoid carcinoma.     

肝炎和肝硬化患者胆囊B超改变的观察与分析

目的研究肝炎肝硬化患者胆囊彩色B超声像图变化，并探讨其临床意义。方法采用彩色超声诊断仪对139例肝炎和肝硬化患者及65例非肝炎体检者进行胆囊超声检查。结果慢性肝炎、重型肝炎、肝硬化患者组与非肝炎对照组彩色B超胆囊异常率比较均差异非常显著（P〈0．01）。慢性肝炎与重型肝炎、肝硬化组之间胆囊异常率也存在明显的差异（P〈0．05）。结论慢性肝病患者胆囊异常并不是胆囊本身炎症所致，胆囊声像图的改变对判断肝脏实质性病变的严重程度及指导临床治疗有一定的作用。     

Pathological fracture of the clavicle causing subclavian pseudoaneurysm formation

Pseudoaneurysm of the subclavian artery is a rare but serious condition generally occurring as a result of penetrating injury to the shoulder region, often iatrogenic. As well as the potential for distal ischaemia, this condition can result in compression of adjacent structures. Untreated it can lead to rupture with life-threatening haemorrhage. We describe the unusual case of a patient with a background of metastatic breast carcinoma who presented with a pseudoaneurysm of the subclavian artery secondary to a pathological fracture of the clavicle. The patient was managed surgically due to the development of necrosis of the overlying skin. Less invasive management of subclavian pseudoaneurysms has recently been described, including endovascular stenting and percutaneous thrombin injection.