Clinical, Histopathological and Immunohistochemical Aspects of Digital Papillary Adenocarcinoma: A Case Report and Literature Review.

Digital papillary adenocarcinoma (DPA) is a rare malignant eccrine tumor. A 62-year-old female presented with a subcutaneous nodular 1.5cm-mass in the thumb. Macroscopically, a poorly circumscribed mass containing cystic and solid components was observed. Microscopically, epithelial neoplasm consisting of tubular-cystic structures with back-to-back arrangements was observed. The lining epithelium was composed of cuboidal/columnar cells with mild atypia, with micropapillary extensions. Immunohistochemistry revealed double-layered neoplastic epithelium containing two different types of cells: basaloid/myoepithelial and luminal. We recommend two out of vimentin, HMWCK, and D2-40 for myoepithelial/basaloid cells, also CK7 and EMA for luminal/columnar cells. As the tumor had infiltrated the surgical margins, the patient underwent axillary sentinel lymph node (SLN) dissection and re-excision with Mohs micrographic surgery (MMS). Two additional MMS stages were required due to suspicious surgical margin positivity in the frozen sections. The operation was continued despite the risk of loss of function. Upon examination of the permanent sections, we observed no tumors in the suspected positive foci. Additionally, no tumor was found in the surgical margins. No metastasis was detected in the sentinel lymph node. We have reached 300 reported cases of DPA in the literature. We discussed the histopathological and intraoperative diagnostic pitfalls of DPA with a literature review and our experience.

Invasive Papillary Adenocarcinoma of Gallbladder: A Case Report.

Papillary gallbladder adenocarcinoma (PGA) represents 5.0% of all malignant tumor of gallbladder. An early diagnosis is essential as this malignancy progresses silently with a late diagnosis, often proving fatal. A 63 year old female presented with right upper quadrant pain, palpable gallbladder on clinical examination and hypoechoic shadow suggestive of gall stone inside on ultrasound. But during an attempt to open cholecystectomy surgeons found tiny papillary growth involving whole fundus and part of the body on June 2020 at an outside hospital, Khulna, Bangladesh. Radical cholecystectomy was done by the surgeon with enlarged portal lymph node dissection and a small portion of hepatic resection. Histopathology demonstrated a well-differentiated invasive papillary adenocarcinoma with muscle invasion. There was no metastasis in the liver and lymph nodes show reactive hyperplasia.

Thyroid-like low-grade nasopharyngeal papillary adenocarcinoma: a clinicopathological and molecular study of four cases.

Thyroid-like low-grade nasopharyngeal papillary adenocarcinoma (TLLGNPPA) is a rare nasopharyngeal carcinoma. To date, less than 60 cases of TLLGNPPA have been reported, and its clinical features and pathogenesis remain unclear. In this paper, four cases of TLLGNPPA were reported to clarify the clinicopathological and molecular features of this disease. Histopathological examination revealed that all tumors had papillary glandular arrangement, with a fibrovascular axis in the tumor stroma and focal nuclear groove. All tumors expressed pan-CK, CK7, and CK19, while TG and Pax-8 were negative, and the Ki-67 index was approximately 1-3%. The expression of TTF-1 was diffusely positive in two cases and focally positive in two cases. EBER was not expressed in four cases. Molecular testing was possible in three cases. No common driver event was noted, but unique, mutually exclusive molecular variants were found in each of the three tumors (FGFR4, PDK1, AXIN2, FOXL2, and PIK3C3), one also with copy number variants in MCL1 and STMN1. All four patients underwent surgical resection of the tumor and had no metastasis or recurrence from 7 to 60 months post-resection. Given the assertion that these tumors do not recur or metastasize in addition to their heterogeneous gene mutation spectrum, we propose that TLLGNPPA is a neoplasm with low malignant potential and should no longer to be referred to as an adenocarcinoma.

[A Case of Intraductal Papillary Mucinous Adenocarcinoma with Hepatic Dysfunction Due to Tumor Perforation into the Bile Duct].

