Paraneoplastic myopathy-related rhabdomyolysis and pancreatic cancer:A case report and review of the literature

BACKGROUND Rhabdomyolysis is a life-threatening condition,often leading to progressive renal failure and death.It is caused by destruction of skeletal muscle and the release of myoglobin and other intracellular contents into the circulation.The most frequent cause of this condition is“crush syndrome”,although several others have been described and paraneoplastic inflammatory myopathies associated with various types of cancer are repeatedly reported.CASE SUMMARY We describe a rare case of a patient with pancreatic cancer who developed rhabdomyolysis early on,possibly due to paraneoplastic myositis leading to acute renal failure and eventually to rapid death.A 78-year-old Caucasian woman was referred to our hospital for obstructive jaundice and weight loss due to a lesion in the pancreatic head.She presented increasingly severe renal insufficiency with anuria,a dramatic increase in creatine phosphokinase(36000 U/L,n.v.20-180 U/L)and myoglobin(>120000μg/L,n.v.12-70μg/L).On clinical examination,the patient showed increasing pain in the lower limbs associated with muscle weakness which was severe enough to immobilize her.Paraneoplastic myopathy linked to the malignant lesion of the pancreatic head was suspected.The patient was treated with hemodialysis and intravenous methylprednisolone.Despite all the efforts to prepare the patient for surgery,her general condition rapidly deteriorated and she eventually died 30 d after hospital admission.CONCLUSION The possible causes of rhabdomyolysis in this patient with pancreatic cancer are discussed,the development of paraneoplastic myopathy being the most likely.Clinicians should bear in mind that these syndromes may become clinically manifest at any stage of the cancer course and their early diagnosis and treatment could improve the patient’s prognosis.

Aggressive behaviour of solid-pseudopapillary tumor of the pancreas in adults:A case report and review of the literature

Solid-pseudopapillary tumor (SPT) is a rare neoplasm of the pancreas that usually occurs in young females. It is generally considered a low-grade malignant tumor that can remain asymptomatic for several years. The occurrence of infiltrating varieties of SPT is around 10%-15%. Between 1986 and 2006, 282 cystic tumors of the pancreas were observed. Among them a SPT was diagnosed in 8 patients (2.8%) with only one infiltrating variety. This was diagnosed in a 49-year-old female 13 years after the sonographic evidence of a small pancreatic cystic lesion interpreted as a pseudocyst. The tumor invaded a long segment of the portal- mesenteric vein confluence, and was removed with a total pancreatectomy, resection of the portal vein and reconstruction with the internal jugular vein. Histological examination confirmed the R-0 resection of the primary SPT, although a vascular invasion was demonstrated. The postoperative course was uneventful, but 32 mo after surgery the patient experienced diffuse liver metastases. Chemotherapy with different drugs was started. The patient is alive and symptom-free, with stable disease, 75 mo after surgery. Twenty-five patients with invasion of the portal vein and/or of mesenteric vessels were retrieved from the literature, 16 recent patients with tumor relapse after potentially curative resection were also retrieved. The best treatment remains a radical resection whenever possible, even in locally advanced or metastatic disease. The role of chemotherapy, and/or radiotherapy, is still to be defined.

Metastatic tumors to the pancreas: The role of surgery

Pancreatic metastases from other primary malignancies are a rare entity. By far, the most common primary cancer site resulting in an isolated pancreatic metastasis is the kidney, followed by colorectal cancer, melanoma, breast cancer, lung carcinoma and sarcoma. Only few data on the surgical outcome of pancreatic resections performed for metastases from other primary tumor have been published, and there are no guidelines to address the surgical treatment for these patients. In this study, we performed a review of the published literature, focusing on the early and long-term results of surgery for the most frequent primary tumors metastasizing to the pancreas. Results for the Literature's analysis show that in last years an increasing number of surgical resections have been performed in selected patients with limited pancreatic disease. Pancreatic resection for metastatic disease can be performed with acceptable mortality and morbidity rates. The usefulness of pancreatic resection is mainly linked to the biology of the primary tumor metastasizing to the pancreas. The benefit of metastasectomy in terms of patient survival has been observed for metastases from renal cell cancer, while for other primary tumors, such as lung and breast cancers, the role of surgery is mainly palliative.

