Rare Cystic Lesion Research Articles

Hepatobiliary Cystadenoma and Cystadenocarcinoma: A Single Center Experience

Hepatobiliary cystadenoma and cystadenocarcinoma are rare cystic lesions of the liver. The aim of the study was to discuss the clinical features, diagnostic methods and surgical treatment of hepatobiliary cystadenoma and cystadenocarcinoma in our hospital. Six patients with hepatobiliary cystadenomas and four with hepatobiliary cystadenocarcinomas were evaluated. We collected detailed clinical data, and all patients were followed. Three patients of the 6 with cystadenomas and 2 patients of the 4 with cystadenocarcinomas had marked elevation of CA19-9 (average, 707.0 U/ml and 1078.5 U/ml, respectively). CT scan with contrast revealed typical lesions in all 10 cases, i.e., cyst-occupying lesions with separations in the liver. All patients with hepatobiliary cystadenoma were treated by partial hepatectomy. None of them recurred at a mean follow-up of 40 months. Three patients with hepatobiliary cystadenocarcinoma underwent hepatectomy, without recurrence or metastasis at a mean follow-up of 32 months. Tumor markers (CA19-9) and imaging findings may be helpful for an early diagnosis. Complete resection is still the best choice. Even for hepatobiliary cystadenocarcinoma, considering the low malignant grade, we suggest that for the best prognosis radical excision should be attempted.

Rare cystic liver lesions: A diagnostic and managing challenge

Cystic formations within the liver are a frequent finding among populations. Besides the common cystic lesions, like simple liver cysts, rare cystic liver lesions like cystadenocarcinoma should also be considered in the differential diagnosis. Thorough knowledge of each entity's nature and course are key elements to successful treatment. Detailed search in PubMed, Cochrane Database, and international published literature regarding rare cystic liver lesions was carried out. In our research are included not only primary rare lesions like cystadenoma, hydatid cyst, and polycystic liver disease, but also secondary ones like metastasis from gastrointestinal stromal tumors lesions. Up-to date knowledge regarding diagnosis and management of rare cystic liver lesions is provided. A diagnostic and therapeutic algorithm is also proposed. The need for a multidisciplinary approach by a team including radiologists and surgeons familiar with liver cystic entities, diagnostic tools, and treatment modalities is stressed. Patients with cystic liver lesions must be carefully evaluated by a multidisciplinary team, in order to receive the most appropriate treatment, since many cystic liver lesions have a malignant potential and evolution.

Multiple Apocrine Hidrocystomas: A Case Report

Hidrocystomas are rare cystic lesions that form benign tumours of the sweat glands. In this report, a clinical case of multiple apocrine hidrocystomas on both the upper and lower eyelids, and on both the malar regions has been discussed. These lesions are less likely than the eccrine lesions to occur at the periorbital region.

Diagnosis and treatment of benign multicystic peritoneal mesothelioma

Benign multicystic peritoneal mesothelioma (BMPM) is a rare cystic mesothelial lesion that occurs predominantly in reproductive aged women. A 56-year-old Caucasian male was admitted to our surgical department with a chief complaint of a painful mass in his right lower abdomen for almost 2 years. The physical examination revealed a palpable painful mass. Computed tomography demonstrated an irregular, cystic tumor in his right lower abdomen. There was no obvious capsule or internal septations. No enhancement after intravenous administration of contrast was noted. An exploratory laparotomy was performed, and a multicystic tumor and adherent to the caecum was noted. The walls of the cysts were thin and smooth, filled with clear fluid, and very friable. An en bloc resection of the tumor, including appendix and caecum, was performed. Histological examination revealed multiple cysts lined with flattened simple epithelial cells, and the capsule walls of the cysts were composed of fibrous tissue. Immunohistochemical analysis documented positive expression of mesothelial cells and calretinin. The final diagnosis was BMPM. The patient was well at 6-mo follow-up. BMPM is exceedingly rare lesion. A complete resection of the tumor is required. The diagnosis of BMPM is based on pathological analysis.

Dysgenetic polycystic disease of the parotid gland: Report of a case and review of the literature

Dysgenetic polycystic disease (DPD) is a rare cystic lesion affecting mainly the parotid salivary glands with only 14 reported cases in English literature. DPD of parotids is more common in females, common in younger age group, mostly bilateral in occurrence and commonly affects the superficial lobe. Surgery is performed for symptomatic relief and cosmosis. We report a case of a unilateral DPD of the parotid gland with distinctive histopathology. Patient was treated by performing total parotidectomy with preservation of the facial nerve.

