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1.
Int J Colorectal Dis ; 29(12): 1445-51, 2014 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-25139370

RESUMO

Mesenteric fibromatosis is a rare, locally invasive, non-metastasizing type of intra-abdominal fibromatoses with a very high rate of recurrence. Because of rarity, these tumors pose a diagnostic and therapeutic challenge. These tumors may remain asymptomatic, but commonly present as a painless mass. There are no clear treatment guidelines. Surgical excision with wide margin is the preferred modality of treatment. Medical therapy is indicated for inoperable and recurrent tumors. There are several isolated case reports on mesenteric fibromatosis with different and unusual presentations and its complications. Though several studies have been published on extra-abdominal fibromatosis as well as on extramesenteric abdominal fibromatosis, but extensive research studies are still lacking on mesenteric fibromatosis. The aim of this article is to present current knowledge on mesenteric fibromatosis, our experience of four cases, and comprehensive review of available literature.


Assuntos
Fibromatose Abdominal , Mesentério , Neoplasias Peritoneais , Diagnóstico Diferencial , Diagnóstico por Imagem , Fibromatose Abdominal/diagnóstico , Fibromatose Abdominal/etiologia , Fibromatose Abdominal/terapia , Humanos , Mesentério/patologia , Recidiva Local de Neoplasia/terapia , Neoplasias Peritoneais/diagnóstico , Neoplasias Peritoneais/etiologia , Neoplasias Peritoneais/terapia
2.
Indian J Pathol Microbiol ; 67(1): 2-9, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38358181

RESUMO

Ovarian tuberculosis is a rare entity with non-specific clinical manifestations, difficult diagnosis, and specific medical management. Ovarian involvement in tuberculosis (TB) may occur in two forms, namely, perioophoritis and oophoritis. The constitutional symptoms of tuberculosis such as anorexia, weight loss, night sweats, and evening rise in temperature have been reported in up to 45% of patients. Misdiagnosis and delayed diagnosis are common. A direct histopathological demonstration is the best diagnostic modality. Fine needle aspiration cytology (FNAC) is the study of choice and polymerase chain reaction (PCR) assay increases its sensitivity. The standard short-course antituberculous for 6 months is recommended for isolated ovarian tuberculosis and for widespread disease, 12 months of therapy is recommended. Surgery is reserved for failure of medical therapy and abscess formation. There are many studies on genito-urinary tuberculosis but a detailed study defining diagnostic studies and management guidelines is still lacking. This article aims to present and share a review of the English-language literature on ovarian tuberculosis to gain a better understanding of etiopathogenesis and diagnostic methods and to provide guidelines for its management.


Assuntos
Tuberculose , Feminino , Humanos , Tuberculose/diagnóstico , Biópsia por Agulha Fina , Citodiagnóstico , Reação em Cadeia da Polimerase/métodos
3.
Indian J Otolaryngol Head Neck Surg ; 75(2): 1302-1310, 2023 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-37275094

RESUMO

Objective: Thyroid tuberculosis has non-specific clinical presentation, difficult diagnosis and specific medical management. The aim of this article is to present and share a review of the English-language literature on thyroid tuberculosis in order to gain a better understanding of diagnostic methods and provide guidelines for its management and to present our experience of three cases. Methods: The systematic search of the literature was performed on Pubmed and Medline from 1950 to 2019 according to the Preferred Reporting Items for Systematic Reviews and Meta-analysis (PRISMA) statement. Results: We retrieved 13 manuscripts meeting our criteria from the search. There were 7 case series, and 6 manuscripts with review of the literature. Conclusion: Direct histopathological demonstration is the best diagnostic modality. FNAC is the study of choice and PCR assay increases its sensitivity. The standard short course ATT for 6 months is recommended for isolated thyroid TB and for widespread disease, 12 months therapy is recommended. Surgery is reserved for failure of medical therapy and abscess formation.

