Friday, December 31, 2010

ITS FIBROLAMALAR VARIENT OF HCC^-www.drkeyurbhatt.in*

70 yrs female with dull aching pain in RHC region no other positive symptoms...
On examination Huge liver Mass...

AFP & CEA LEVELS ARE NORMAL
LFT, CBC,RFT : ALL NORMAL...

ARTERIAL PHASE

PORTAL PHASE

DELAYED PHASE 


ANGIO RECONSTRUCTION 



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CASE: CONGENITAL DIAPHRAGMATIC HERNIA PRESENTED IN ADULTHOOD^-www.drkeyurbhatt.in*

Incidetaly detected Congenital diaphragmatic hernia......explained and Reassurance given....surgery is needed only in case of symptoms....




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CASE : Sad and Strange-- perforation in both Jejunal limbs of Previous J-J & LPJ done in 2008 for Chronic pancreatitis^-www.drkeyurbhatt.in*

Past history:
21 yrs boy with recurrent pain in abdomen...diagnosed as chronic pancreatitis and underwent Pustow's procedure in 2008, Eight days post op had acute obstruction and underwent re laparotomy...(Hydrabad)

remain okey for about  6 months ...than started developing recurrent episodes of pain in abdomen...all the time thought of sub acute obstruction / ? recurrent pancreatitis....became addict to Dynapar / Contromol...

developed acute intestinal obstruction...in Jan 2010...and once again was operated and adhesiolysis done....(mumbai)
again having multiple episodes of pains......was planned of Head corring and revision of surgery of Pancreas in Mumbai...admitted and evaluated for surgery but in view of malnutrition and low albumin deferred of surgery...and 1 month TPN given...and discharged to build up...

Again started of having pain ...got admitted...and thought of adhesions / ? pancreatitis...2-3 days treated conservatively....not responding to Rx and started more detoriating ....CECT was done....and it was peritonitis...
I got REF....and I had To Explore .....for the fourth time....and Oh My God....
he had 2.5 liters of peritonitis ...3 days old...already on Inotrops and low albumin..sepsis...
and Two perforations in both the limbs proximal of Previous J-J......horrible...just 1 feet from DJ...



WE SHOULD  RULE OUT ALL ORGANIC CAUSES BEFORE LABELING THAT PATIENT IS PSYCHOTIC....OR ADDICT TO ANY MEDICINE.................

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Wednesday, December 29, 2010

CASE: Grade IV liver Trauma (Once thought unsurvivable now .......Child Back to School......)^-www.drkeyurbhatt.in*

PAST HISTORY:

16 yrs boy with Blunt trauma abdomen --> liver laceration and hemoperitoneum (2 liters), hemodynamically unstable
Explored--> hemostasis tried....massive bleeding---packing done

remained on venti for 2 days...bleeding continue...>10 blood / products given...

PRESENTATION : Cont. bleeding even after packing..and Hemodynamic unstability (again more than 1.5 liter blood loss)

RE EXPLORED AFTER 48 hrs of previous surgery & hemostasis achieved...drains kept in Morrison's puch and pelvis.......gradually stabilized...Extubated after 6 critical  days....pelvic drain removed...started on Oral diet...

developed Biliary fistula from injured peripheral seg 7 of liver...Per cutaneous Malacot catheter placed in collection near seg 7...and drain removed....fistula localised.....discharged of hospital after 1 month stay...
ERC and stenting done...gradually fistula out put decreased.....unfortunately developed stent blockage and cholangitis...
Re admitted and stent changed with smaller caliber straight flap stent on Rt side...
External Fistula healed...and malacot removed....no residual collection....