A 58-year-old woman presented with a complaint of weight loss. Abdominal computed tomography showed dilatation of the biliary and pancreatic ducts and a mural nodule in the pancreatic duct. The diagnosis was intraductal papillary mucinous neoplasm(IPMN). Endoscopic retrograde cholangiopancreatography(ERCP)and cholangioscopy revealed a fistula between the common bile duct and the IPMN. A sudden increase in hepatobiliary enzymes was noted preoperatively. ERCP showed that the common bile duct was obstructed by mucus. A nasobiliary drainage tube was inserted into the bile duct endoscopically and kept open by daily tube washing, and the liver dysfunction improved. Total pancreatectomy, splenectomy, and regional lymph node dissection were performed. Histological examination confirmed that the primary tumor was mixed invasive intraductal papillary mucinous adenocarcinoma. The patient remains alive and well with no evidence of recurrence 18 months after resection.

Value of Immunohistochemistry to Differentiate Digital Papillary Adenocarcinoma From Acral Hidradenoma With Papillary Structures.

ABSTRACT: Digital papillary adenocarcinoma is a malignant adnexal tumor with a predilection for acral sites. Hidradenoma is a benign solid and cystic sweat gland neoplasm with focal ductal and glandular differentiation and good outcomes. Hidradenomas can occur at acral sites and show papillary structures; for this reason, they are included in the differential diagnosis of digital papillary adenocarcinoma, and immunohistochemistry is a valuable tool in this scenario. We described a case of a 43-year-old man with an epithelial tumor showing papillary structures in the intermediate phalanx of the fourth finger. There was diffuse positivity for p63 and negativity for S100 protein, suggesting that this tumor was an acral hidradenoma with papillary structures.

A Retrospective Cohort Study of Factors Influencing Lymph Node Metastasis in Patients With Early Gastric Papillary Adenocarcinoma.

INTRODUCTION: High risk of lymph node metastasis (LNM) in gastric papillary adenocarcinoma causes endoscopists to worry about the suitability of endoscopic resection for early gastric papillary adenocarcinoma (EPAC). We compared risk factors and attempted to establish a scoring system to stratify LNM risk in patients with EPAC. METHODS: A retrospective analysis was performed on 2,513 patients with early gastric carcinoma (EGC) who underwent radical resection in 4 tertiary hospitals in China. Univariate and multivariate analyses were performed to compare the invasiveness in EPAC and other types of EGC and to evaluate potential factors in predicting LNM risk in EPAC groups. RESULTS: Three hundred thirty-five patients with EPAC were enrolled in our study, of which 62 patients were found to have LNM. After comparing clinicopathological characteristics of EPAC with and without LNM, the following factors were included in the risk scoring system: 1 point each for lower stomach location and tumor size >2.0 cm, 3 points for lymphovascular invasion, and 4 points for submucosal invasion; the risk scoring system was validated in a small internal validation set with an area under the curve of 0.844. DISCUSSION: Our results suggested that EPAC was highly invasive compared with other EGCs, especially differentiated EGC types, and need to be treated more rigorously. This proposed risk scoring system could stratify LNM risk in patients with EPAC, and endoscopic resection may only be performed safely on the groups with a low LNM rate.

[A case of invasive intraductal papillary mucinous carcinoma arising from jejunal heterotopic pancreas].

Intraductal papillary mucinous carcinoma (IPMC) arising from the heterotopic pancreas is rare. A case of IPMC metastasis from the jejunal heterotopic pancreas was described. The heterotopic pancreas could be the source of the submucosal tumor-like lesion found in the small intestine with an elevated carbohydrate antigen (CA) 19-9 level. A 60-year-old woman was admitted to the hospital with pulmonary thromboembolism and anemia. The level of CA19-9, a tumor marker, was found to be 211.8U/ml. A tumor in the jejunum was discovered using contrast-enhanced computed tomography. There were also a number of hepatic tumors found. A submucosal tumor-like lesion in the jejunum was discovered during an enteroscopy, and a biopsy revealed it to be an adenocarcinoma. Partial resection of the jejunum was performed to control hemorrhage. Histopathology revealed an invasive IPMC arising from a heterotopic pancreas (Heinrich type II) and chemotherapy with gemcitabine and nab-paclitaxel was initiated. There have only been three cases of invasive IPMC from a heterotopic pancreas reported, and this is the first one to include chemotherapeutic treatment of distant metastasis.