Acinar Cystadenoma of the Pancreas

Pancreatic acinar cystadenomas (ACAs) are rare cystic lesions showing acinar differentiation with benign outcome. Although debated, ACAs are favored to be neoplastic and potentially the benign counterpart of acinar cystadenocarcinoma. We present the largest single institution series to date comprising 10 cases. The mean age was 49 years with a female predominance (M:F=1:2.3). Abdominal/flank pain was the most common presentation (n=6). Serum amylase/lipase and cyst fluid amylase were often elevated. All lesions had a benign outcome on follow-up (5 to 67 mo). The lesions were unilocular (n=3) or multilocular (n=7) with mean size of 3.8 cm (range, 2.9 to 5.0 cm) and 5.1 cm (range, 2.0 to 7.5 cm), respectively. Eight lesions were unifocal with locations as follows: head (n=2), head/neck (n=2), body (n=1), tail (n=1), predominantly extrapancreatic with a microscopic intrapancreatic component (n=1), and unspecified location (n=1). Two lesions were multifocal, involving the head/uncinate/body and pancreatic head, respectively. Two aspects of ACAs that may represent a diagnostic pitfall include the propensity for acinar epithelium to appear as nondescript flat/cuboidal epithelium (trypsin/chymotrypsin immunopositive) and epithelial heterogeneity, with focal mucinous and squamous epithelium, the latter particularly in multilocular variants. In addition, 2 cases with intracystic nodules were observed. Array comparative genomic hybridization performed on 1 of these cases showed multiple chromosomal gains involving 1p, 3p, 5q, 6p, 7q, 8, 10q, 11, 14, 20, and X. These findings provide preliminary evidence that ACAs represent a cystic neoplastic lesion.

Endonasal Endoscopic Approach for Recurrent Nasolabial Cyst

Nasolabial cysts are rare nonodontogenic cystic lesions in the soft tissue of the midface and usually present with painless asymptomatic swelling in the nasolabial region. Surgical excision via sublabial approach is considered as the standard treatment. However, endonasal endoscopic marsupialization of the cyst is reported as an effective alternative method for nasolabial cysts. Marsupialization of the cyst is believed to integrate the nasolabial cyst into a part of the nasal cavity as an air-containing sinus. In this article, we present an endonasal endoscopic marsupialization technique in case of recurrent nasolabial cyst. To draw attention to this approach, we present our case with current literature.

Cutaneous Ciliated Cyst on the Finger

As previously recognized by various authors, "cutaneous ciliated cyst" is a confusing term. Typically, the term refers to rare cystic lesions, commonly found on the lower limbs of women in their reproductive years. To date, 40 cases diagnosed as "cutaneous ciliated cyst" have been reported in the literature. Histologically, the cysts are composed of a simple layer of ciliated columnar cells along with nonciliated columnar cells, cuboidal cells, and round "peg-like" cells, resembling fallopian tube epithelium. This histology has been described in cysts found in males and females and in locations other than the lower limbs. Controversy has thus arisen over the etiology of these lesions, with some believing that the cysts arise from heterotopic Mullerian rests and others advocating for ciliated metaplasia of eccrine glands. We herein describe the first case of cutaneous ciliated cyst of Mullerian origin occurring on the dorsal thumb of a 16-year-old female. A review of literature shows that 2 groups of cysts are covered under the umbrella term "cutaneous ciliated cysts." We thus propose the abandonment of the confusing term "cutaneous ciliated cyst" and the adoption of "cutaneous Mullerian cysts" for estrogen receptor/progesterone receptor-positive lesions resembling simple fallopian tube epithelium and "Cutaneous ciliated eccrine cyst" for estrogen receptor/progesterone receptor-negative lesions usually occurring in males, which are immunohistochemically compatible with an eccrine origin.

Lymphoepithelial Cysts of Oral Mucosa: Two Cases in Different Regions

Lymphoepithelial cyst of the oral cavity is a rare cystic lesion that presents as an asymptomatic, well-circumscribed, yellowish small submucosal nodule covered by normal overlying mucosa, usually located in the floor of the mouth or in the ventral or posterolateral surface of the tongue. Histopathological examination reveals a cyst lined by a stratified squamous epithelium surrounded by lymphoid tissue. In this paper, we report two cases of oral lymphoepithelial cyst, one on the anterior floor of the mouth and another on the posterior lateral tongue. In both cases, the patients were women aged approximately 50 years and the lesions were treated by local surgical excision, with no evidence of recurrence after 2 years of follow-up.