4.
Indian J Surg Oncol ; 14(1): 42-47, 2023 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-36891431

RESUMO

Gastrosplenic fistula is an uncommon manifestation of malignancy of the stomach and spleen. The aim of this study is to present our 10-year experience on gastrosplenic fistula secondary to malignant etiology. Endoscopy, imaging, and histopathology records of all the patients with gastric and splenic malignant pathologies were reviewed retrospectively. The protocol was approved by the ethical review board of the institute. Descriptive statistics were used to summarize the data. A total of 5 cases were found to have gastrosplenic fistula. Of these 5 cases, 2 were due to large B cell lymphoma of the spleen, 1 was secondary to Hodgkin's lymphoma of the stomach, 1 case was due to diffuse large B cell non-Hodgkin's lymphoma of the stomach, and 1 patient was secondary to gastric adenocarcinoma. Gastrosplenic fistula is an exceptionally rare complication of gastrointestinal malignancy. Lymphoma of the spleen is the commonest cause while gastric adenocarcinoma causing gastrosplenic fistula is extremely rare. Most cases occur spontaneously.

5.
Indian J Surg Oncol ; 13(3): 652-660, 2022 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-36187537

RESUMO

Gastrosplenic fistula is an unusual complication of benign as well as malignant gastric and splenic pathologies. This pathology acquires an important clinical significance due to its rare association with life-threatening upper gastrointestinal haemorrhage. The aim of this article is to review the English-language literature in order to gain a better understanding of etiological factors, diagnostic evaluation, and management of gastrosplenic fistula. The systematic search of the literature was performed on PubMed and MEDLINE from January 1950 to September 2020 according to the Preferred Reporting Items for Systematic Reviews and Meta-analysis (PRISMA) statement. We retrieved 44 articles matching our selection criteria from the search. There were 3 case series, 37 case reports, and 4 review of the literature. In our appraisal of articles published in PUBMED, a total of 36 cases of malignant and 10 cases of benign gastrosplenic fistula could be identified. Gastrosplenic fistula is an exceptional complication of malignancies of the gastrointestinal tract. Lymphomas particularly arising from the spleen are the commonest cause. Gastric adenocarcinoma causing GSF is extremely rare. Most cases occur spontaneously, but at times, it can be secondary to tumour necrosis following chemotherapy.

6.
Indian J Surg Oncol ; 13(4): 765-775, 2022 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-36687233

RESUMO

Solid-pseudopapillary neoplasm (SPN) is a variety of solid and cystic tumors of the pancreas. It was first described by Frantz in 1959. It is an unusual form of pancreatic carcinoma, with unknown etiopathogenesis, which accounts for about 0.17 to 2.7% of all pancreatic tumors. Here, we are describing 5 cases of pancreatic solid pseudopapillary neoplasm, out of 180 pancreatic tumors, operated in our institution in the 5-year period (2015-2020). Also, we have reviewed all available case series (from 2006 to 2020) in the literature, of pancreatic pseudopapillary neoplasm, for demographic information, etiopathogenesis, diagnosis, and extent of operation to establish the optimal management of this condition. Retrospective analysis of pancreatic tumors was carried out from February 2015 to January 2020. A total of 180 patients underwent pancreatic resection in this period for pancreatic tumor, out of which, the solid pseudopapillary neoplasm was confirmed in 5 cases (2.76%). Among these 5 cases, 4 cases (80%) were female and one (20%) male, with age group range from 14 to 45 years (mean age - 28 years). Abdominal pain was the most frequent presenting symptom (60%). Mean tumor diameter was 6.9 cm (range, 2-18 cm). Two patients were diagnosed preoperatively by CECT and MRI findings, and three patients were diagnosed preoperatively by percutaneous/USG-guided and CT-guided FNA cytology. Two patients underwent pancreatoduodenectomy; one patient underwent enucleation; and two patients underwent spleen preserving distal pancreatectomy. Four patients are alive and on regular follow-up, while one patient died on the 5th post-operative day due to post-operative sepsis.