Gallbladder Cancer: A Single-Institution 10-Year Experience-Analysis of Adenocarcinoma Subtypes and Tumors Arising from Intracholecystic Papillary Neoplasms.

BACKGROUND: Gallbladder cancer accounts for 1.2% of global cancer diagnoses. Literature on biliary-type adenocarcinoma (BTA), and specifically carcinoma arising from intracholecystic papillary-tubular neoplasms (ICPNs), is limited. This study describes a retrospective, single-institution experience with gallbladder cancer, focusing on histological subtypes and prognosis. METHODS: A retrospective review was performed of patients who underwent cholecystectomy for a malignant neoplasm of the gallbladder between 2007 and 2017. Demographic, clinicopathologic, and operative variables, as well as survival outcomes, were analyzed. RESULTS: From a total of 145 patients, BTAs were most common (93, 64%). Compared with non-BTAs, BTAs were diagnosed at a lower American Joint Committee on Cancer stage (p = 0.045) and demonstrated longer median recurrence-free survival (38 vs. 16 months, p = 0.014; median follow-up 36 months). Tumors arising from ICPNs (18, 12%) were more commonly associated with BTA (14 cases). Compared with BTAs not associated with ICPNs (29 patients), associated cases demonstrated lower pathologic stage (p = 0.006) and lower rates of liver and perineural invasion (0% vs. 49% and 14% vs. 48%, respectively; p < 0.05). Cumulative 5-year survival probability was higher for patients with gallbladder neoplasm of any subtype associated with ICPNs compared with those that were not associated with ICPNs (54% vs. 41%, p = 0.019; median follow-up 23 months). This difference was also significant when comparing BTAs associated with ICPNs and non-associated cases (63% vs. 52%, p = 0.005). CONCLUSIONS: This study demonstrated unique pathological and prognostic features of BTAs and of carcinomas arising from ICPNs. Histopathological variance may implicate prognosis and may be used to better guide clinical decision making in the treatment of these patients.

Survival comparison of invasive intraductal papillary mucinous neoplasm versus pancreatic ductal adenocarcinoma.

BACKGROUND: The aim of this study was to assess the relevance of highlighting T1a invasive intraductal papillary mucinous carcinoma as a separate subcategory and to compare the tumor biology between invasive intraductal papillary mucinous carcinoma and pancreatic ductal adenocarcinoma. METHODS: A total of 144 and 328 consecutive patients with intraductal papillary mucinous neoplasms and pancreatic ductal adenocarcinoma, respectively, were analyzed. RESULTS: Patients with T1a invasive intraductal papillary mucinous carcinoma comprised 25% (11/44) of the overall subject population with invasive intraductal papillary mucinous carcinoma with 5-year disease-specific survival rate being 100%. None of the patients with pancreatic ductal adenocarcinoma were classified as having T1a disease. When patients with invasive intraductal papillary mucinous carcinoma and pancreatic ductal adenocarcinoma were compared after excluding patients with T1a invasive intraductal papillary mucinous carcinoma, the 5-year disease-specific survival rates were 63% vs 40% in node-negative status (P = .018); and they were 20% vs 13% in node-positive status (P = .385). Subsequent analyses revealed that this survival superiority was limited to patients without evidence of lymphatic invasion. CONCLUSION: T1a invasive intraductal papillary mucinous carcinoma is a clinical entity specifically observed in patients with intraductal papillary mucinous carcinoma, but not in patients with pancreatic ductal adenocarcinoma, and is associated with excellent postoperative survival outcomes. In the survival comparison after exclusion of patients with T1a tumors, when the analysis was limited to patients without lymphatic invasion or lymph node metastasis, the disease-specific survival rate remained higher in patients with invasive intraductal papillary mucinous carcinoma compared with those with pancreatic ductal adenocarcinoma, and this difference was considered as being attributable to the intrinsic indolent biological behavior of invasive intraductal papillary mucinous carcinoma. However, this survival advantage was lost once lymphatic invasion occurred.

