Hepatic epithelioid hemangioendothelioma: A clinocopathological correlation

Primary hepatic epithelioid hemangioendothelioma (HEHE) is a rare tumor with an incidence of <0.1 per 100,000. The clinical course is variable with variable outcomes. Due to its rarity, treatment protocols, prognostic and predictive factors are not well established underscoring the need for such a study. Pathologists’ awareness of this entity, a meticulous morphologic examination coupled with immunohistochemistry can aid in accurate diagnosis.

Polymorphisms in cytotoxic T-lymphocyte associated antigen 4 gene does not affect scytotoxic T-lymphocyte associated antigen 4 levels in human papillomavirus-infected women with or without cervical cancer

Background: Cervical cancer (CaCx) is the second most common cancer in Indian women. Cytotoxic T-lymphocyte-associated antigen 4 (CTLA-4) + 49 AA polymorphism is known to be associated with CaCx. Current attempt is to use immunotherapy for the treatment of metastatic melanoma and metastatic castration-resistant prostate cancer, i.e., blocking of CTLA-4 using a fully human monoclonal CTLA-4 antibody to disrupt its inhibitory signal. This allows the CTLs to destroy the cancer cells. There is no information available on the soluble level of CTLA-4 on which the immunotherapy is targeted. This is specifically in Indian population including cases with CaCx. Objective: The aim of this study is to evaluate the levels of soluble CTLA-4 (sCTLA-4) in human papillomavirus (HPV)-infected women with or without CaCx and their association with the polymorphism at CTLA-4 + 49 A/G and CTLA-4 ?318 C/T genotypes. Materials and Methods: This is an exploratory case–control study involving two groups of HPV-infected women, the cases were with invasive CaCx and the control group was women with the healthy cervix. sCTLA-4 levels were measured using ELISA in 92 CaCx cases and 57 HPV-positive women with the healthy cervix. Results: Both cases and controls have similar sCTLA-4 levels. Comparison of CTLA-4 + 49A/G and ?318 C/T genotypes with sCTLA-4 levels among cases and control also did not show any statistically significant difference. Conclusion: The present study suggests sCTLA-4 levels are not affected by a polymorphism at + 49 A>G CTLA-4. Hence, levels of CTLA-4 are similar in both CaCx cases and control group.

Clinical characteristics with patterns of relapse and survival analysis of ovarian clear cell carcinoma.

AIMS: To analyze clinical characteristics, patterns of relapse, and treatment outcomes of clearcell carcinoma of the ovary (CCO). MATERIALS AND METHODS: Case files of 51 patients diagnosed with CCO between 2003 and 2010 were reviewed. RESULTS: The median age at diagnosis was 48 years (27-64 years). Fifty percent presented with nonspecific gastrointestinal symptoms. The median serum Ca125 was 74 IU/ml (6-1567 U/ml). Optimal cytoreduction was achieved in 32 (62.7%) patients. Of the 51 patients in this series, 34 (66.6%) had Stage I disease; Stage Ia in 12 (23.6%), Stage Ib in 1(1.9%), and Stage Ic in 21 (41.1%). Thirteen (25.6%) presented with Stage III and 4 (7.8%) with Stage IV. No patient had Stage II disease. All patients received 4-6 cycles of platinum‑based combination chemotherapy. There were 18 relapses (35.2%), with disease‑free intervals <6 months in 9, 6-12 months in 4, and >12 months in 5, respectively. Of them 33.3% had a recurrent pelvic mass. The median survival after relapse was 14 months. There were 13 deaths, 11 due to disease progression, 1 due to chemo toxicity, and 1 unrelated to disease. At a median follow up of 28 months, disease‑free survival (DFS) and overall survival (OS) of patients with Stage I-Stage II (early) disease was 64% and 80%, respectively. In patients with advanced disease, that is, Stages III and IV, DFS and OS were 35% and 38%, respectively. CONCLUSION: CCO generally presents at an early stage but has a high propensity for relapse. Patients with early‑stage disease have a relatively good prognosis as compared with those with advanced‑stage disease.

A cystic hepatic lesion: when to worry? / La lesión cística del hígado: ¿cuándo preocuparse?

A 16-year old female presented to hospital with abdominal pain. Features on computed tomography raised the possibility of biliary cystadenoma or cystadenocarcinoma. She underwent a liver resection, and histopathology confirmed a serous biliary cystadenoma. This case is presented to highlight the radiological features of this uncommon pre-malignant condition as well as to summarize a management algorithm for cystic liver lesions.