Once thought unsurvivable now .......Child Back to School......

last MRI before episode of Cholangitis...
showing healing laceration and
compensatory hypertrophy of left lobe of Liver


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Friday, December 24, 2010

CASE: ANURSM LIGATED DOUBLY SURGICALLY...DELAYED HEMORRHAGE IN A CASE OF LIVER TRAUMA ? PSEUDOANURISM OF Rt hepatic artery^-www.drkeyurbhatt.in*

HISTORY:

30 YRS male
h/o fall and liver contusion/hematoma 26 days back in Rajasthan
USG : s/o no e/o free fluid and only liver hematoma.
Rx conservatively...........
remained well till 14 days ...sudden increase in pain----CECT was done : s/o 11 x10 cm size liver contusion
no free fluid....conservative Rx....in 3 days sudden fall in Hb. with gross haemoperitoneum, shock, anuria, and renal failure with Creat progressed to  6.2
Explored---- liver laceration and 2 liters of hemorrhage drained....no active bleeding....drains kept...Repair of laceration tried near GB fosa.

PRESENTATION :

post op pt remained stable...on day 2 developed biliary fistula draining frank bile in both drains later localized to sub hepatic drain 200 ml / day.

remained stable for 12 days and was walking / tolerating oral normal diet, normal urine out put with creat of 2.5

suddenly 2 liter of fresh bleeding in drain no hemoperitoneum. resucitation given again after 3 hrs massive bleeding of 2 liters....and hemoperitoneum as well.

DIAGNOSIS: Rt hepatic artery pseudo anurism was suspected...... as CECT/MRI OUT OF question in view of on going ARF and Pt in Shock....

Explored cholecystectomy and Rt hepatic artery ligated 1.5 liters of hemoperitoneum drained...and all bleeding secured...abdomen closed with drains and repair of liver laceration....IN HOPE OF STABILITY.....


now as expected pt is confirmed having Rt hepatic artery pseudoanurism...and the sad part is that anurism has now taken supply from left hepatic artery and is still getting filled.......its of 1 cm in size (quite big for a segmental hepatic branch..3 times bigger)....all radiological options are out of question in view of previous life saving attampt and ligation of RHA....will require formal resection / doubble ligation on both side of anurism surgically.....






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Wednesday, December 22, 2010

CASE: Flo reed abdominal TB with millions of tubercles and adhesive mid small bowel obstruction^-www.drkeyurbhatt.in*

24 yrs guy with CEREBRAL PALSY.
pain in abdomen with distension for 7 days and constipation
conservative Rx given for a week....distension increased with frank obstruction and features of early sepsis...

CECT : showed mid small bowel obstruction with ascitis...

on exploration....kink at jejuno ileal inter phase with grossly distended jejunal loops....adhesiolysis done....


oooooooohhhhhhhhh

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Monday, December 20, 2010

Clinical resistance to Imatinib (Gleevec)^-www.drkeyurbhatt.in*

Unfortunately, the majority of patients treated with imatinib mesylate will develop clinical resistance to this
agent and eventual progression of disease. Much research has been focused on the predictors and potential mechanisms of the development of recurrence to targeted therapy. A review of 934 patients with advanced GISTs treated with imatinib determined that patients who developed early resistance (defined as resistance within 3 months of initiating therapy) were more likely to have lung metastases without liver metastases, low hemoglobin, and a high granulocyte count . On the other hand, prognostic factors for the development of late resistance (after 3 months of initiating therapy) were found to be a high baseline granulocyte count, large size of tumor, and nongastric primary.


Because progression eventually develops in a significant number of patients with GISTs treated with imatinib,
additional targeted inhibitors have been evaluated for the treatment of these patients. As yet, only sunitinib has been approved by the US Food and Drug Administration (FDA) for patients with imatinib-resistance or imatinib-intolerance. Sunitinib is a multitargeted agent, an inhibitor of tyrosine kinase, of KIT and PDGFRA/B and of the vascular endothelial growth factor receptors (VEGFRs)-1, -2 and 3, FMS-like tyrosine kinase-3 (FLT3), colony stimulating factor 1 receptor (CSF-1R), and glial cell-line derived neurotrophic factor receptor.

In any patient with advanced GIST who underwent targeted therapy, the treating physician should periodically reassess the potential operability, after the completion of a certain course of imatinib or other form of therapy. 