7.
Indian J Otolaryngol Head Neck Surg ; 74(Suppl 3): 5910-5920, 2022 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-36742928

RESUMO

Involvement of esophagus with tuberculous infection is a rare form of extrapulmonary tuberculosis. Secondary esophageal tuberculosis is much more common than primary TB. The most common source of secondary esophageal involvement is tuberculous mediastinal lymphadenitis. Esophageal tuberculosis mimics carcinoma esophagus. Clinical features are same and it is difficult on imaging studies also to differentiate the two pathologies. Misdiagnosis is common. The disease is medically curable; therefore, it is essential to make all efforts to diagnose the pathology with non-surgical diagnostic modalities in suspected cases so as to save patients from the trauma of major surgical resection. Surgical intervention is indicated for failed medical therapy and complications. A total of 133 cases of esophageal TB have been reported till date. The authors encountered 4 cases of esophageal TB between April 2011 and March 2019. The aim of this article is to present our data and to provide comprehensive review of the available literature on this pathology in order to gain a better understanding of diagnostic methods and provide guidelines for the diagnosis and management of esophageal TB.

8.
Indian J Otolaryngol Head Neck Surg ; 74(Suppl 2): 2319-2323, 2022 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-36452638

RESUMO

The tuberculous infection of major salivary glands is rare because they are relatively resistant to tubercle bacilli. The aim of this study is to present our 13 year experience on major salivary gland tuberculosis. The clinical, histopathological, microbiological and imaging records of 9 cases of major salivary gland tuberculosis were reviewed retrospectively. The cases diagnosed with either microbiological culture and/or polymerase chain reaction assay and/or Histopathological examination were included in the study. Descriptive statistics were used to summarize the data. There were 9 cases with tuberculous involvement of major salivary glands; parotid (n = 5), submandibular (n = 3), sublingual (n = 1). The most common local symptom was painless swelling present in 8 cases and constitutional symptoms were present in all the cases. The previous history of TB was present in 5 out of 9 cases. Surgical intervention was done in 2 cases of parotid TB for diagnostic purpose and therapeutic intervention was required in 1 patient. Patients responded well to medical therapy. Two patients with disseminated disease died. The major salivary gland is rare in both immunocompetent and immunocompromised patients. Diagnosis is difficult and requires high degree of suspicion especially in isolated cases. Medical management yields satisfactory results.

9.
Indian J Otolaryngol Head Neck Surg ; 74(Suppl 2): 1910-1916, 2022 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-36452728

RESUMO

Submandibular salivary gland is an uncommon and unusual site for tuberculosis. It is a rare extrapulmonary manifestation of a common infection caused by Mycobacterium tubercle bacillus. Submandibular gland TB can be due to primary or secondary infection. The clinical features are non-specific and depend on its manifestations. A delay in diagnosis is common because of the rarity of disease. Direct histological examination is the best method of diagnosis. As the disease is curable with antituberculous drugs; treatment should not be withheld in suspected cases until the diagnosis is confirmed. Surgery is indicated for non-responsive disease and complications. Till date, only 15 cases have been reported in the literature. First case was reported in the year 1990. The authors encountered three cases in last 10 years. The aims of this study are to review clinical presentation, epidemiological features, diagnostic methods and to provide our data and guidelines for optimum management of this rare pathology.

10.
Indian J Tuberc ; 69(3): 268-276, 2022 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-35760476

RESUMO

Rectal tuberculosis is an uncommon entity. It has unique epidemiological features, specific medical treatment and surgery is rarely indicated. The first case of rectal tuberculosis was reported in 1957. Delayed diagnosis is common. Patients who develop rectal tuberculosis have been reported to have some risk factors or associated comorbid conditions or pathologies with some form of abnormal host-defence mechanism such as acquired immunodeficiency syndrome, complement deficiency. Rectal tuberculosis has been reported to be more common in females as compared to males. Haematochezia is the most common presenting symptom. The definite diagnosis requires demonstration of Mycobacterium tuberculosis bacillus on histopathologic examination. Once a correct diagnosis has been made, rectal tuberculosis is curable with antituberculous treatment. Surgery is indicated for diagnostic dilemmas, non-responsive disease and complications. The authors encountered 3 cases in the last 10 years. The aim of this study is to provide our data on this rare disease and to review the reported literature comprehensively so as to provide guidelines for diagnosis and management.