Multilocular Cystic Renal Cell Carcinoma: Comparison of Imaging and Pathologic Findings

The purpose of this study was to retrospectively correlate the imaging and pathologic features of multilocular cystic renal cell carcinoma (RCC), a low-grade neoplasm that has an excellent prognosis. Institutional databases were searched for the period between 2001 and 2010 to identify cases of resected renal tumors that had been evaluated with CT or MRI and been analyzed by a uropathologist to confirm the histologic diagnosis of multilocular cystic RCC. The images (nine CT, 14 MRI) were reviewed, and a Bosniak cyst category was assigned. Of 23 confirmed cases of multilocular cystic RCC, imaging revealed seven lesions were Bosniak category IIF, 13 were category III, and three were category IV. Pathologic examination of the category IIF lesions revealed 99% fluid, 0.001-1% clear cells lining the septum, and 0% fibrosis. The category III lesions were 98-99% fluid, 1-2% clear cells, and 0% fibrosis. The category IV lesions were 20-40% fluid, 1-5% clear cells, and 60-80% fibrosis. The patient demographics were similar across groups. Clinical follow-up showed no evidence of recurrent or metastatic disease. Multilocular cystic RCC is a rare cystic lesion of the kidney that is low risk to the patient and benign in behavior. It has a variable imaging pattern, the Bosniak category ranging from IIF to IV. As multilocular cystic RCC lesions increase in complexity on images (higher Bosniak category), there is a corresponding increase in the volume of malignant cells lining the tumor and an increase in the presence of vascularized fibrous tissue. Regardless of the imaging appearance, the behavior of these tumors was benign in this study. Clinicians and radiologists should be aware that when this carcinoma is reported to occur, the patient has an excellent prognosis.

Thoracic Duct Cyst in Mediastinum - A case report -

The thoracic duct cyst is an extremely rare cystic lesion in the mediastinum. Surgical treatment of the cyst is necessary to confirm histologic diagnosis and prevent potential complications such as spontaneous or traumatic rupture of the cyst and chylothorax.

Marsupialization of juvenile paradental cyst: A case report

Juvenile paradental cysts (JPC) are rare cystic lesions that usually develop on buccal, distal, or mesial side of first, and rarely second permanent mandibular molars. They occur during tooth eruption, in children aged 6 to 12 years, and belong to the group of inflammatory paradental cysts. Clinical symptomatology is scarce and often manifested as appearance of painless swelling on the buccal side of the offending tooth. Different therapeutic procedures could be used: enucleation, enucleation with tooth extraction or marsupialization. This study presents the marsupialization procedure of bilateral JPC attached to the first permanent lower molars in seven year old girl.

Sublingual-plunging ranula as a complication of supraomohyoid neck dissection

Ranulas are rare cystic lesions resulting from damage or rupture of one or more of the ducts of the sublingual gland, that lead to mucus extravasation or dilatation of the gland's duct. Extravasation cysts are more common than retention cysts. We present a case of a 45-year-old male with a squamous cell carcinoma of the ventral surface of the tongue that was treated with excision of the oral lesion and bilateral supraomohyoid neck dissection without supplementary radiotherapy. A left myocutaneous platysma flap was raised for defect closure. Ten months postoperatively he presented complaining of swelling of the right submandibular region. The diagnosis, based on his medical anamnesis and the CT imaging, was a sublingual-plunging ranula. It is postulated that the ranula resulted from damage to the ducts of the sublingual gland during selective neck dissection. One year postoperatively there are no signs of recurrence either of the ranula or of the cancer. We suggest that sublingual gland excision and intraoral cyst marsupialization is a logical treatment for sublingual-plunging ranulas.

Microendoscopic Resection of Lumbar Discal Cysts

A lumbar discal cyst is a relatively rare cystic lesion that communicates with lumbar intervertebral discs. Surgical resection of the cyst is the reported treatment of choice. In this study, the authors report the minimally invasive surgical resection of lumbar discal cysts using a microendoscopy. Seven male patients with lumbar discal cysts underwent microendoscopic resections (mean age: 25.1+/-3.2 years and the mean follow-up period: 27.9 months). During the surgeries, the cysts were subtotally resected in a piecemeal fashion, and the fistulas forming the communications between the cysts and the corresponding intervertebral discs were coagulated using a bipolar coagulator. All the patients obtained relief from their pain after surgery, and no recurrences occurred during a mean follow-up period of 28 months. The mean operation time was 72.6+/-20.2 min, and the mean blood loss was 44.4+/-13.7 grams. No intra- or peri-operative complications were noted in any of the patients. Microendoscopic resection appears to be a minimally invasive and feasible surgical option for the treatment of lumbar discal cysts.