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Gastrointestinal Stromal Tumors (GISTs): An Updated Experience^-www.drkeyurbhatt.in*

Review article: Dig Dis Sci (2010) 55:3315–3327



summary:


Gastrointestinal stromal tumors (GISTs) represent the most common mesenchymal tumors of the gastrointestinal tract. Over the last decade, GISTs have gained an extremeinterest, not only for surgeons but also for oncologists. The role of targeted therapy with tyrosine kinase inhibitors has revolutionized the care of these patients, and has made GISTs the paradigm for molecular cancer therapy. For patients with primary GISTs surgery is the treatment of choice. A combination of imatinib therapy and surgery may be effective in a subset of patients with metastatic or unresectable primary GISTs. Meanwhile, the advances in the understanding of the pathogenesis and treatment of these tumors may render feasible, in the near future, the advent of newer and more efficacious treatment options.

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Sunday, December 19, 2010

Clinical practice guidelines for gastrointestinal stromal tumor (GIST) in JAPAN^-www.drkeyurbhatt.in*

Int J Clin Oncol (2008) 13:416–430

The Japan Society of Clinical Oncology 2008


Toshirou Nishida · Seiichi Hirota · Akio Yanagisawa
Yoshinori Sugino · Manabu Minami
Yoshitaka Yamamura · Yoshihide Otani
Yasuhiro Shimada · Fumiaki Takahashi · Tetsuro Kubota

SPECIAL ARTICLE


Clinical practice guidelines for gastrointestinal stromal tumor (GIST)
in Japan: English version

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International guidelines for GIST management^-www.drkeyurbhatt.in*

ESMO (European Society of Medical Oncologists) and NCCN (National Comprehensive Cancer Network) recommend:

1. Available data confi rm the safety and effi cacy of Imatinib mesylate at 400 mg per day as the initial standard dose to achieve response induction.
2. Data have been provided that patients with exon 9 KIT mutations fare better in terms of progression-free survival on a higher dose level i.e. 800 mg daily, which is therefore standard treatment in this subgroup.
3. Th e standard approach in the case of tumour progression is to increase the Imatinib mesylate dose to 800 mg daily. Also patient non-compliance should be ruled out as a possible cause of tumour progression, as well as drug interactions with concomitant medications.
4. Treatment should be continued indefi nitely, since treatment interruption is generally followed by relatively rapid tumour progression in virtually all cases.

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Emergence of Imatinib (Glivec) Resistance^-www.drkeyurbhatt.in*

J Gastrointest Surg (2010) 14:557–561

Abstract
Introduction Gastrointestinal stromal tumors (GISTs) are the most common gastrointestinal mesenchymal tumors. The activating mutation in the KIT (c-kit; CD117) proto-oncogene with subsequent tyrosine kinase activation plays a central role in the pathogenesis of GIST. Tyrosine kinase inhibitors are an integral part of GIST therapy. Initial response to neoadjuvant imatinib can be expected in up to 70% of the patients, thus offering an opportunity to surgically treat those with locally advanced primary or recurrent GIST. This favorable response to imatinib, however, is plagued with development of secondary resistance during the course of therapy. .
Discussion Continued monitoring by a multidisciplinary team, including a surgeon, is vital for the success of neoadjuvant imatinib therapy for unresectable primary or recurrent GIST in the context of emergence of secondary resistance. As such, surgeons should participate in managing imatinib-treated GIST, as resection may become a viable curative option.  major oncologic resections can be safely performed in older persons when their performance status and comorbidities are carefully considered.

