Intraoral Salivary Gland Cystadenomas: A Case Series Study and Literature Review.

BACKGROUND: Salivary gland cystadenoma (SGCA) is a rare benign tumor that predominantly occurs in the parotid gland. SGCAs affecting the minor salivary glands are uncommon and often resemble, clinically and histopathologically, other salivary gland lesions. METHODS: This study aimed to describe a series of four cases of SGCA affecting intraoral sites and performed a literature review of well-reported SGCA published in the English-language literature. RESULTS: SGCA cases included in this series were diagnosed in the buccal mucosa, lip, and hard palate of female patients aged between 19 and 78 years. All cases underwent excisional biopsy and were histologically characterized by a multicystic growth with variable degrees of capsule formation and were lined by several types of epithelium, including some cell types that are infrequently reported in SGCA. In some cases, a small collection of lymphocytes was observed adjacent to cystic formations. All SGCA were positive for periodic acid-Schiff, and immunohistochemical reactions were positive for CK7 and p63. The follow-up time ranged widely from 3 to 53 months, and to date, no recurrence has been observed. CONCLUSION: The literature review revealed a total of 33 published studies accounting for 55 SGCA cases.

Dissecting microenvironment in cystadenomas and hepatic cysts based on single nucleus RNA-sequencing data.

Hepatic cystadenoma is a rare disease, accounting for about 5% of all cystic lesions, with a high tendency of malignant transformation. The preoperative diagnosis of cystadenoma is difficult, and some cystadenomas are easily misdiagnosed as hepatic cysts at first. Hepatic cyst is a relatively common liver disease, most of which are benign, but large hepatic cysts can lead to pressure on the bile duct, resulting in abnormal liver function. To better understand the difference between the microenvironment of cystadenomas and hepatic cysts, we performed single-nuclei RNA-sequencing on cystadenoma and hepatic cysts samples. In addition, we performed spatial transcriptome sequencing of hepatic cysts. Based on nucleus RNA-sequencing data, a total of seven major cell types were identified. Here we described the tumor microenvironment of cystadenomas and hepatic cysts, particularly the transcriptome signatures and regulators of immune cells and stromal cells. By inferring copy number variation, it was found that the malignant degree of hepatic stellate cells in cystadenoma was higher. Pseudotime trajectory analysis demonstrated dynamic transformation of hepatocytes in hepatic cysts and cystadenomas. Cystadenomas had higher immune infiltration than hepatic cysts, and T cells had a more complex regulatory mechanism in cystadenomas than hepatic cysts. Immunohistochemistry confirms a cystadenoma-specific T-cell immunoregulatory mechanism. These results provided a single-cell atlas of cystadenomas and hepatic cyst, revealed a more complex microenvironment in cystadenomas than in hepatic cysts, and provided new perspective for the molecular mechanisms of cystadenomas and hepatic cyst.

Apocrine cystadenoma: A long-standing apocrine hidrocystoma with an adenomatous proliferation.

BACKGROUND: Apocrine cystadenoma is a rare, benign adenomatous cystic neoplasm, the pathogenesis of which is not fully understood. We sought to characterize the clinical, dermatoscopic, and histopathologic features of apocrine cystadenoma and its relationship to hidrocystoma. METHODS: We retrospectively analyzed cases of apocrine cystadenoma and hidrocystoma retrieved from the dermatopathology laboratory information system. RESULTS: Of the 350 cases apocrine cystic lesions, 13 cases of apocrine cystadenomas met the inclusion criteria. The age ranged from 20 to 84 years with an average of 64 years. They were long-standing (duration 3-15 years), slow-growing, large tumors usually found on the scalp. Dermatoscopy accentuated translucent light to dark blue color and prominent vessels that were present more at the periphery. All lesions were multilocular with columnar to cuboidal lining and decapitation secretion. A large portion of the lesion consisted of a simple nonproliferative epithelial lining, identical to that observed in apocrine hidrocystomas, while the proliferative adenomatous component made up a smaller portion with two patterns: (1) tubular proliferation, which either protruded into the cystic cavity or expanded outward peripherally, or (2) papillary projections, which were multiple layers thick with fibrovascular core, sometimes accompanied by tubular proliferation. Immunohistochemical stains showed strong staining for p40 and a sparse number of cells stained for Ki-67 and p53. CONCLUSIONS: The long duration of the lesion and the large areas of simple apocrine epithelial lining suggest that apocrine cystadenomas arise from long-standing apocrine hidrocystomas. However, the retrospective nature of the study from a single institution is a limitation.

