Acinar cell carcinoma with PRKAR1A and PTEN alterations and paraneoplastic panniculitis.

Pancreatic acinar cell carcinoma is a rare type of pancreatic malignancy, which can be confused with pancreatic neuroendocrine neoplasm. Here, we describe a woman in her 80s who presented with abdominal pain and bilateral lower extremity panniculitis. She underwent surgery for a presumed diagnosis of neuroendocrine tumour with PTEN and PRKAR1A alterations; 19 months, later, a recurrence of her pancreatic malignancy was discovered. The patient underwent repeat resection and this time immunohistochemical staining confirmed the diagnosis of acinar cell carcinoma. Staining for acinar cell carcinoma should be prompted based on clinical suspicion in context of PTEN or PRKAR1A mutation when appropriate.

Painful lower limb nodules as first symptom of resectable pancreatic acinar cell cancer: a case report.

BACKGROUND: Pancreatic panniculitis is characterized by subcutaneous fat necrosis and is a rare presentation of an underlying pancreatic disease, appearing in approximately 2-3% of all patients with a pancreatic disease. The nodules usually involve the lower extremities. Pancreatic panniculitis is commonly associated with acute or chronic pancreatitis, and occasionally with pancreatic cancer, especially acinar cell carcinoma. CASE PRESENTATION: A 77-year-old Caucasian woman with no significant medical history was referred to our center with multiple painful, itchy, and warm red/blue cutaneous nodules on the left lower leg. These skin lesions were consistent with the clinical diagnosis of panniculitis. The skin biopsy obtained showed a predominantly lobular panniculitis with fat necrosis of which the aspect was highly suspicious for pancreatic panniculitis. Further analysis revealed high lipase serum of > 3000 U/L (normal range < 60 U/L), and on computed tomography scan a mass located between the stomach and the left pancreas was seen. Endoscopic ultrasonography-guided fine-needle biopsy confirmed the diagnosis of acinar cell carcinoma. After discussing the patient in the pancreatobiliary multidisciplinary team meeting, laparoscopic distal pancreatectomy including splenectomy and en bloc wedge resection of the stomach due to tumor in-growth was performed. The cutaneous nodules on both legs disappeared 1-2 days after surgery. No long-term complications were reported during follow-up. One year after surgery, the patient presented with similar symptoms as preoperatively. Computed tomography scan showed local recurrence and distal metastases, which were subsequently confirmed by biopsy. She started with palliative folinic acid-fluorouracil-irinotecan-oxaliplatin chemotherapy but stopped after two cycles because of disease progression. The patient died 2 months later, 13 months after surgical resection. CONCLUSION: This case illustrates the importance of clinically recognizing cutaneous nodules and pathological recognizing the specific microscopic changes as sign of a (malignant) pancreatic disease.

ACTH-secreting parotid acinic cell carcinoma unusually reported as a paraneoplastic syndrome.

Paraneoplastic syndromes, induced by an immunological cross-reaction or hormone/peptide secretion, are an atypical presentation of tumors. Some tumors, such as small cell lung cancer and bronchial carcinoid, can be adrenocorticotropic hormone (ACTH) secreting tumors. Less commonly, parotid acinic cell carcinoma can be ACTH-secreting tumor leading to Cushing's syndrome. Few literature cases have described ACTH related paraneoplastic syndrome of parotid adenocarcinoma. Because of the rarity of the condition, little is known about the management and prognosis of this phenomenon. In this report, we highlighted the case of a 59-year-old male with a past medical history of parotid adenocarcinoma treated with surgery, chemotherapy, and radiation therapy presented with clinical and biochemical signs of hyperaldosteronism. Abdominal ultra-sound, computed tomography, and magnetic resonance imaging showed hepatic mass. Liver biopsy with immunohistochemistry confirmed the presence of parotid adenocarcinoma secreting ACTH. He is on paclitaxel and carboplatin medication with good clinical response.

