Cistoadenocarcinoma biliar / Biliary Cystadenocarcinoma

Introducción: El cistoadenocarcinoma biliar es un tumor quístico poco frecuente, que se origina a partir del epitelio hepatobiliar, cuyo crecimiento es lento y sus síntomas son variables e inespecíficos. Objetivo: Presentar el caso de un paciente portador de un cistoadenocarcinoma biliar. Presentación del caso: Se presenta el caso de una paciente femenina de 56 años de edad que acude a la institución por presentar dolor en el hipocondrio derecho, fiebre y pérdida de peso. Al examen físico se constata una masa palpable en dicha zona abdominal, de bordes lisos, no dolorosa, dura, de escasa movilidad y relacionada con el hígado. Conclusiones: El cistoadenocarcinoma biliar es una neoplasia quística maligna poco frecuente del hígado. La mayoría de los pacientes presentan dolor abdominal, masa palpable y pérdida de peso. El tratamiento quirúrgico es la norma y la resección con márgenes libres se considera la variante de tratamiento más empleada(AU)

Introduction: Biliary cystadenocarcinoma is a rare cystic tumor, which originates from the hepatobiliary epithelium, whose growth is slow, and whose symptoms are variable and nonspecific. Objective: To present the case of a patient with a biliary cystadenocarcinoma. Case presentation: The case is presented of a 56-year-old female patient who came to our institution with pain in the right hypochondrium, fever and weight loss. Physical examination revealed a palpable mass in such abdominal area, with smooth edges, nonpainful, hard, with low mobility and related to the liver. Conclusions: Biliary cystadenocarcinoma is a rare malignant cystic neoplasm of the liver. Most patients present with abdominal pain, a palpable mass and weight loss. Surgery is the standard treatment and margin-free resection is considered the most commonly employed treatment variant(AU)

Management of Mucinous Cystic Neoplasms of the Liver.

BACKGROUND: Mucinous cystic neoplasms of the liver (MCN-L) including biliary cystadenomas (BCA) and biliary cystadenocarcinomas (BCAC) are rare cystic lesions that comprise less than 5% of all liver cysts and affect only a small subset of individuals. We herein review the current evidence regarding the clinical presentation, imaging characteristics, tumor markers, pathological findings, clinical management, and prognosis of MCN-L. METHODS: A comprehensive review of the literature was performed using MEDLINE/Pubmed and Web of Science databases. In PubMed, the terms "biliary cystadenoma," "biliary cystadenocarcinoma," and "non parasitic hepatic cysts" were queried to identify the most recent data on MCN-L. RESULTS: US imaging, CT, and MRI, as well as consideration of clinicopathological features, are required to appropriately characterize and diagnose hepatic cystic tumors. BCA are premalignant lesions and cannot be reliably differentiated from BCAC based on imaging alone. As such, both types of lesions should be treated with margin-negative surgical resection. Following surgical resection, recurrence is fairly low among patients with BCA and BCAC. Despite having worse long-term outcomes than BCA, the prognosis following surgical resection of BCAC still remains more favorable than other primary malignant liver tumors. CONCLUSION: MCN-L are rare cystic liver tumors that include BCA and BCAC, which can be difficult to differentiate based on imaging alone. Surgical resection remains the mainstay of management for MCN-L with recurrence being generally uncommon. Future multi-institutional studies are still required to better understand the biology behind BCA and BCAC to improve the care of patients with MCN-L.

[Two Operations for Intrahepatic Recurrence of Biliary Cystadenocarcinoma-A Long-Term Survivor].

We report a case of biliary cystadenocarcinoma in which long-term survival was achieved after 2 operations for intrahepatic recurrence. A 72-year-old man with biliary cystadenocarcinoma located mainly in segment 3 of the liver underwent left hepatectomy, extrahepatic bile duct resection, and lymph node dissection. Seven years and 9 months after the initial resection, he underwent partial liver resection(segment 5)for intrahepatic recurrence detected by computed tomography. Fifteen years and 7 months after the initial resection, he underwent repeat partial resection of the liver(segment 5)for intrahepatic recurrence. Histologically, these tumors were confirmed to be recurrence of biliary cystadenocarcinoma. He remains alive and well with no further recurrence 21 years and 6 months after the initial resection. This case and a literature review suggest that hepatic resection is a useful treatment option for intrahepatic recurrence of biliary cystadenocarcinoma.

Cystadenocarcinoma of the intrahepatic bile duct in a dog.

A 14-year-old spayed female Shih-Tzu was referred to the Veterinary Medical Teaching Hospital of Konkuk University for evaluation of an abdominal mass. In diagnostic imaging, two large cystic masses were identified. The affected liver lobes were surgically resected, and the specimens were submitted for histopathological evaluation and immunohistochemical staining. The two cystic lesions were diagnosed as biliary cystadenocarcinoma (BCAC). Recurrence and regional invasion were identified on ultrasonography 36 days postoperatively. The patient died on postoperative day 271. To the best of our knowledge, previously reported case studies of BCAC in dogs presented limited clinical information. In this report, we present a detailed picture comprising a range of clinical information and histopathological examination of BCAC in a dog.