Assuntos
Síndrome da Imunodeficiência Adquirida , Mycobacterium tuberculosis , Tuberculose dos Linfonodos , Feminino , Humanos , Masculino
11.
Surg J (N Y) ; 8(1): e92-e97, 2022 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-35252566

RESUMO

Addison's disease was first described by Thomas Addison in 1855. He demonstrated the destruction of bilateral adrenal gland by tuberculosis (TB) in six patients. Since then, the incidence of TB has declined in the Western world, but in developing countries, it is still the most common cause of adrenal insufficiency. Because of the introduction of antituberculous chemotherapy, the incidence of adrenal TB has been declined in the past decades. The most common symptoms are nonspecific; therefore, diagnosis is often delayed, and patients may first present with a life-threatening adrenal crisis. The most commonly identified organism for adrenal failure in adrenal TB is Mycobacterium tuberculosis infection. Adrenal TB involves bilateral adrenal glands more frequently than unilateral glands. Computed tomography (CT) scan and magnetic resonance imaging (MRI) are useful investigations to differentiate between tuberculous Addison's disease and the other causes of adrenal insufficiency. In CT scans or MRI, features of adrenal TB are bilateral adrenal enlargement and peripheral rim enhancement with or without calcifications. Antituberculous drugs, biochemical monitoring of adrenal function, and steroid therapy are essential for the management of adrenal TB and adrenal insufficiency. Here, we describe a case of adrenal TB with abscess formation followed by a review of the current literature of adrenal TB for better diagnosis and management of this condition.

12.
Indian J Surg Oncol ; 12(1): 61-66, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-33814833

RESUMO

Coexistence of carcinoma colon and tuberculosis is rare. The aim of this study is to present our 12-year experience on colonic carcinoma with coexisting colonic TB. Histopathology and imaging records of 189 patients of colonic carcinoma patients who underwent treatment in surgical unit 6 in our institute between January 2006 and December 2017 were reviewed. In 7 patients, histopathology and/or imaging studies were suggestive of coexistence of colonic carcinoma and tuberculosis. Fifteen cases of colonic tuberculosis were also reviewed. Descriptive statistics were used to summarize the data. Colonoscopic biopsy was suggestive of only malignant lesion in 6 cases and coexistence of TB and carcinoma in 1 case. The duration of symptoms spanned between 2 and 7 weeks. Anorexia and weight loss were the predominant symptoms. Colonoscopic biopsy was suggestive of only malignant lesion in 6 cases and coexistence of TB and carcinoma in 1 case. The morbidity was low and there was no surgery associated mortality. Clinical awareness and advancement in diagnostic studies and improvement in management strategies may guide and lead to early diagnosis, optimum therapeutic guidelines and thus improved outcome.

13.
Indian J Tuberc ; 68(2): 272-278, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-33845964

RESUMO

Thyroid tuberculosis is a rare disease. Its incidence is low even in countries where prevalence of pulmonary tuberculosis is high (0.1-0.4%). In literature, there are only a few cases which were diagnosed as thyroid tuberculosis. It can be explained by a high resistance of the thyroid gland to infectious processes. However, the prevalence of tuberculosis has increased worldwide and thyroid involvement can be a primary manifestation of the disease. The incidence of extrapulmonary tuberculosis has been showing a progressive increase in the recent years(Barnes and Weatherstone, 1979). The most frequent clinical presentation is a solitary thyroid nodule that may present as a cystic nodule. It may also present as thyroid abscess with pain, fever and other non-specific signs and symptoms. ATT results in complete cure therefore it is important to differentiate it from other form of thyroiditis. Patients are usually euthyroid, but cases of hypothyroidism and hyperthyroidism are described. For accurate diagnosis of thyroid tuberculosis, clinical and radiological features are nonspecific and histological examination is required for confirmation of diagnosis. PCR may help in diagnosis. The authors encounter 3 cases of thyroid tuberculosis in last 5 year which are described in this article. The aim of this study is to review all the cases published in literature to describe clinical presentation, appropriate diagnostic method and possible treatment options of the disease.