Wide Local Excision with Sentinel Lymph Node Biopsy for Digital Papillary Adenocarcinoma: A Case Report.

Digital papillary adenocarcinoma (DPA) is a rare but locally aggressive eccrine tumour that has metastatic potential and most frequently found in the upper extremity. It usually presents as an unremarkable swelling of the skin, commonly misdiagnosed as a benign lesion. We present a 67-year-old male who presented with a slowly growing mass of the right middle finger that was biopsied and diagnosed with DPA. The patient was treated with wide local excision (WLE) and sentinel lymph node biopsy by us during the same operative encounter. This report highlights the need for increased clinical vigilance to diagnose DPAs, and that WLE and sentinel lymph node biopsies can be safe and effective treatments offered. Level of Evidence: Level V (Therapeutic).

Factors associated with lymph node metastasis upstage after resection for patients with micropapillary lung adenocarcinoma.

BACKGROUND: Micropapillary adenocarcinoma has a poor prognostic histological pattern. Additionally, preoperative detection of lymph node metastases by preoperative examination is difficult in some patients with micropapillary adenocarcinoma, and postoperative upstage may occur. However, clinicopathological features of patients with micropapillary adenocarcinoma with nodal upstage have not been established, therefore this study aimed to identify the factors associated with potential lymph node metastases during preoperative examination to ensure effective surgical procedures. METHODS: Between January 2011 and December 2020, 1029 patients received complete resection for primary non-small-cell lung cancer by lobectomy or more extensive resection with systematic lymph node dissection at this institution. One hundred and thirty-one patients diagnosed with adenocarcinoma with micropapillary component were included in this study. The clinicopathological features of patients with nodal upstage whose postoperative N stage was more advanced than the preoperative N stage were examined. RESULTS: Forty patients had nodal upstage after resection. 18 F-fluorodeoxyglucose (FDG) positron emission tomography-computed tomography (PET-CT) revealed that a maximum standardized uptake value (SUVmax) ≥5 for the primary lesion was significantly associated with postoperative nodal upstage. There were no significant differences in terms of sex, age, smoking history, surgical procedure, and diabetes. Among 38 patients with nodal upstage, 23 patients had no significant preoperative lymphadenopathy and showed no abnormal FDG uptake in the lymph nodes on 18 F-FDG-PET-CT, respectively. CONCLUSIONS: Lymph node metastases were suspected in patients preoperatively diagnosed with micropapillary adenocarcinoma with FDG SUVmax ≥5 for the primary tumor. Therefore, standard surgical resection and careful lymph node dissection should be performed for such patients.

Digital Papillary Adenocarcinoma in the Dorsal Proximal Ring Finger.

Digital papillary adenocarcinoma (DPA) is a rare and aggressive tumor arising from the eccrine sweat glands. It is found on the hands and feet and most commonly occurs on the volar and distal finger tips. In this report, we describe a DPA in a 45-year-old woman who presented with a slowly enlarging mass on the dorsal aspect of her proximal ring finger. This report shows that DPA may clinically present as a relatively benign-appearing mass and in an atypical location. Surgical excision, followed by histologic and immunohistochemical evaluations of even benign-appearing digital masses, is important because this is the only way a DPA can be diagnosed.

Preoperative confirmation of the accurate location of a fistula using endoscopy and fluoroscopy in a patient with intraductal papillary-mucinous carcinoma of the pancreas penetrating the stomach and colon.

Highlight Tanisaka and colleagues report a case of intraductal papillary mucinous carcinoma of the pancreas that formed a fistula between the stomach and colon. Both the fistula of the stomach and that of the colon were confirmed preoperatively on endoscopic and fluoroscopic findings, and histologically after surgery.

Primary nasopharyngeal papillary adenocarcinoma: A CARE-compliant case report.