Una mujer de 16 años de edad acudió al hospital con un dolor abdominal. Las características observadas con tomografía computarizada apuntaban a un cistoadenoma biliar o un cistoadenocarcinoma como diagnósticos diferenciales. La paciente fue sometida a una resección del hígado, y la histopatología confirmó un cistoadenoma biliar seroso. Presentamos este caso para resaltar los rasgos radiológicos de esta condición premaligna rara, así como para resumir un algoritmo de tratamiento para las lesiones císticas de hígado.

Efficacy of species specific anti-scorpion venom serum (AScVS) against severe, serious scorpion stings (Mesobuthus tamulus concanesis Pocock)--an experience from rural hospital in western Maharashtra.

BACKGROUND: Death caused by scorpion envenoming is a common event in the tropical and subtropical countries including many regions in India. Severe scorpion envenoming causes an autonomic storm producing multi-system organ-failure (MSOF) and death. OBJECTIVES: To determine the efficacy of Anti-scorpion venom serum (AScVS) in patients stung by scorpions (Mesobuthus tamulus concanesis Pocock--earlier called Buthus tamulus); to compare it with other modalities of therapy and to detect complications, if any, arising out of AScVS treatment. METHODS: Total 48 patients of severe, serious scorpion envenoming syndrome were studied during the period from 1992 to 2002. In 17 patients AScVS was the only mode of treatment. Others had received adjunctive therapy along with AScVS. RESULTS: 47 patients out of 48 scorpion sting victims recovered completely. Recovery period in patients given AScVS (10 hours) was faster than those who received alpha blockers (16-42 hours). No anaphylactic reaction with AScVS was observed. CONCLUSIONS: AScVS is effective and safe method of therapy in severe scorpion envenoming syndrome.

Portal hypertensive jejunopathy: a case control study.

BACKGROUND: Small bowel mucosa is a recognized potential source of bleeding in portal hypertension. However, the frequency of its involvement is not known. AIMS: To document the nature, severity and frequency of endoscopic and histologic changes in the jejunum in patients with portal hypertension. METHODS: Forty consecutive patients with portal hypertension and 43 patients with non-ulcer dyspepsia (controls) underwent push enteroscopy and jejunal, duodenal and gastric biopsies. Biopsies were randomized and examined by a blinded pathologist for inflammation and vascular dilatation, which was quantified by morphometry. RESULTS: Endoscopic jejunopathy was observed in 6 patients and none of the control subjects. All patients with jejunopathy had portal hypertensive gastropathy (PHG) and 5 had duodenopathy. Vascular dilatation was observed in 15 patients and 25 control subjects (p = ns). The degree of vascular dilatation was similar in both groups. Inflammatory changes were observed in 24 patients and 25 control subjects (p = 0.05). CONCLUSIONS: Endoscopic jejunopathy was present in 15% of patients with portal hypertension. These changes were mild in 83% of them. All patients with jejunopathy also had PHG. Histologic changes were similar in patients and control subjects.

Animal model for liver dysfunction using lomustine in Wistar rats.

AIM: To induce intrahepatic cholestasis in rats using lomustine 1(2-chloroethyl)-3-cyclohexyl-l-nitrosourea (CCNU). METHODS: Doses of 10 mg, 20 mg and 30 mg/Kg body weight of CCNU were injected intraperitoneally in separate groups of animals. RESULTS: With 10 mg/Kg body weight of CCNU, serum bilirubin levels increased for up to 72 hours and then slowly returned to normal. With a dose of 20 mg/Kg body weight of CCNU, serum bilirubin, AST, ALT and alkaline phosphatase levels increased for 72 hours and then returned to normal over 4-5 weeks. With a dose of 30 mg/Kg body weight peak levels of serum bilirubin were reached on day 17. Pathological studies were carried out after injection of 30 mg/Kg body weight of CCNU. After 72 hours hepatocytes were normal, with minimal nonspecific inflammation and bile duct proliferation. After 16 days, triaditis was observed with deposition of collagen. Focal fibrosis was also noticed. There was no significant abnormality of hepatocytes. After 75 days, hepatocytes showed focal ballooning. Bile duct proliferation was seen invading the parenchyma. Nodules of hepatocytes separated by irregular fibrous bands indicated cirrhosis. CONCLUSIONS: An animal model of intrahepatic cholestasis has been developed using CCNU; this model may be used to assess the utility of hepatobiliary radiopharmaceuticals.