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Saturday, December 18, 2010

Surgically Managed Gastrointestinal Stromal Tumors: A Comparative and Prognostic Analysis^-www.drkeyurbhatt.in*

Presented in part at the annual meeting of the Society of Surgical Oncology, April 2007, Washington, DC.
Annals of Surgical Oncology 15(1):52–59


Background: Tyrosine kinase inhibitors have been shown to have marked clinical efficacy in patients with unresectable or metastatic gastrointestinal stromal tumors (GIST). We performed a comparative and prognostic analysis of our experience with surgically managed GIST to determine factors associated with adverse oncologic outcomes.
Methods: Oncologic outcomes of 191 patients with primary GIST surgically managed between 1978 and 2004 at a single institution were reviewed. Prognostic factors were analyzed by Cox analysis (hazard ratio [HR] and 95% confidence interval [95% CI]) and included age, sex, disease presentation (asymptomatic vs. symptomatic), tumor site (stomach, small bowel, colorectal), disease extent (localized vs. metastatic) and risk levels (high, intermediate, low, very-low) assigned on the basis of size and number of mitoses according to current National Institutes of Health recommendations. Primary end points were disease-free survival (DFS)  and disease-specific survival (DSS).
Results: A total of 186 patients (97%) had c-kit–positive GIST. There were 54% high, 22% intermediate, 18% low, and 8% very low risk GIST originating from the stomach (54%), small bowel (36%), and colon and rectum (10%). Median patient age was 65 (range, 13–91) years, and 108 subjects (57%) were male. Seventy-two percent of patients had symptomatic local disease, and 21% patients had synchronous metastases. Most (95%) underwent R0 resections of their primary tumor. Among 146 patients (76%) with localized disease at presentation undergoing R0 resection, the 5-year DFS was 65%. High-risk GIST (HR 12, 95% CI, 5–32, P < .0001), symptomatic presentation (HR 2.5, 95% CI, 1.1–6, P = .04), and GIST in the small bowel (HR 2.8, 95% CI, 1–5, P = .003) were independently associated with decreased DFS. After a median follow-up of 63 months among survivors, the 5-year DSS was 68%. High-risk disease (HR 14.3, 95% CI, 5–41, P < .0001), symptomatic presentation (HR 3.1, 95% CI, 1.2–7.9, P = .02), and GIST in the small bowel (2.6,3 95% CI, 1–5, P = .006) were independently associated with decreased DSS. Conclusions: High-risk GIST are associated with increased disease recurrence and decreased survival despite complete surgical resection. These patients should receive adjuvant therapy in the form of tyrosine kinase inhibitors

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Rectal GIST^-www.drkeyurbhatt.in*

World J Gastroenterol 2008 February 28; 14(8): 1302-1304

Rectal GISTs make up 0.1% of all tumours originating in the rectum


Since the incidence of rectal GIST is much lower than that of GIST in the stomach or small intestine,
the clinicopathological profiles of rectal GIST have not yet been accurately characterised, and there is therefore the tendency to validate the same prognostic factors for the latter as for such tumours at other sites, particularly gastric GIST.
A rate of ≤ 5 mitoses per 50 HPF is commonly used as a limit for a tumour with expected benign behaviour, and according to a large study, this can discriminate between benign and malignant tumours, especially gastric GIST.Tumours of 2 cm in diameter Transrectal ultrasound confirming a predominantly exophytic, heterogenous, hypoechoic submucosal mass (measuring 35 mm × 26 mm) on the
lateral left rectal wall. TC confirming the sonographic findings of the presence of a mass with a marked, irregular, eccentric thickening of the lateral left wall of the lower third of the rectum, but providing no evidence for either pelvic lymphadenopathy or distant metastasis. are generally expected to behave in a benign fashion.
Tumours of < 5 cm in diameter are associated with a better survival rate than those of 5 cm-10 cm in diameter, which in turn have a better prognosis than those of > 10 cm in diameter.


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Thursday, December 16, 2010

Imatinib where does it stays^-www.drkeyurbhatt.in*

The role of neoadjuvant therapy of Imatinib in GIST is under evaluation by the Radiation Therapy Oncology Group (RTOG) that will correlate molecular markers with clinical response as assessed by imaging and pathological evaluation of the resected specimen. The neoadjuvant therapy will be useful in patients with large-size tumor or location of the tumor at the site where resection is associated with the risk
of severe organ dysfunction where a negative margin is difficult to achieve. The aim of such a treatment is to
downsize the tumor for a less morbid surgical procedure. In addition, the use of Imatinib neoadjuvant therapy with or without an adjuvant treatment might help in controlling micrometastatic disease. Again, the duration and dose of Imatinib in the neoadjuvant setting are yet undecided. Less than 5% patients have complete clinical response to Imatinib.