RATIONALE: Primary nasopharyngeal papillary adenocarcinoma is a rare nasopharyngeal neoplasm with a good prognosis and a low propensity for regional recurrence. To date, only few cases of primary nasopharyngeal papillary adenocarcinoma have been reported in the literature. PATIENT CONCERNS: A 24-year-old female patient presented with intermittent hemoptysis and blood tinge nasal discharge. DIAGNOSIS: An exophytic and pedunculated mass over the roof of the nasopharynx was found on nasopharyngoscope. Biopsy was done and the pathology confirmed well-differentiated primary nasopharyngeal papillary adenocarcinoma, strongly positive for CK7, and transcription termination factor 1; but negative for thyroglobulin. The final diagnosis was primary nasopharyngeal papillary adenocarcinoma, well-differentiated, pT1N0M0, stage I. INTERVENTIONS: The patient underwent excision of nasopharyngeal tumor under sinuscopic assistance. OUTCOMES: : No local recurrence or distant metastasis was noted during the 6 months of follow-up. LESSONS: We aim at highlighting the importance of a thorough differential diagnosis of nasopharyngeal tumor. Further investigation is still needed for providing evidence to standardize the treatment protocol.

Thyroid-like low-grade nasopharyngeal papillary adenocarcinoma with squamous differentiation in the posterior nasal septum: A rare case report.

RATIONALE: Thyroid-like low-grade nasopharyngeal papillary adenocarcinoma (TL-LGNPPA) is a rare neoplasm characterized by papillary epithelial proliferation and abnormal expression of thyroid transcription factor-1. To our knowledge, squamous differentiation in TL-LGNPPA is an unusual phenomenon, and only 1 case has been reported to date. The tumor occurs mainly on the roof of the nasopharynx. Herein, we report a case of TL-LGNPPA with squamous differentiation located on the posterior nasal septum. PATIENT CONCERNS: A 45-year-old Chinese women presented to our hospital with a complaint of nasal obstruction for 10 years and the symptom has been getting worse for recent 3 years. DIAGNOSES: Microscopic examination of the tumor tissue revealed findings typical of TL-LGNPPA, and in addition to the typical components, squamous differentiation could be found in some areas within the fibrovascular cores of the papillary components. Immunohistochemically, the squamous cells were positive for P40 and P63 but negative for thyroid transcription factor-1. INTERVENTIONS: The patient underwent complete resection of the tumor. OUTCOMES: Follow up results showed that the patient had no recurrence 41 months after removal of the tumor. LESSONS: Our report anticipates that, although TL-LGNPPA with squamous differentiation in the posterior nasal septum is rare, this report will expand the existing knowledge associated with TL-LGNPPA.

An Aggressive Digital Papillary Adenocarcinoma and a Grossly Similar, Contralateral Callus: A Case Report.

CASE: This is a case of a 44-year-old man with 10 years of history of painful callus-like swellings of bilateral index fingers. Sudden enlargement of the left index finger prompted biopsy, which revealed aggressive digital papillary adenocarcinoma (ADPA) that was treated with partial amputation. Biopsy of the contralateral finger revealed acanthotic skin with no evidence of malignancy. CONCLUSION: Several case reports cite antecedent insult and chronic swelling before diagnosis. We describe a case of bilateral lesions with similar gross appearances, a long history of occupational repetitive trauma, and sudden enlargement of 1 lesion leading to the diagnosis of ADPA.

High-grade Papillary Early Gastric Carcinoma With High Risk for Lymph Node Metastasis and Poor Prognosis: A Clinicopathologic Study of 96 Cases Among 1136 Consecutive Radical Gastrectomies.