Síndrome de hipersecreción de lipasa y lesiones óseas en el cáncer de células acinares del páncreas / Lipase hypersecretion syndrome and bone lesions in acinar cell cancer of the pancreas. Report of one case

Lipase hypersecretion syndrome (LHS) is a rare paraneoplastic syndrome, associated with acinar cell carcinoma of the pancreas (ACCP) in 10% to 15% of patients. Clinically, LHS manifests itself with the appearance of subcutaneous fibrocystic nodules, associated with trophic changes in the overlying skin, such as ulcers or fistulas that are difficult to manage, mainly affecting the lower extremities. Additionally, lipolysis near the joints and in the intraosseous adipose tissue can cause bilateral arthralgias, especially of the knees and ankles. We report a 57-year-old man, with a history of insulin resistance and allergic rhinitis, who presented in June 2019 with multiple subcutaneous nodules in the lower extremities, predominantly in both ankles, associated with arthralgia in that region. Additionally, a CT scan of the abdomen revealed a significant abdominal mass, measuring approximately 17 cm and in contact with the body and tail of the pancreas, pathologically compatible with an ACCP. Treatment with capecitabine was started with a favorable progression. The patient currently presents a small left lateral retro malleolar fistula, which, given the analyzes, studies and reviewed literature is concluded to be a lesion in the context of LHS.

A case of pathologically complete response after preoperative chemotherapy in a pancreatic acinar cell carcinoma patient with portal vein tumor thrombosis.

Preoperative treatment is being proposed as a standard treatment for pancreatic ductal adenocarcinoma though few cases show a pathologically complete response. On the other hand, there is no consensus regarding preoperative chemotherapy for pancreatic acinar cell carcinoma (ACC). The present study described a rare case of ACC in the pancreatic head with portal vein tumor thrombosis (PVTT) treated with preoperative chemotherapy using modified FOLFIRINOX, which achieved a pathologically complete response. A 65-year-old man was referred for consideration of treatment strategy. Contrast-enhanced abdominal computed tomography revealed a pancreatic tumor and PVTT. The pancreatic tumor was diagnosed as ACC by an endoscopic ultrasound-guided fine-needle aspiration biopsy. Initially, the tumor was assessed as unresectable due to the presence of PVTT, and therefore, a chemotherapy using modified FOLFIRINOX was administered. After 14 courses of the chemotherapy, imaging studies revealed that the tumor and PVTT showed marked reduction in size; thus, the patient underwent pancreaticoduodenectomy with combined resection of the portal vein (PV). A pathological examination uncovered a complete degeneration of the primary tumor and the PV embolus without any residue of carcinoma. The patient did not receive adjuvant chemotherapy and survived with no evidence of recurrence for 33 months after surgery. The chemotherapy using modified FOLFIRINOX could give a complete response in patients with pancreatic ACC with PVTT.

[Lipase hypersecretion syndrome and bone lesions in acinar cell cancer of the pancreas. Report of one case]. / Síndrome de hipersecreción de lipasa y lesiones óseas en el cáncer de células acinares del páncreas.

Lipase hypersecretion syndrome (LHS) is a rare paraneoplastic syndrome, associated with acinar cell carcinoma of the pancreas (ACCP) in 10% to 15% of patients. Clinically, LHS manifests itself with the appearance of subcutaneous fibrocystic nodules, associated with trophic changes in the overlying skin, such as ulcers or fistulas that are difficult to manage, mainly affecting the lower extremities. Additionally, lipolysis near the joints and in the intraosseous adipose tissue can cause bilateral arthralgias, especially of the knees and ankles. We report a 57-year-old man, with a history of insulin resistance and allergic rhinitis, who presented in June 2019 with multiple subcutaneous nodules in the lower extremities, predominantly in both ankles, associated with arthralgia in that region. Additionally, a CT scan of the abdomen revealed a significant abdominal mass, measuring approximately 17 cm and in contact with the body and tail of the pancreas, pathologically compatible with an ACCP. Treatment with capecitabine was started with a favorable progression. The patient currently presents a small left lateral retro malleolar fistula, which, given the analyzes, studies and reviewed literature is concluded to be a lesion in the context of LHS.