Papillary cystadenoma of the major salivary gland: A case report and review of literature.

Papillary cystadenoma (PC) of the salivary gland is an uncommon benign epithelial neoplasm that shows predominantly multicystic growth pattern with intraluminal papillary proliferation and areas of oncocytic differentiation. We report a case of papillary cystadenoma of the parotid gland in a 44-years-old female. The patient presented with painful nodular swelling in the right parotid region for two months. Ultrasonography revealed a well marginated oval lesion with altered signal intensity involving the superficial lobe. The excision specimen showed a neoplasm with multicystic spaces having papillary projections lined by benign low-grade epithelium and supported by fibrovascular cores. No significant cytological atypia or mitosis was observed. The cells were immunoreactive for Keratin, Keratin 7, and were negative for Keratin 20, AR, HeR2/neu, TTF1, CDX2, and GATA3. p63 and Keratin 5/6 highlighted the myoepithelial cell layer lining the cystic spaces as well as the papillary projections. The Ki-67 proliferation index was 6%. The patient is on close clinical and imaging follow-up for the last 1year and 8 months without any evidence of disease recurrence or metastasis. Rarity of the lesion and distinct histomorphology warrants appropriate knowledge and discussion of the subject.

Apocrine Cystadenoma of the Eyelid, a Rare Neoplasm: Expanded Immunohistologic Profile.

An 83-year-old woman experienced the slow enlargement of a right lower eyelid mass. Histopathologic examination of the excised tissue showed a mucin-filled cystic tumor emanating from an apocrine bilayer that displayed bleb-like apocrine decapitation secretion. The outer flattened myoepithelial layer of the bilayer reacted with immunohistochemical stains for smooth muscle actin and calponin. In foci, the tumor exhibited a cribriform architecture with small pockets of mucin. Tumor cells were reactive for cytokeratin 7, Gross Cystic Disease Fluid Protein 15 (BRST-2), estrogen and progesterone receptors, androgen receptors, mammaglobin, epithelial membrane antigen, and GATA3. Ki67 showed a very low proliferation fraction. The lesion exemplifies the fourth instance of an eyelid apocrine cystadenoma in the literature.

Air Sac Cystadenoma in a Pet Chicken.

A 2-yr-old female Brahma chicken was presented to the Poultry Mobile Clinic of the College of Veterinary Medicine at North Carolina State University with a 3-wk onset of a wet sneeze that progressed to wheezing with a whistle-type sound. Upon observation, a cyst was found above the left clavicle in the area around the crop. The bird was euthanatized due to the progressive and chronic nature of the symptoms. Postmortem examination revealed an ovoid, soft to fluctuant, smooth, pale brown mass (2 × 0.9 × 0.8 cm), encased within the cranial membrane of the left cervical air sac. Histologically, focally expanding the left cervical air sac was a pedunculated, nonencapsulated, well-demarcated, moderately cellular neoplasm that consisted of cuboidal cells predominantly arranged in variably sized cystic structures lined by a single layer of cells. Neoplastic cells have strong cytoplasmic immunolabeling against cytokeratin AE1/AE3. Gross and histologic findings were consistent with an air sac cystadenoma. Primary respiratory neoplasia in birds is infrequent. Air sac carcinomas, adenocarcinomas, and cystadenocarcinomas have been described in Psittaciformes, Columbiformes, Falconiformes, and Cuculiformes. Benign air sac tumors are poorly documented, and detailed descriptions of this neoplasm in poultry literature are lacking.