Parenchyma-sparing pancreatectomies for benign or border-line tumors of the pancreas

Standard pancreatic resections, such as pancreaticoduodenectomy, distal pancreatectomy, or total pancreatectomy, result in an important loss of normal pancreatic parenchyma and may cause impairment of exocrine and endocrine function. Whilst these procedures are mandatory for malignant tumors, they seem to be too extensive for benign or border-line tumors, especially in patients with a long life expectancy. In recent years, there has been a growing interest in parenchyma-sparing pancreatic surgery with the aim of achieving better functional results without compromising oncological radicality in patients with benign, border-line or low-grade malignant tumors. Several limited resections have been introduced for isolated or multiple pancreatic lesions, depending on the location of the tumor: central pancreatectomy, duodenum-preserving pancreatic head resection with or without segmental duodenectomy, inferior head resection, dorsal pancreatectomy, excavation of the pancreatic head, middle-preserving pancreatectomy, and other multiple segmental resections. All these procedures are technically feasible in experienced hands,with very low mortality, although with high morbidity rate when compared to standard procedures. Pancreatic endocrine and exocrine function is better preserved with good quality of life in most of the patients, and tumor recurrence is uncommon. Careful patient selection and expertise in pancreatic surgery are crucial to achieve the best results.

Para-aortic node involvement is not an independent predictor of survival after resection for pancreatic cancer

To analyze the importance of para-aortic node status in a series of patients who underwent pancreaticoduodenectomy (PD) in a single Institution. METHODSBetween January 2000 and December 2012, 151 patients underwent PD with para-aortic node dissection for pancreatic adenocarcinoma in our Institution. Patients were divided into two groups: patients with negative PALNs (PALNs-), and patients with metastatic PALNs (PALNs+). Pathologic factors, including stage, nodal status, number of positive nodes and lymph node ratio, invasion of para-aortic nodes, tumor’s grading, and radicality of resection were studied by univariate and multivariate analysis. Survival curves were constructed with Kaplan-Meier method and compared with Log-rank test: significance was considered as P < 0.05. RESULTSA total of 107 patients (74%) had nodal metastases. Median number of pathologically assessed lymph nodes was 26 (range 14-63). Twenty-five patients (16.5%) had para-aortic lymph node involvement. Thirty-three patients (23%) underwent R1 pancreatic resection. One-hundred forty-one patients recurred and died for tumor recurrence, one is alive with recurrence, and 9 are alive and free of disease. Overall survival was significantly influenced by grading (P = 0.0001), radicality of resection (P = 0.001), stage (P = 0.03), lymph node status (P = 0.04), para-aortic nodes metastases (P = 0.02). Multivariate analysis showed that grading was an independent prognostic factor for overall survival (P = 0.0001), while grading (P = 0.0001) and radicality of resection (P = 0.01) were prognostic parameters for disease-free survival. Number of metastatic nodes, node ratio, and para-aortic nodes involvement were not independent predictors of disease-free and overall survival. CONCLUSIONIn this experience, lymph node status and para-aortic node metastases were associated with poor survival at univariate analysis, but they were not independent prognostic factors.

Pancreatic resection in very elderly patients:A critical analysis of existing evidence

The aging of the population results in a rise of number of elderly patients (aged 80 years and older) with pancreatic or periampullary cancer, and more pancreatectomies could eventually be performed in such complex patients. However, early and long-term results after pancreatic resection in octogenarians are still controversial, and may trouble the surgeon when approaching this type of population. Evaluation of reported experiences shows that for almost all Authors, pancreatectomy can be performed safely in elderly population, although overall morbidity and mortality rates were 34.9% and 13.2% respectively, with a mean length of hospital stay of 18 d. These features appear higher in older patients compared to the younger counterpart. Less than 50% of patients underwent adjuvant therapy after operation. Long-term survival is reported not significantly different in aged 80 years and older patients, with a median overall survival time of 17.6 mo. The quality of life after pancreatic resection is only sporadically evaluated but, when considered, it highlights the need of health facility service after operation for these &ldquo;frail&rdquo; patients. Prospective studies on the quality of life of pancreatectomized octogenarians are welcome. Proper selection of patients, geriatric assessment with multidisciplinary approach, centralization of pancreatic surgery in high-volume centres and rehabilitation programs after surgery appear to be crucial points in order to improve surgical treatments of pancreatic tumors in very elderly patients.