Discography for disc cysts is obsolete

Dear Editor,Werecentlyencounteredadisccystina32-year-oldmanwithrefractory low back pain and radiation into the left L5dermatome since almost 1 year. MRI revealed a liquid-containingcystwithextensionintotheleftlateralrecessofL5and scalloping of the adjacent vertebral body (Fig. 1). Thisradiological sign is indicative of a long-standing process as itis the result of bone remodeling. The cyst caused dorsaldisplacement and significant compression of the thecal sacand left L5 root. The cyst was removed through a left L4hemilaminectomy. A small annular fissure on the L4–L5 discwas noted after cyst removal, and subsequently, a discec-tomy was performed. After surgery, the low back pain andsciatica disappeared. Histological examination of the cystwall exhibited chondrocytes and fibrillary stroma of discmaterial with areas of myxoid degeneration.Disc cysts (also known as discal cysts) are rare cysticlesions in the ventrolateral epidural space. Theyare located atL4–L5 more often thanat other lumbar levels, and they occurmostly in young men who present with refractory low backpain and sciatica. Disc cysts were first described one decadeago and distinguished from ganglion cysts of the posteriorlongitudinal ligament (PLL) by means of their cyst–disccommunication at discography [4]. Ganglion cysts of thePLL [7] and disc cysts differ from juxtafacet cysts [10]byvirtue of a ventral location, an intimate or pedicledattachment to the annulus fibrosus or PLL, and absentconnection with a degenerative facet joint. Unlike juxtafacetcysts, disc cysts and ganglion cysts of the PLL are bothwalled-in by fibrous connective tissue without a synoviallining and frequently show myxoid degeneration. Chondro-cytes could be demonstrated in the cyst wall of our case andin only a very few other disc cysts reported in the literature[9]. Because of the clinical, radiological, surgical, andhistological similarities between disc cysts and ganglioncysts, Marshman et al. [6] suggested that their distinction onthe basis of disc space communication is unnecessary. Forthe same reason, they also proposed to use the term “disccyst” for both disc cysts and ganglion cysts of the PLL.Discography is an invasive diagnostic procedure inwhich radiopaque contrast medium is injected into thenucleus pulposus. In disc cysts, this technique can be usedto assess disc–cyst communication and to reproduceradiating pain into the affected leg [8]. As explained above,disc–cyst communication at discography was originallyemployed as a means to distinguish disc cysts fromganglion cysts of the PLL. Nevertheless, discography wasnot used in nearly all of the reported ganglion cysts of thePLL, and the same is true for some reports on disc cysts [2,3, 5]. The reason is that patients were either treatedconservatively [2, 3] or the disc–cyst communication wasevaluated surgically [5]. This can be achieved by directvisualization of a connection between the cyst and itscorresponding disc or by observation of a defect in theannulus fibrosus after cyst removal [6, 8]. The onlyremaining importance of disc–cyst communication lies inthe fact that its presence is generally regarded as anindication for discectomy in addition to cystectomy.

Biliary cystadenocarcinoma with mesenchymal stroma

Hepatobiliary cystadenocarcinomas (BCACs) with mesenchymal stroma are a rare cystic lesion. This tumour needs to be distinguished from benign biliary cystadenoma, which is antecedent in most cases. The treatment of choice is radical excision of the mass. The diagnostic evaluation, surgical management, pathological characteristics, treatment and follow-up of one patient with hepatobiliary cystadenocarcinoma with ovarian stroma is described. Preoperative diagnosis of BCACs is often difficult, because their clinical manifestations are similar to those of other hepatic cystic lesions. MRI is suitable for accurate characterisation of cystic biliary lesions, but distinguishing between cystadenoma and cystadenocarcinoma remains difficult on the basis of imaging findings. Complete surgical excision gives a relatively good chance of long-term survival because of the slow growth rate of these tumours.