Assuntos
Glândula Tireoide , Tuberculose/diagnóstico , Humanos , Tireoidectomia , Tuberculose/cirurgia
14.
Pol Przegl Chir ; 93(5): 1-5, 2021 Jun 25.
Artigo em Inglês | MEDLINE | ID: mdl-34552028

RESUMO

OBJECTIVE: The aim of this study was to compare the post-operative effects of closed incision negative pressure wound therapy with conventional dressing in emergency laparotomy. METHODS: This study was conducted from 1st November 2018 to 31st March 2020 in ABVIMS & Dr. R.M.L. Hospital, New Delhi. The potential candidates for the study were patients of 18 years and above who were admitted in surgical emergency and underwent emergency laparotomy by a midline incision. Fifty random patients were alternatively allotted to group A (25 patients) and group B (25 patients). In the patients of group A, closed incision negative pressure wound therapy (ciNPWT) was applied on midline closed wound after an exploratory laparotomy procedure. The patients in group B, standard dry gauze dressing was done. RESULTS: The mean age of patients in group A and group B were 46.76±12.20 and 41.96±8.33 years, respectively (p-value-0.11). The wound infection was present in 12% of cases in group A and 32% in group B, but when we calculate the p-value, it was found to be statistically non-significant (p-value-0.08). Similarly, seroma formation and wound dehiscence were found less in group A as compared to group B but not reached up to a statistically significant limit (p-value 0.55 and 0.38 respectively). The frequency of dressing change was 1-2 per week in 92% of cases in group A while it was 3-4 per week in 68% of cases in group B. The mean time of the frequency of dressing change was 1.24±0.72 per week and 4.28±1.90 per week in both the groups respectively (p-value <0.001). There was no significant (p>0.05) difference in the duration of hospital stay between group A (mean hospital stay 8.20±2.34 days) and group B (mean hospital stay 8.21±3.37 days). CONCLUSION: Closed incision negative pressure wound therapy has no advantages over conventional dressing in terms of post-operative complications and hospital stay. However, it reduces the frequency of dressing change significantly, which reduces the mental stress of the patient and the burden of changing daily dressing.


Assuntos
Tratamento de Ferimentos com Pressão Negativa , Adulto , Bandagens , Humanos , Laparotomia , Pessoa de Meia-Idade , Deiscência da Ferida Operatória , Infecção da Ferida Cirúrgica/prevenção & controle
15.
Indian J Tuberc ; 66(3): 411-417, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31439189

RESUMO

Tuberculosis of the stomach is an extremely rare manifestation of Mycobacterium tuberculosis infection and mimics gastric carcinoma in its presentation. Most of our knowledge about this rare disease comes from case reports and there are only a few case series published on this disease and thus the majority of the part remains uncovered. Diagnosis is made commonly only after a major surgery. Endoscopy and guided biopsy are the diagnostic modality of choice. Surgery is indicated in cases which present with complications. Patients respond well to antituberculous therapy. The authors encountered 4 cases of gastric tuberculosis over 5 years. This study summarises the available literature and gives comprehensive update on this rare disease.


Assuntos
Gastropatias/diagnóstico , Tuberculose Gastrointestinal/diagnóstico , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Antituberculosos/uso terapêutico , Terapia Combinada , Diagnóstico Diferencial , Feminino , Gastroscopia , Humanos , Masculino , Pessoa de Meia-Idade , Gastropatias/tratamento farmacológico , Gastropatias/patologia , Gastropatias/cirurgia , Tuberculose Gastrointestinal/tratamento farmacológico , Tuberculose Gastrointestinal/patologia , Tuberculose Gastrointestinal/cirurgia , Adulto Jovem
17.
Indian J Tuberc ; 64(3): 161-166, 2017 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-28709482

RESUMO

Parotid gland tuberculosis is an uncommon manifestation of one of the most common infections even in the developing countries, caused by Mycobacterium tuberculosis. There are no specific symptoms or clinical signs of parotid tuberculosis, and such an infection most commonly presents as a slow growing painless parotid mass. Because of its rarity, tuberculosis of parotid gland is often mistaken for a malignant growth, and it most commonly gets diagnosed after superficial protidectomy. Complete cure is possible with standard antituberculous therapy. Most of our knowledge about this rare entity comes from case reports and short case series. The authors encountered three cases of parotid tuberculosis in the last 10 years. This article aims at presenting a comprehensive review of all the available literature and thus providing detailed information and an update on parotid tuberculosis and our experience of three cases.