Now the first line of treatment of the recurrent or 
metastatic GIST is Imatinib. The use of Imatinib mesylate 
in recurrent or metastatic GIST in prospective trial has 
shown response in 50% patients. 
Surgical resection of the localized GIST is the mainstay therapy, as a resection of tumor renders only a chance of cure


Gastrointestinal cancers symposium, San Francisco, 2004.



Imatinib adjuvant therapy
The use of Imatinib as an adjuvant therapy after the complete resection of primary GIST is under evaluation. The American College of Surgeons Oncology Group (ACOSOG) is conducting a phase-II prospective trial on patients of GIST.

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Gastrointestinal stromal tumor (GIST)^-www.drkeyurbhatt.in*

Chirurgia (Bucur). 2010 Jul-Aug;105(4):577-85.      Surgical Oncology (2008) 17, 129–138
Although their overall incidence is low, GISTs are distinctive subgroup of gastrointestinal mesenchymal tumors which express CD117 or platelet derived growth factor receptor alpha (PDGFRA). Considered as rare digestive cancers, tumors like schwannomas, neurofibromas, gastrointestinal leiomiomas are now reclassified as GIST based on immunohistochemistry studies. 
GIST are more frequent in stomach (40-70%), small bowel (20-40%), colon (5-15%), meanwhile locations such as mesentery, omentum, retro peritoneum in less of 5%. 10 GIST patients were surgically managed during 2004-2009. 5 gastric and 5 small bowel GIST. Most with symptomatic disease: palpable tumor, abdominal pain, anemia, fatigue, superior digestive hemorrhage or occlusion. Imagistic diagnosis consisted of: barium swallow, abdominal sonography, CT and PET-CT. 
Confirmation was made by hystopathological exam and immunohistochemistry. All patients had more or less wide surgical resections. For some patients there was also a specific adjuvant treatment. All patients survived after surgery. 
The principle of surgery for GIST is RO resection of the tumor. Tumor rupture or R1 resection of the primary tumor has a negative impact on disease free survival. Some patients (great volume tumors, R1 or R2 resection) had adjuvant treatment. Imatinib mesylate and derivates showed a significant improvement of recurrence free survival with one condition: permanent treatment. Surgery remains the mainstay of treatment in patients with localized, resectable GIST. Recurrence rate of 17-21% and 5 years survival rate of 48-70%, even in resectable GIST, impose an adjuvant treatment


A long-term follow-up is essential for all patients with GIST independent of a benign or malignant designation, since these tumors have an uncertain biological behavior. Although active post-operative surveillance program is important there is no consensus on a standard protocol for the follow-up of these patients. As most of the recurrences occur within the first 3–5 years, intense surveillance is required during this period


FOLLOW UP:


According to the National Comprehensive Cancer Network guidelines, contrast CT of
the abdomen and pelvis is recommended every 3–6 months for 3–5 years and then yearly [105]. The European Society of Medical Oncology guidelines stratify the surveillance based on the tumor size and mitoses [106]. Tumor size 45 cm and mitoses 45/HPF require contrast CT for every 3–4 months for 3 years followed by every 6 months for the next 2 years, and later yearly. Smaller size tumors (o5 cm) and lowmitotic count (o5/HPF) requires contrast CTevery 6 months for 5 years [106]. According to Novitsky et al. [6] most of the recurrence occurs during the first 2 years after surgical resection. They follow-up the patient with physical examination
every 3–4 months for 2 years, then every 6 months for the next 2 years, then yearly. Chest X-ray and abdominal CT scan and blood test were obtained yearly. Flexible upper endoscopy is performed at 6 months and 1-year postoperatively
and then annually for 2 years. PET scanning of abdomen, MR imaging, or chest CT scan is done if abnormalities are found in any of the surveillance studies.

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