Hepatic Cysts: Reappraisal of the Classification, Terminology, Differential Diagnosis, and Clinicopathologic Characteristics in 258 Cases.

The literature on liver cysts is highly conflicting, mostly owing to definitional variations. Two hundred and fifty-eight ≥1 cm cysts evaluated pathologically using updated criteria were classifiable as: I. Ductal plate malformation related (63%); that is, cystic bile duct hamartoma or not otherwise specified-type benign biliary cyst (35 with polycystic liver disease). These were female predominant (F/M=2.4), large (10 cm), often multifocal with degenerative/inflammatory changes and frequently misclassified as "hepatobiliary cystadenoma." II. Neoplastic (13%); 27 (10.5%) had ovarian-type stroma (OTS) and qualified as mucinous cystic neoplasm (MCN) per World Health Organization (WHO). These were female, solitary, mean age 52, mean size 11 cm, and 2 were associated with carcinoma (1 in situ and 1 microinvasive). There were 3 intraductal papillary neoplasms, 1 intraductal oncocytic papillary neoplasm, 1 cystic cholangiocarcinoma, and 2 cystic metastasis. III. Infectious/inflammatory (12%). These included 23 hydatid cysts (including 2 Echinococcus alveolaris both misdiagnosed preoperatively as cancer), nonspecific inflammatory cysts (abscesses, inflammatory cysts: 3.4%). IV. Congenital (7%). Mostly small (<3 cm); choledochal cyst (5%), foregut cyst (2%). V. Miscellaneous (4%). In conclusion, hepatic cysts occur predominantly in women (3/1), are mostly (90%) non-neoplastic, and seldom (<2%) malignant. Cystic bile duct hamartomas and their relative not otherwise specified-type benign biliary cysts are frequently multifocal and often misdiagnosed as "cystadenoma/carcinoma." Defined by OTS, MCNs (the true "hepatobiliary cystadenoma/carcinoma") are solitary, constitute only 10.5% of hepatic cysts, and have a significantly different profile than the impression in the literature in that essentially all are perimenopausal females, and rarely associated with carcinoma (7%). Since MCNs can only be diagnosed by demonstration of OTS through complete microscopic examination, it is advisable to avoid the term "cystadenoma/cystadenocarcinoma" solely based on radiologic examination, and the following simplified terminology would be preferable in preoperative evaluation to avoid conflicts with the final pathologic diagnosis: (1) noncomplex (favor benign), (2) complex (in 3 subsets, as favor benign, cannot rule out malignancy, or favor malignancy), (3) malignant features.

Sequential thermal ablation in combination with sclerotherapy using lauromacrogol as a successful translative therapy for an unresectable huge biliary cystadenocarcinoma: The first experience assisted by contrast-enhanced ultrasound.

Biliary cystadenocarcinoma (BCAC) is an extremely rare intrahepatic cystic tumor. Patients usually present with nonspecific symptoms such as abdominal pain, abdominal distention, and abdominal mass. This tumor occurs most commonly in the left hemiliver and is thought to mainly develop from a benign biliary cystadenoma (BCA). At present, the disease is mainly diagnosed by ultrasound, CT, MR, and other imaging methods, and the main treatment is radical surgical resection. We reported a 75-year-old female with an unresectable huge BCAC (i.e., 161×145×122 mm in three orthogonal directions) and poor general condition (40 in Karnofsky Performance Status, KPS) who received sequential thermal ablation (i.e., cryoablation and microwave ablation) in combination with sclerotherapy using lauromacrogol. The diagnosis of intrahepatic BCAC was confirmed pathologically. Preablation grayscale US showed the BCAC with a clear boundary, regular shape, and cystic-solid mixed echogenicity, which appeared as a huge multilocular cystic lesions with thick internal sepatations. Preablation contrast-enhanced ultrasound (CEUS) showed honeycomb-like hyper-enhancement of the thick internal sepatations and cystic wall in the arterial and portal phase, and sustained enhancement of the thick internal sepatations and cystic wall in the late phase. 6-month postablation CEUS showed non-enhancement in most parts of the lesion in the arterial phase and 6-month postablation MRI showed the volume reduction ratio (VRR) was about 70%. The abdominal pain and abdominal distension were relieved remarkably, and her quality of life was greatly improved (70 in KPS). In conclusion, sequential thermal ablation in combination with sclerotherapy provides a successful translative therapy for this unresectable huge BCAC with a poor general condition, which makes subsequent curative surgery or ablation possible.

Nevus Sebaceous of the Scalp With Stepwise Progression Into Invasive Syringocystadenocarcinoma Papilliferum.

Syringocystadenocarcinoma papilliferum (SCACP), the malignant counterpart of syringocystadenoma papilliferum (SCAP), is an extremely rare malignant adnexal neoplasm. It is described by the World Health Organization as a malignant transformation of SCAP occurring in middle-aged to elderly individuals with a predilection for the head and neck. SCACP seems to arise from a long-standing syringocystadenoma probably on a background of nevus sebaceous (NS) through a multistep progression. A 75-year-old man was referred to our department with a long-standing NS with a recent newly developing nodule on his scalp. The tumor was excised. On histology, the overall architecture of the tumor still resembled an unusual SCAP within NS but simultaneously showed transition to syringocystadenocarcinoma papilliferum in situ and invasive SCACP as recognizable by the presence of areas of nuclear atypia, increased proliferative activity, and infiltrative growth. In summary, we report an extremely rare case of an invasive SCACP of the scalp that demonstrates histological evidence for all transitive steps in the hypothetical multistep progression from NS to invasive SCACP in one single lesion. The implications of these findings are discussed in the light of the relevant literature.