68Ga-DOTATATE PET/CT Uptake in Prostate With an Incidental Finding of Prostatic Acinar Adenocarcinoma and Metastatic Neuroendocrine Cancer to the Liver.

ABSTRACT: A 69-year-old man with history of metastatic neuroendocrine tumor presented for initial staging with 68Ga-DOTATE PET/CT. 68Ga-DOTATATE PET/CT showed incidental focal increased DOTATATE uptake in the left apical prostate tissue, which was thought to be of benign etiology. Digital rectal examination later was consistent with a palpable nodule along with elevated prostate-specific antigen of 7.0 ng/mL. MRI of prostate demonstrated a 3.8-cm lesion followed by a targeted biopsy that revealed prostatic acinar adenocarcinoma. Chronic inflammatory cell infiltrates were also noted on biopsy, and this may have been the cause of increased DOTATATE uptake seen on 68Ga-DOTATATE PET/CT study.

Ectopic ACTH Secretion Secondary to Metastatic Acinic Cell Carcinoma of the Parotid Gland: A Case Report and Review of Current Evidence for Systemic Therapy.

Acinic cell carcinoma is a rare, typically indolent, neoplasm that arises in the salivary glands. Metastatic disease is uncommon, occurring in around 10% of cases. We report the case of a 46-year-old male in whom the first sign of disseminated disease was increased skin pigmentation due to paraneoplastic Cushing's syndrome. He underwent 3 cycles of chemotherapy with carboplatin and paclitaxel with no symptomatic improvement and a mixed response on imaging. There is no evidence that systemic therapy prolongs survival in metastatic acinic cell carcinoma, and we lack a consensus as to which treatment options are most beneficial. A summary of published evidence regarding choice of palliative chemotherapy regimens and response is discussed in relation to the case.

Rare case of venous tumour thrombus from acinic cell carcinoma of the parotid gland.

Head and neck tumour thrombus is a rare pathology and at present there are no reported cases of tumour thrombus secondary to acinic cell carcinoma of the parotid gland. We report a case of an 81-year-old man with an acinic cell carcinoma of the left parotid and an intravenous tumour thrombus extending from the retromandibular vein into the internal jugular vein. This case also highlights the importance of radiological imaging in the management of tumour thrombus.

Ectopic ACTH Production and Cushing's Syndrome in a Patient with Parotid Acinic Cell Carcinoma with High-Grade Transformation: Tumor Context and Clinical Implications.

We report a rare case of Cushing's syndrome in a 59-year-old man who initially presented with concurrent acinic cell carcinoma of the parotid with high-grade transformation and co-existing papillary and medullary thyroid carcinomas, without noticeable cushinoid symptoms. Six-months later, he developed clinical features of Cushing's syndrome which coincided with disease progression in the form of lung metastasis and mediastinal lymphadenopathy. Ectopic adrenocorticotropic hormone (ACTH) production and protein expression was limited to the high-grade transformed component of acinic cell carcinoma and in the lymph node metastasis, and was absent in the conventional acinic cell carcinoma as well as in the papillary and medullary thyroid carcinoma. He received adjuvant chemotherapy and supportive management with interval improvement for 8 months followed by disease progression with increasing serum cortisol levels and bone metastasis. He was offered palliative chemotherapy, however, declined further therapy and was lost to follow up. We discussed clinical and pathologic implications of ectopic ACTH production associated with acinic carcinoma and also reviewed the literature of this rare paraneoplastic syndrome.

Lipase hypersecretion syndrome: A distinct form of paraneoplastic syndrome specific to pancreatic acinar carcinomas.