Adrenal metastasis from endometrial cancer: A case report

BACKGROUND Metastases to adrenal glands originate principally from lung, breast, or gastrointestinal cancers, followed by malignant melanoma and thyroid neoplasms. We present an unusual case of uterine cancer metastasizing to the adrenal glands with a review of the English literature on the management of this rare disease. CASE SUMMARY A 53-year-old Caucasian woman with a history of endometrial cancer (grade 2;International Federation of Gynecology and Obstetrics III A) was hospitalized in November 2017 for a left adrenal mass found on a follow-up computed tomography scan 3 years after her gynecological surgery. Laboratory test results were normal. A laparoscopic left adrenalectomy was performed. The postoperative course was uneventful, and no chemotherapy was administered. The pathological report confirmed an adrenal endometrioid metastasis. At 36 mo of follow-up, the patient is alive and well, with no evidence of recurrent disease. A literature review identified only 11 previously-published cases of adrenal metastases from uterine cancer. CONCLUSION Adrenal metastasis from uterine cancer is very rare. Laparoscopic adrenalectomy may be an effective treatment in selected cases of localized adrenal metastasis.

Staging chronic pancreatitis with exocrine function tests: Are we better?

Chronic pancreatitis(CP) is an inflammatory disease of the pancreas evolving in progressive fibrotic disruption of the gland with exocrine and endocrine pancreatic insufficiency. Although imaging features of CP are well known, their correlation with exocrine pancreatic function tests are not obvious, particularly in the early stage of the disease. There are many clinical classification of CP, all suggested for better distinguish and manage different forms based on etiological and clinical factors, and severity of the disease. Recently, a new classification of CP has been suggested: the M-ANNHEIM multiple risk factor classification that includes etiology, stage classification and degree of clinical severity. However, more accurate determination of clinical severity of CP requires a correct determination of exocrine function of the pancreas and fecal fat excretion. Recently, Kamath et al demonstrated that the evaluation of exocrine pancreatic function by acid steatocrit and fecal elastase-1(EF-1) was helpful, but EF-1 was able to detect exocrine pancreatic insufficiency in more patients, upgrading some patients in higher stage of disease according to M-ANNHEIM classification. So, EF-1 is a more accurate test to determine exocrine pancreatic insufficiency and to stage chronic pancreatitis in the M-ANNHEIM classification. On the contrary, EF-1 determination shows low sensitivity in detecting exocrine pancreatic insufficiency in early stage of the disease.

Multimodality treatment of recurrent pancreatic cancer: Mith or reality?

Pancreatic adenocarcinoma is the fourth cause of cancerrelated death in the United States. Surgery is the only potentially curative treatment, but most patients present at diagnosis with unresectable or metastatic disease. Moreover, even with an R0 resection, the majority of patients will die of disease recurrence. Most recurrencesoccur in the first 2-year after pancreatic resection, and are commonly located in the abdomen, even if distant metastases can occur. Recurrent pancreatic adenocarcinoma remains a significant therapeutic challenge, due to the limited role of surgery and radiochemotherapy. Surgical management of recurrence is usually unreliable because tumor relapse typically presents as a technically unresectable, or as multifocal disease with an aggressive growth. Therefore, treatment of patients with recurrent pancreatic adenocarcinoma has historically been limited to palliative chemotherapy or supportive care. Only few data are available in the Literature about this issue, even if in recent years more studies have been published to determine whether treatment after recurrence have any effect on patients outcome. Recent therapeutic advances have demonstrated the potential to improve survival in selected patients who had undergone resection for pancreatic cancer. Multimodality management of recurrent pancreatic carcinoma may lead to better survival and quality of life in a small but significant percentage of patients; however, more and larger studies are needed to clarify the role of the different therapeutic options and the optimal way to combine them.