Enucleation of Biliary Cystadenomas: a Review.

BACKGROUND: Biliary cystadenomas are very rare benign tumors which can transform into cystadenocarcinomas. The largest case series reported on 221 cases over 30 years from 10 HPB centers, i.e., about 7 cases per center per decade. The recommended treatment is liver resection. Enucleation of biliary cystadenomas has been done rarely. The purpose of the study was to determine the outcome of enucleation of these cysts, particularly the mortality rate and the recurrence rate. METHODS: A keyword search was done using OVID followed by a search of the bibliography of papers describing the enucleation of biliary cystadenomas. Of 45 articles obtained, 25 were retained. The main reasons for exclusion were non-English language and review articles. RESULTS: One hundred three patients in the 25 studies were treated with enucleation. Thirteen studies described prior treatments that had failed with resulting recurrence requiring re-treatment. The main indication for enucleation was large central cysts for which liver resection would be high risk. There were no postoperative deaths in patients treated by enucleation. Thirteen studies provided long-term follow-up in 40 patients, a substantial number given the rarity of the tumor. There were no recurrences or transformations to malignancy. CONCLUSIONS: Enucleation seems to represent a reasonable treatment technique for BCA, especially when a large cystic lesion is located centrally and/or would require a large liver resection with significant loss of parenchyma.

Primary peritoneal mucinous cystadenocarcinoma mimicking possible recurrent ovarian mucinous cystadenoma: coincidental pathology or a spectrum of disease?

We present the case of a 32-year-old woman with a previous surgical history of benign mucinous cystadenoma resected in 2012 who underwent magnetic resonance cholangiopancreatography following her second pregnancy in 2020. This demonstrated a large cystic mass in the left subdiaphragmatic space. Histopathology confirmed a well-differentiated primary peritoneal mucinous cystadenocarcinoma displaying cells of a Mullerian origin. We subsequently discuss the aetiology of these conditions separately and explore the possibility of a connection between the two regarding origin or potential malignant transformation that may otherwise have occurred coincidentally in this young patient. We also acknowledge a paucity of evidence regarding subsequent management strategies.

Salivary gland cystadenocarcinoma: Rare neoplasm in buccal mucosa.

A 72-year-old female was referred for diagnosis of a lesion located in the right buccal mucosa, with duration unknown. At intraoral examination, the lesion appeared as a well-delimited, mobile, and submucosal nodule. A benign mesenchymal neoplasm was the main hypothesis of diagnosis. Histopathological analysis revealed salivary gland neoplasm formed by atypical cells often arranged in microcystic structures, with frequent intraluminal papillary projections. The tumor cells presented positivity for CK7 and negativity for CK20. Based on these features, the diagnosis of cystadenocarcinoma was established. The patient was submitted to wide surgical resection. No recurrence was observed after 48 months. Although rare, cystadenocarcinoma should be considered in the differential diagnosis of oral submucosal nodules.

Ovarian masses in children and adolescents: Analysis on 146 patients.

PURPOSE: Ovarian masses in pediatric population are the most common genital neoplasms, and these masses are often benign. The purpose of this study is to evaluate the pediatric ovarian masses operated in our hospital. METHOD: The records of patients, under the age of 18 who were operated in our hospital due to ovarian mass between 2012 and 2018 were reviewed retrospectively. Clinical findings, operational procedures, histopathologies, tumor markers and radiological images were evaluated. FINDINGS: During the study, 146 patients (5 patients were bilateral) were evaluated. The average age of the study patients was 14.01 ± 4.02 years. 107 of the study patients were benign, 37 were malignant and 2 were borderline. The most common symptom in benign masses was tenderness in lower abdominal (75.7 %). 124 of the patients (86.1 %) were in post-menarche period. 34 of the patients had ovarian torsion. Open surgery was conducted on 79.5 % (116/146) of the patients, and laparoscopic surgery was conducted on 20.5 % (30/146). The rate of oophorectomy was 24.6 % (36/146) throughout the operations. The most frequently conducted surgical procedure was cyst excision in benign masses and oophorectomy in malignant masses. In neoplastic masses, the ratio of pelvic mass palpation; and in non-neoplastic masses, lower abdominal tenderness was more apparent. The rate of ovarian torsion was 23.6 % (25/107) in benign masses and 24.3 % (9/37) in malignant masses. OUTCOMES: Pediatric and adolescent ovarian masses are mostly benign and majority of these occur at post-menarche period. The most common symptom was pelvic tenderness in benign masses, and palpable pelvic masses in malignant masses. For future fertility and low incidence of malignancy in these patients, ovarian preserving surgery should be considered for the first operation.