Autopsy study of patients dying of bleeding diathesis.

Ten thousand & thirty seven autopsies performed from the year 1982 to 1992 were studied retrospectively, to find out the number of deaths due to bleeding diathesis. Eighty-seven (0.87%) patients died due to bleeding diathesis, out of which haemolytic uraemic syndrome (HUS) was seen in 9 cases (10.34%), disseminated intravascular coagulation (DIC) in 67 cases (77.01%) & 11 cases were grouped as miscellaneous. Martius scarlet blue stain was carried out to demonstrate fibrin & depending on the number of thrombi in the glomerulus & blood vessels, the lesions were graded as mild, moderate or severe. Kidney was the most common organ involved in all groups of bleeding diathesis. In DIC kidney & lung involvement was almost equal.

Malignant interstitial (Leydig) cell tumour of the testis--a case report.

Interstitial (Leydig) cell tumour of the testis is an extremely uncommon tumour. We report one such tumour displaying histologic features of malignancy--large size capsular invasion and moderate nuclear pleomorphism in an adult male. The tumour also showed crystalloids of Reinke, confirming Leydig cell origin.

Blastopathies--symmetrical conjoined twins.

Conjoined twins are one of the rarest blastopathies, challenging the medical profession. The incidence in India is reported to be 1:60,000 pregnancies. This report records two cases of female conjoined twins. The sharing of various organs and complex cardiac anomalies encountered were very fascinating. Omphalopagus conjoined twins had only three lower extremeties, a common gastro-intestinal tract beyond the terminal ileum, crossed ectopia, hypoplastic kidneys and bicornuate uterus. The heart showed dextrocardia, A.S.D. and V.S.D. No attempt was made for surgical separation in this case. The second case was a thoracopagus, with the heart showing V.S.D., cor triatrium dexter with a posterior venous chamber. Surgical separation was attempted, but was unsuccessful. Both cases showed a single fused liver. The need for antenatal diagnosis and successful separation with good pre- and post-operative management is stressed.

Spontaneously occurring liver cell adenoma in an adult male.

Spontaneously occurring liver cell adenoma is an extremely rare event. One such tumour in an adult male which was resected surgically is reported here.

Hepatic hemangioendothelioma of infancy with congestive cardiac failure--report of a case.

A case of infantile hemangioendothelioma of the liver is reported in a 18 day-old male neonate. The neonate presented with CHF. He had hepatomegaly, but lacked the third component of the classical triad i.e. cutaneous hemangiomas. The S.A.F.P levels were raised to 920 ng/ml, though rise is rare in infantile hemangioendothelioma and led to the clinical diagnosis of hepatoblastoma. A left hepatic resection was performed, the histology showed a type I pattern of the tumor. This is known to be associated with a good prognosis. The baby is well post resection for a follow up period of six months.

Autopsy study of infant kidneys in cases of urinary tract anomalies.

The infant autopsies were scrutinised to find the incidence, etiology & significance in terms of fatality of urinary tract anomalies. On analysing 1329 infant autopsies, 24 cases (1.8%) of significant urinary tract anomalies were found. There was marked male predominance. Intrauterine obstruction of urinary tract was the main etiological factor leading to dysplastic changes in the renal tissue. Dysplasia is severe in extent and may be associated with persistent blastemal zones when the obstruction is complete & bilateral in nature.

Esophageal involvement in pemphigus vulgaris.

Twenty five patients with pemphigus vulgaris were prospectively studied for esophageal involvement by endoscopic examination. Eighteen (72%) had esophageal involvement; the majority of the lesions were bullae. Histological examination showed acantholysis of the cells and exuberant inflammation.

Retrospective treatment analysis of Salmonella infections.

Twenty four culture proved and nine postmortem histopathology proved cases of enteric fever were analysed retrospectively with special interest in use of various antisalmonella agents. Chloramphenicol resistance was noted in 91.7% and yet 70% of all patients received chloramphenicol alone or in combination with another antisalmonella agent. Time required for remission of fever with chloramphenical, cotrimoxazole and ciprofloxacin was 4.5, 4.1 and 6.9 days respectively. An interesting feature noted in post-mortem histopathology proved cases was enteric carditis which was documented on postmortem examination of the heart in three out of four patients who died of peripheral circulatory failure.