Lipase hypersecretion syndrome (LHS) is a paraneoplastic syndrome seen exclusively as a result of pancreatic acinar cell carcinoma (ACC). In LHS, acinar enzymes (lipase, trypsin and chymotrypsin) which are normally secreted to the duodenum for digestive purposes, are instead released to the blood by the carcinoma cells. In a way, it is "endocrine-ization" of an "exocrine" function. These circulating enzymes, especially lipase, exerts its digestive action on other tissues, especially on the subcutaneous tissues in the pressure poins of legs, creating a picture often mistaken as erythema nodosum or rheumatic nodules. The bone and joints may also be effected, which mostly appears to be secondary to the complications and super-infection of the skin lesions. Eosinophilia also often accompanies this syndrome. The accurate diagnosis of LHS requires the identification of the pancreatic primary as well as its correct classification as acinar because a variety of pancreatic tumors can be associated with skin lesions, ranging from rare metastasis of adenocarcinoma to the necrolytic migratory erythema caused by glucagon-producing neuroendocrine tumors. Towards this differential, the diagnostic characteristics of acinar cell carcinomas that have been better elucidated in the past decade often need to be employed in increasingly smaller specimens and the liver, especially since most LHS cases also have liver metastasis (presumably due to the by-pass of the "first-pass" liver metabolism phenomenon). ACC (and LHS) occur in patients in their 60's. The pancreatic mass is often large, round, demarcated and closely resemble neuroendocrine and solid-pseudopapillary neoplasms but are more atypical/proliferative, and commonly show single prominent nucleoli and a distinctive chromophilia. Immunostaining with trypsin/chymotrypsin, negativity of beta-catenin help in the differential; as a caveat, neuroendocrine differentiation is common in ACCs. In conclusion, LHS is a rare type of paraneoplastic syndrome specific to ACC. The accurate diagnosis requires attention to their subtle diagnostic characteristics.

Paraneoplastic Cast Nephropathy Associated With Pancreatic Mixed Acinar-Neuroendocrine Carcinoma: A Case Report.

Acute kidney injury is common in patients with cancer and may result from sepsis, obstruction, radiotherapy, chemotherapeutic agents, and nonsteroidal anti-inflammatory drugs. Rare reports of acute kidney injury due to cast nephropathy in patients with pancreatic acinar cell carcinoma have been described, but a pathogenetic link between cast nephropathy and carcinoma was not established. We report a patient with pancreatic mixed acinar-neuroendocrine carcinoma who developed severe acute kidney injury. Kidney biopsy showed cast nephropathy characterized by fractured periodic acid-Schiff-negative casts, associated with mononuclear and giant cell reaction. The patient did not have multiple myeloma and casts did not show immunoglobulin light chain restriction on immunofluorescence. Analysis using liquid chromatography-tandem mass spectrometry and immunohistochemistry identified 2 acinar cell-specific proteins, regenerating islet-derived 1α and carboxypeptidase A1, in both tubular casts and tumor cells. Thus, this case demonstrates that solid tumor-specific proteins can be nephropathic by obstructing renal tubules, resulting in acute kidney injury, a previously proposed but not characterized pathophysiologic mechanism for paraneoplastic nephropathy associated with carcinoma.

Paniculitis pancreática como síntoma de debut de un carcinoma de células acinares / Pancreatic panniculitis as a presentation symptom of acinar cell carcinoma

La paniculitis pancreática es una rara manifestación cutánea asociada a patología pancreática. De características similares a otras paniculitis, su evolución es paralela al proceso desencadenante, pudiendo en ocasiones preceder a la clínica propia del mismo. Presentamos el caso de una paciente con paniculitis pancreática por lo demás asintomática que tras el estudio etiológico resultó padecer un carcinoma pancreático de células acinares con metástasis hepáticas

Pancreatic panniculitis is a rare skin manifestation associated with pancreatic conditions. This condition has similar characteristics to those of other panniculitis types and its course parallels the triggering condition and may occasionally precede it. We report the case of a female patient with asymptomatic pancreatic panniculitis; the etiologic study identified a pancreatic acinar cell carcinoma with liver metastases

Pancreatic panniculitis as a presentation symptom of acinar cell carcinoma.

Pancreatic panniculitis is a rare skin manifestation associated with pancreatic conditions. This condition has similar characteristics to those of other panniculitis types and its course parallels the triggering condition and may occasionally precede it. We report the case of a female patient with asymptomatic pancreatic panniculitis; the etiologic study identified a pancreatic acinar cell carcinoma with liver metastases.