Reporte de caso- Cistadenoma en los sacos aéreos de un pollo mascota. Una gallina Brahma de dos años fue remitida al Servicio Ambulatorio de Avicultura de traspatio de la Universidad Estatal de Carolina del Norte debido a la presentación de un cuadro clínico de estornudos que progresó a la emisión de ruidos respiratorios tipo silbido en el curso de tres semanas. Se observó un quiste de 2-3 cm de diámetro en el área de la clavícula izquierda alrededor del buche. A dicha ave se le practicó la eutanasia debido a la naturaleza progresiva de los signos. El examen post mortem reveló una masa, ovalada, suave y fluctuante, de color café pálido, de 2 cm × 0.9 cm × 0.8 cm, contenida en la membrana craneal del saco aéreo cervical izquierdo. Histológicamente la pared del saco aéreo cervical izquierdo estaba reemplazada por una neoplasia no encapsulada, pedunculada, bien demarcada, compuesta de células cuboidales organizadas en múltiples estructuras quísticas de tamaño variable y recubiertas por una monocapa celular. Las células neoplásicas poseían una fuerte immunorreactividad citoplasmática para citoqueratina AE1/AE3. Los hallazgos macroscópicos y microscópicos son consistentes con un cistoadenoma de sacos aéreos. En aves, las neoplasias primarias de origen respiratorio son infrecuentes. Carcinomas, adenocarcinomas, y cistoadenocarcinomas de sacos aéreos se han reportado en Psitaciformes, Columbiformes, Falconiformes y Cuculiformes. Los tumores benignos de sacos aéreos han sido escasamente documentados y se carece de descripciones detalladas de estas neoplasias en gallináceas en la literatura.

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.

Laryngeal oncocytic cystadenoma and sudden death.

An 86-year-old woman with Alzheimer disease collapsed in her nursing home and was not able to be resuscitated. At autopsy, the major findings were in the larynx where a pedunculated oncocytic cystadenoma had occluded the glottis. Oncocytic cysts or cystadenomas of the larynx are rare histologically benign lesions that account for only 0.1-1% of laryngeal lesions. While the usual presentation is of a sensation of a mass in the throat, hoarseness, or stridor, very occasionally, there may be acute airway compromise and sudden death. Oncocytic cystadenoma should, therefore, be included in the differential diagnosis of potentially lethal obstructive laryngeal lesions.

Low-grade myofibroblastic sarcoma of the liver misdiagnosed as cystadenoma: A case report.

BACKGROUND: Low-grade myofibroblastic sarcoma (LGMS) is a rare malignant tumor. It has no specific clinical manifestations and commonly occurs in the head and neck, extremities and other body parts, with the liver not as its predisposing site. CASE SUMMARY: We report a case report of a 58-year-old man with right upper abdominal pain for 11 d. Contrast-enhanced computed tomography (CECT), CE magnetic resonance imaging and CE ultrasound (US) all showed a cystic-solid mass in the right liver. As the initial clinical diagnosis was hepatic cystadenoma, surgical resection was performed, and the postoperative pathology indicated hepatic LGMS. The 3-mo follow-up showed favorable recovery of the patient. However, at 7-mo follow-up, two-dimensional US and CECT showed a suspected metastatic lesion in the right-middle abdomen. CONCLUSION: Hepatic MS is particularly rare and easily misdiagnosed, more cases will contribute to the understanding and the diagnosis accuracy.

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.

Sex cord stromal tumors and tumors of the paratestis: new and old entities in a landscape of rare tumors.

PURPOSE OF REVIEW: The 5th edition of WHO classification incorporates the most relevant new data available in the literature regarding tumors of the male genitourinary tract. In this review, the authors summarize and critically discuss the most relevant new information regarding tumors occurring in the stromal testis and in the paratestis that will be reported in the new edition of WHO classification of tumors of the male genitourinary tract. RECENT FINDINGS: Signet-ring stromal tumors (SRST) and myoid gonadal stromal tumors (MGST) are two new entities brought in the 5th WHO classification of testicular tumors. All cases of SRST and MGST reported so far have behaved in a benign fashion after resection and whenever possible a conservative surgery is recommended. A future perspective is to aim at creating large multiinstitutional case series to link different morphologic patterns and molecular bases to the biologic behavior of these neoplasms. Another innovation in WHO consists in the inclusion in the group of Sertoli cell tumors of the sertoliform cystadenoma. The sertoliform cystadenoma is localized in the rete testis and it is of unknown origin. It was included in the group of gonadal stromal tumors because of a high morphological and immunohistochemical similarity to the Sertoli cell tumor. SUMMARY: Although further studies with long-term follow-up are needed to estimate the main oncologic outcomes in patients with rare gonadal stromal tumors, we highlight the importance of an accurate characterization by molecular and immunohistochemical assays of these entities.