Assuntos
Doenças Parotídeas/diagnóstico , Doenças Parotídeas/microbiologia , Tuberculose/complicações , Tuberculose/diagnóstico , Biópsia por Agulha Fina , Humanos , Doenças Parotídeas/terapia , Glândula Parótida/patologia , Tomografia Computadorizada por Raios X , Tuberculose/terapia , Ultrassonografia
18.
Indian J Tuberc ; 63(4): 245-250, 2016 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-27998497

RESUMO

AIM/OBJECTIVE: India accounts for the highest tuberculosis burden in the world, and abdominal tuberculosis has been an endemic surgical and gastroenterological problem. Aim of this study is to present our two decades experience on abdominal (gastrointestinal) tuberculosis. METHOD: 756 patients, who received standard antituberculous treatment with or without surgical treatment with the diagnosis of abdominal tuberculosis from January 1996 and May 2014, were reviewed retrospectively. On the basis of clinical presentation, four groups of clinical presentation were identified and various diagnostic measures used in different groups were studied. Numeric values were determined as percent or mean±standard deviation. Kruskal-Wallis test was used for quantitative results and chi-square test was used for qualitative results between groups. p value of less than 0.05 was considered to indicate the statistical significance. RESULTS: The duration of symptoms was variable in this study. Out of 756 patients, 64 patients gave definite past history of tuberculosis. Most of the patients in the acute pain abdomen group required surgery while most patients in chronic pain group responded well to medical management. There was significant difference in mortality among the four groups (p=0.025). CONCLUSION: Prognosis seems significantly related to the severity of disease, with graver prognosis and less symptomatic improvement in more seriously ill presentations.


Assuntos
Abdome , Tuberculose Gastrointestinal/complicações , Tuberculose Gastrointestinal/diagnóstico , Abdome Agudo/etiologia , Dor Abdominal/etiologia , Coleta de Dados , Humanos , Índia , Prognóstico , Estudos Retrospectivos , Índice de Gravidade de Doença , Tuberculose , Tuberculose Gastrointestinal/terapia
19.
Ann Gastroenterol ; 28(1): 49-54, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-25608706

RESUMO

Inflammatory myofibroblastic tumors (IMTs), also known as inflammatory pseudotumors and inflammatory fibrosarcomas, are uncommon mesenchymal tumors composed of myofibroblastic spindle cells admixed with lymphocytes, plasma cells and eosinophils. Once thought to be reactive, these lesions are now considered to be neoplastic. These tumors can occur throughout the body, most commonly in the lung, mesentery and omentum. Patients commonly present with painless abdominal mass or with intestinal obstruction. IMTs may be multicentric, have a high local recurrence rate and may metastasize in rare cases. The lesions show wide variability in their histologic features and cellularity, and marked inflammatory infiltration, predominantly of plasmatocytes and lymphocytes, and occasionally neutrophils and eosinophils. Anaplastic lymphoma kinase (ALK) rearrangements and/or ALK1 and p80 immunoreactivity are reported in 33-67% of the tumors. Owing to the rarity of these lesions, there are no specific imaging findings that distinguish IMTs from other mesenteric masses. Complete surgical resection is the treatment of choice. Local recurrence rates are high, and re-excision is the preferred therapy for local recurrences. ALK-positive tumors show good response to ALK inhibitors. Current knowledge and comprehensive review of the available literature on IMTs is herein presented.

20.
Indian J Surg ; 77(3): 226-31, 2015 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-26246707

RESUMO

Pancreatic acinar cell carcinoma is a rare tumour, accounting for only about 1 % of all pancreatic tumours. The long-term survival for patients with acinar cell carcinoma is significantly better than the long-term survival of patients with pancreatic adenocarcinoma. As no large series of patients with acinar cell carcinomas exist, our understanding of this disease comes mainly from small case series and case reports. Aggressive surgical resection with negative margins is associated with long-term survival in these more favourable pancreatic cancers. There are no clear treatment guidelines for patients in whom complete surgical resection with curative intent is not possible. Acinar cell carcinomas are chemoresponsive to agents that have activity against pancreatic adenocarcinomas and colorectal carcinomas because of the shared genetic alterations between these cancers. The role of neoadjuvant or adjuvant chemoradiotherapy remains unproven. The aim of this article is to present current knowledge on acinar cell carcinoma and comprehensive review of available literature.

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