Papillary early gastric carcinoma (EGC) is believed to have a low risk of lymph node metastasis (LNM) and thus can be resected endoscopically. We observed anecdotally that some papillary EGC tumors showed conspicuous high-grade dysplastic features, but the significance of these observations is unknown. In this bicenter study we investigated papillary EGCs that were divided into high-grade (n=96) and low-grade (n=118) groups among 1136 consecutive EGC radical resection cases. Concurrent 464 well-moderately differentiated tubular EGCs were served as the control group. Compared with low-grade papillary and well-moderately differentiated tubular EGCs, high-grade papillary EGC displayed significantly larger sizes (mean 2.51 cm), higher frequencies of the elevated macroscopic type (51%), lymphovascular invasion (LVI) (38.5%), and LNM (31.2%). Low-grade papillary EGCs exhibited a higher prevalence of the elevated macroscopic type, but not LVI nor LNM, compared with tubular EGC. Independent risk factors for LNM included high-grade histology, female sex, distal location, submucosal invasion, and LVI. The 5-year overall survival rate was significantly lower in high-grade (79.6%) papillary than in low-grade (88.9%) papillary or tubular (92.8%) EGCs, while no significant difference in prognosis was observed in the latter 2 groups. Age of 66 years or older and LNM were independent risk factors for overall survival. In conclusions, high-grade papillary EGC was associated with high frequencies of LVI, LNM, and poor prognosis, and thus unsuitable for endoscopic therapy, while low-grade papillary EGC showed clinicopathologic features and prognosis similar to well-moderately differentiated tubular EGC and may be treated endoscopically in appropriate clinical settings.

Thymic adenocarcinoma accompanied by type A thymoma and pulmonary minimally invasive adenocarcinoma and harboring distinct gene alterations: A case report.

RATIONALE: Thymic adenocarcinoma is an extremely rare thymic carcinoma. The exact genetic alteration associated with thymic adenocarcinoma is unclear. Here, we report a case of thymic adenocarcinoma accompanied by type A thymoma and pulmonary minimally invasive adenocarcinoma (MIA). PATIENT CONCERNS: A 53-year-old woman presented with multiple nodules in the mediastinum and lung. Thoracic computed tomography revealed nodules in the anterior superior mediastinum and anterior mediastinum near the right pericardium and ground-glass opacity (GGO) in the right superior lobe of the lung. DIAGNOSIS: The tumor in the anterior superior mediastinum was diagnosed as primary thymic papillary adenocarcinoma. The tumor in the anterior mediastinum near the right pericardium was diagnosed as type A thymoma. The GGO of the right superior lobe of the lung was diagnosed as a MIA. INTERVENTION: The patient underwent thoracoscopic mediastinal tumor resection and partial lobectomy in our hospital. OUTCOMES: The postoperative course was uneventful. The patient is alive and free of the disease for 22 months after diagnosis. LESSONS: Thyroid transcription factor 1 (TTF-1) was positive in this case of thymic adenocarcinoma, which indicated that a thymic adenocarcinoma with TTF-1-positive may not necessarily be a metastasis of lung or thyroid adenocarcinoma. The positive staining of CD5 and CD117 can help us to confirm the thymic origin. Molecular genetic analysis indicated that these tumors harbored different mutations. The thymic adenocarcinoma and type A thymoma both had the mutation of KMT2A, but the mutation sites were different. KMT2A mutation may be a common genetic change in thymic tumorigenesis. The genetic alterations disclosed in this study will help expand the understanding of thymic tumors.

Well differentiated carcinoma with a poor prognosis: a retrospective analysis of papillary gastric adenocarcinoma.

PURPOSE: To explore the clinicopathological features and prognosis of papillary gastric adenocarcinoma (PGC). METHODS: The subjects of this retrospective analysis were 1525 patients with gastric cancer in a single center in China. RESULTS: The patients with PGC were generally of advanced age and the tumor was located in the upper 1/3 of the stomach. PGC was well or moderately differentiated, with serosal infiltration, early lymph node metastasis, TNM stages I/II, liver metastasis, and a short postoperative overall survival time. Patients with the secondary pathological type of papillary adenocarcinoma presented with clinicopathological similarities to those with primary PGC. PGC was a risk factor for poor survival in both univariate and multivariate analyses. CONCLUSION: Papillary gastric adenocarcinoma (PGC) showed different clinicopathological characteristics and prognosis to other types of gastric cancer (GC), even if it was not the primary pathological type. The higher the proportion of papillary adenocarcinoma in gastric cancer samples, the shorter the postoperative survival time of patients. PGC needs further multicenter studies.