Mucinous nonneoplastic cyst of the pancreas: apomucin phenotype distinguishes this entity from intraductal papillary mucinous neoplasm

Mucinous nonneoplastic cyst of the pancreas is a newly described and rare cystic lesion with unknown histogenesis. It is defined as a cystic lesion lined with mucinous epithelium, supported by hypocellular stroma and not communicating with the pancreatic ducts. It is very challenging to differentiate this lesion from other cystic mucinous neoplasms of the pancreas such as branch-duct intraductal papillary mucinous neoplasm by morphology. In this study, a total of 436 pancreatic specimens resected between 2002 and 2007 in our institution were reviewed. Fifteen (3.4%, 15/436) mucinous nonneoplastic cysts were identified. They included 3 males and 12 females, with a median age of 60 years. Forty-six percent of cases (7/15) occurred in pancreatic head, 27% (4/15) in neck, 7% (1/15) in body, and 20% (3/15) in tail. The size of lesions ranged from 0.5 to 3.5 cm in greatest dimension. In most cases (12/15, 80%), mucinous nonneoplastic cyst was associated or adjacent to acinar-ductal mucinous metaplasia. These morphologic data indicate that mucinous nonneoplastic cyst is not really a rare disease and may originate from acinar-duct mucinous metaplasia histogenestically. Furthermore, apomucin immunostains of mucinous nonneoplastic cyst showed MUC1 expressed in 27% (4/15) cases, MUC5AC in 67% (10/15 cases), and MUC2 was were negative in all cases, whereas intraductal papillary mucinous neoplasm (n = 17; 5 main duct type, 12 branch-duct type) showed focal and weak MUC1 positivity in 18% (3/17) cases, MUC2 positivity in 71% (12/17) cases, and all intraductal papillary mucinous neoplasm (17/17) were MUC5AC positive. The clonality assay with the HUMARA gene revealed that the mucinous nonneoplastic cysts were of polyclonal origin. For the first time, using HUMARA assay, we demonstrate the nonneoplastic nature of these cysts and further characterize morphologic and immunophenotypic properties that allow differentiation from intraductal papillary mucinous neooplasm.

Subpubic cartilaginous cystic lesion presenting as a vulvar mass: a case report

IntroductionA subpubic cartilaginous cyst is a rare and innocent defect originating from the symphysis pubis and may present as a vulvar mass and/or chronic abdominal pain. The symphysis pubis is a non-synovial amphiarthrodial joint that forms a fibrocartilaginous union between the two pubic bones and in general lies without mainstream interest, despite its clinical relevance. This case report focuses attention on this joint.Case presentationA 55-year-old Caucasian woman presented with a painful and rapidly increasing vulvar mass. Imaging techniques revealed a cyst-like structure originating from a degenerated symphysis pubis. The cyst was excised, however, recovery was complicated by a symphysiolysis.ConclusionA subpubic cartilaginous cyst is a rare cystic lesion originating from the symphysis of the pubic bone which presents as a vulvar mass which might be complicated by symphysiolysis. Several diseases affect the symphysis pubis and result in significant discomfort and disability in patients. The symphysis pubis is therefore clinically important and should not be omitted in the differential diagnosis of a vulvar mass and/or chronic abdominal pain.

Laparoscopic Excision for an Enlarged Ciliated Hepatic Foregut Cyst as a Minimally Invasive Procedure

Ciliated hepatic foregut cyst (CHFC) is a very rare cystic lesion of the liver. Although CHFC has traditionally been considered to lack malignant potential, recent reports have shown the malignant transformation of this lesion into squamous-cell carcinoma. It is difficult to differentiate CHFC from malignant tumor, and it is important to consider in aspiration cytology for the evaluation of possible neoplastic disease. In this paper, we describe the case of a 57-year-old female who underwent a laparoscopic excision of an enlarged CHFC that was diagnosed by fine-needle aspiration cytology. Laparoscopic excision may be adopted as a minimally invasive surgical procedure for CHFC.

Placental Transmogrification of the Lung Presenting as a Small Solitary Nodule

Placental transmogrification of the lung is a rare cystic lesion of the lung, which has some histologic resembling to placental tissue. Placental transmogrification of the lung has been considered a variant of unilateral bullous emphysema, but in our case, the patient was a 47-year-old man who had no coexisting emphysema of the lung. Histologically our case showed an interstitial proliferation of clear cells with cystic change interpreted as secondary; we also agree with our colleagues that this proliferation may be the primary event pathogenetically. Surgical resection of this lesion seems to be curative.