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.

Giant Multilocular Prostatic Cystadenoma in a 14-Year-Old Male: A Case Report of a Pediatric Pelvic Mass.

Giant multilocular prostatic cystadenoma (GMC) is an extremely rare, benign tumor seen in both adult and pediatric males. The neoplasm originates from prostatic tissue and is typically found within the rectovesical pouch, varying in both size and morphology. Microscopically, GMC contains both glandular and cystic prostatic tissue lined by cuboidal and columnar epithelium. Symptoms often arise once the pelvic mass begins to obstruct the surrounding structures and organs, although invasion into surrounding tissue is unlikely. Common symptoms include abdominal pain, urinary retention, and dysuria. The standard treatment for GMC is surgical removal of the mass with good outcomes and only 1 known case of recurrence. Here we present the case of a 14-year-old male with GMC-the youngest patient reported to date-who presented with abdominal pain, difficulty voiding, and hydroureteronephrosis.

Emerging Entities in Salivary Pathology: A Practical Review of Sclerosing Microcystic Adenocarcinoma, Microsecretory Adenocarcinoma, and Secretory Myoepithelial Carcinoma.

In recent years, increased molecular testing and improved immunohistochemical panels have facilitated more specific classification of salivary gland carcinomas, leading to recognition of several novel tumor types and unique histologic variants. Sclerosing microcystic adenocarcinoma, microsecretory adenocarcinoma, and secretory myoepithelial carcinoma are three such recently described entities that demonstrate low-grade cytology, production of prominent secretory material, and variable amounts of sclerotic stroma. This review provides a practical overview of these important and overlapping emerging entities in salivary gland pathology with a focus on distinctive histologic features and helpful ancillary studies that differentiate them from a wide range of familiar morphologic mimics.

Sclerosing Polycystic Adenoma.

Sclerosing polycystic adenoma (SPA) is the more appropriate name for sclerosing polycystic adenosis. SPA is an uncommon salivary gland lesion with a constellation of unusual histologic findings that were originally interpreted as analogous to breast fibrocystic changes. The histologic findings in SPA include fibrosis, cystic alterations, apocrine metaplasia, and proliferations of ducts, acini, and myoepithelial cells in variable proportions. Because of its unusual mixed histology, SPA may be confused with a variety of lesions, ranging from reactive conditions to benign or even malignant neoplasms. The features of SPA are reviewed, with an emphasis on resolving its differential diagnosis.

Laparoscopic resection of a large mixed epithelial-stromal tumour of the seminal vesicle: a rare entity and review of the current literature.

Mixed epithelial-stromal tumours (MESTs) of the seminal vesicle (SV) are a rare neoplasm, with biological behaviour ranging from benign to malignant. Due to their rarity, there are no established guidelines for their treatment. We report a 37-year-old man with a large MEST of the SV which was successfully resected by laparoscopic transperitoneal approach. Amidst the controversy regarding the nomenclature and grading of MESTs in literature, we reclassified the previous reports of MESTs incorporating both the WHO and Reikie et al grading.

Laryngeal Oncocytic Cystadenoma with Rare Location and Clinicopathological Aspects.

Laryngeal oncocytic cysts and cystadenomas are uncommon and observed in 0.1-1% of laryngeal biopsy specimens. A 66-year-old male was admitted to the hospital with a hoarse voice of 6 months duration. On laryngoscopic examination, a lesion of the left anterior vocal cord was present. It was excised by direct laryngoscopy and histopathologic examination revealed a cystic cavity with papillary projections into the lumen. The epithelial lining comprised bland oncocytic epithelium. These findings are characteristic of laryngeal oncocytic cystadenoma. Endoscopic excision is curative in these lesions. Oncocytic cystadenoma is a rare, cystic neoplasm that occurs in late adulthood. It may be considered in the clinicopathologic differential diagnosis of laryngeal lesions.