Indolent T-lymphoblastic proliferation concomitant with acinic cell carcinoma mimicking T-lymphoblastic lymphoma: case report and literature review.

AIMS: Indolent T-lymphoblastic proliferation (iT-LBP) is a non-clonal benign condition showing extrathymic proliferation of T-lymphoblasts positive for CD3, CD4, CD8, and TdT. Isolated iT-LBP has been observed, but the majority of iT-LBPs have been seen in conjunction with other disorders, including Castleman disease, hepatocellular carcinoma, follicular dendritic cell tumours, angioimmunoblastic T-cell lymphoma, myasthenia gravis, and acinic cell carcinoma (ACC). The clinical course of iT-LBP is indolent, and no therapy is usually required. A major concern is misdiagnosis as T-lymphoblastic lymphoma, and a correct diagnosis of iT-LBP often requires not only pathological analysis but also careful monitoring of the clinical course. The aim of this study was to broaden the knowledge of pathologists and physicians concerning this as yet not well-recognised entity. METHODS AND RESULTS: We report a case of iT-LBP concomitant with ACC, along with a literature review of all 14 cases of iT-LBP reported to date. CONCLUSIONS: iT-LBP should always be considered as a differential diagnosis of T-lymphoblastic lymphoma, as the two disorders show extremely similar traits.

Severe panniculitis and polyarthritis caused by acinar cell carcinoma arising from an ectopic pancreas.

The pancreatitis, panniculitis and polyarthritis (PPP) syndrome is a rare condition caused by pancreatic diseases, such as acute or chronic pancreatitis or pancreatic carcinoma. We report the first case of PPP syndrome caused by metastatic acinar cell carcinoma from an ectopic pancreas. The symptoms were successfully managed by the treatment of the metastatic carcinoma. Pancreatic cytosteatonecrosis should be always considered in a patient who is showing symptoms of panniculitis and polyarthritis.

Repeat Imaging in Idiopathic Unilateral Vocal Fold Paralysis: Is It Necessary?

OBJECTIVE: Imaging plays a critical role in the evaluation of patients presenting with unilateral vocal fold paresis or paralysis of unknown etiology. In those with idiopathic unilateral vocal fold paralysis (iUVFP), there is no consensus regarding the need or timing of repeat imaging. This study seeks to establish the rate of delayed detection of alternate etiologies for these patients to determine if and when imaging should be repeated. METHODS: Retrospective chart review was conducted identifying patients at our institution with vocal fold movement impairment between 1998 and 2014. Idiopathic paralysis was diagnosed if physical examination, laryngoscopy, and initial imaging excluded a cause. Demographic data, length of follow-up, and the presence of late lesions were noted. Time to detection was plotted using the Kaplan-Meier method. RESULTS: Of 3210 patients reviewed, 207 had a diagnosis of iUVFP. Of these patients, 8 went on to develop alternate diagnoses, including pulmonary disease, skull-base and laryngeal neoplasms, and thyroid malignancy. In Kaplan-Meir analysis, 90% remained "idiopathic" at 5 years of follow-up. The mean time to detection was 27 months. CONCLUSIONS: Patients initially diagnosed with iUVFP may have an occult cause that later becomes evident. We recommend repeat imaging within 2 years after diagnosis, but this is likely unnecessary beyond 5 years.

[Lung Segmentectomy Using Video-assisted Thoracic Surgery for Lung Cancer in a Patient with Situs Inversus Totalis].

The case was 83-year-old man who had complete situs inversus, and was pointed out to have peripheral adenocarcinoma with the size of 1.8 cm at the left upper lobe( S3). Because of severe emphysema and other multiple comorbidities, left S3 segmentectomy with hilar lymph node sampling was performed using video-assisted thoracic surgery (VATS). Preoperatively, the simulation of operation was performed using the 3 dimension computed tomography images of pulmonary arteriovenous and bronchus (3DCTAB). Postoperative course was uneventful. 3DCTAB was thought to be useful in understanding the anatomical location of pulmonary arteriovenous and bronchus directly, and in performing segmentectomy in the case of situs inversus like this.