OBJECTIVES: Pulmonary Langerhans cell histiocytosis (PLCH) is a clonal disease, characterized by proliferation of Langerhans cells that derived from bone marrow infiltrating the lungs and other organs. Due to the rarity of the disease, the current understanding of the disease is insufficient, often misdiagnosed or missed diagnosis. This study aims to raise clinicians' awareness for this disease via summarizing the clinical characteristics, imaging features, and treatment of PLCH. METHODS: We retrospectively analyzed clinical and follow-up data of 15 hospitalized cases of PLCH from September 2012 to June 2021 in the Second Xiangya Hospital of Central South University. RESULTS: The age of 15 patients (9 men and 6 women, with a sex ratio of 3 to 2) was 21-52 (median 33) years. Among them, 8 had a history of smoking and 5 suffered spontaneous pneumothorax during disease course. There were 3 patients with single system PLCH and 12 patients with multi-system PLCH, including 7 patients with pituitary involvement, 7 patients with lymph node involvement, 6 patients with bone involvement, 5 patients with liver involvement, 2 patients with skin involvement, 2 patients with thyroid involvement, and 1 patients with thymus involvement. The clinical manifestations were varied but non-specific. Respiratory symptoms mainly included dry cough, sputum expectoration, chest pain, etc. Constitutional symptoms included fever and weight loss. Patients with multi-system involvement experienced symptoms such as polyuria-polydipsia, bone pain, and skin rash. All patients were confirmed by pathology, including 6 by lung biopsy, 3 by bone biopsy, 2 by lymph node biopsy, and 4 by liver, skin, suprasternal fossa tumor, or pituitary stalk biopsy. The most common CT findings from this cohort of patients were nodules and/or cysts and nodular and cystic shadows were found in 7 patients. Three patients presented simple multiple cystic shadows, 3 patients presented multiple nodules, and 2 patients presented with single nodules and mass shadows. Pulmonary function tests were performed in 4 patients, ventilation dysfunction was showed in 2 patients at the first visit. Pulmonary diffusion function tests were performed in 4 patients and showed a decrease in 3 patients. Smoking cessation was recommended to PLCH patients with smoking history. Ten patients received chemotherapy while 2 patients received oral glucocorticoid therapy. Among the 11 patients with the long-term follow-up, 9 were in stable condition. CONCLUSIONS PLCH is a neoplastic disease closely related to smoking. The clinical manifestations and laboratory examination are not specific. Pneumothorax could be the first symptom which is very suggestive of the disease. Definitive diagnosis relies on histology. There is no unified treatment plan for PLCH, and individualized treatment should be carried out according to organ involvement. Early smoking cessation is essential. Chemotherapy is the main treatment for rapidly progressing PLCH involved multiple organs. All diagnosed patients can be considered for the detection of BRAF^V600E gene and relevant targeted therapies have been implemented recently.
Adult ; Cysts ; Female ; Histiocytosis, Langerhans-Cell/therapy* ; Humans ; Lung/pathology* ; Male ; Retrospective Studies ; Smoking/adverse effects* ; Smoking Cessation

The diagnosis of cystic liver disease has made great progress with the advent of enhanced imaging techniques. At the same time, its management has gradually improved over the past few decades, providing the basis for the development of appropriate diagnostic and treatment guidelines. To this end, the European Association for the Study of the Liver has developed clinical guidelines for the diagnosis and treatment of non-infectious cystic liver disease. This guideline put forward recommendations based on an in-depth review of the relevant literature for addressing clinical issues, including the diagnosis and treament of hepatic cysts, hepatic mucocystic tumors, biliary hamartomas, polycystic liver disease, Caroli disease or Caroli syndrome, biliary hamartomas, and peribiliary cyst.
Humans ; Liver Diseases/pathology* ; Cysts/pathology* ; Caroli Disease/diagnosis* ; Liver Neoplasms/therapy* ; Hamartoma

Bone Cysts, Aneurysmal/pathology* ; Fasciitis/pathology* ; Gene Rearrangement ; Humans ; Ubiquitin Thiolesterase

BACKGROUND: The absence of thyroid cysts may indicate latent thyroid damage, as demonstrated in our previous study. However, the association between the absence of thyroid cysts and latent functional damage of the thyroid is unknown. At low thyroid hormone productivity, which may be associated with latent functional damage of the thyroid, the association between thyroid-stimulating hormone (TSH) and hypertension might be enhanced. Therefore, we evaluated the association between TSH level and hypertension stratified by thyroid cyst status. METHODS: We conducted a cross-sectional study of 1724 euthyroid Japanese individuals aged 40-74 years who participated in an annual health checkup in 2014. RESULTS: In the study population, 564 and 686 participants had thyroid cysts and hypertension, respectively. A significant positive association was observed between TSH and hypertension in subjects without a thyroid cyst but not in subjects with thyroid cysts. There was a significant positive association between hypertension and TSH in subjects without a thyroid cyst (odds ratio [OR] 1.27; 95% confidence intervals [CI] 1.01, 1.61) but not in subjects with thyroid cysts (OR 0.79; CI 0.57, 1.09) in the model fully adjusted for known confounding factors. The correlation between the TSH and free triiodothyronine (fee T3) levels (simple correlation coefficient [r] = - 0.13, p < 0.01) was stronger in the subjects without thyroid cysts than in those with thyroid cysts (r = - 0.03, p = 0.525). CONCLUSIONS TSH is positively associated with hypertension only in individuals without thyroid cysts. The correlation between the TSH and free T3 levels was stronger in the subjects without thyroid cysts than in those with thyroid cysts. Therefore, the absence of thyroid cysts could be related to the association between TSH level and hypertension, possibly by indicating that the subjects without thyroid cysts had limited thyroid hormone reserves. Therefore, the absence of thyroid cysts could indicate the latent functional damage of the thyroid.
Aged ; Cross-Sectional Studies ; Cysts/etiology* ; Female ; Humans ; Hypertension/metabolism* ; Japan ; Male ; Middle Aged ; Thyroid Diseases/etiology* ; Thyroid Gland/pathology* ; Thyrotropin/metabolism*

Adolescent ; Ciliary Body ; pathology ; surgery ; Cysts ; surgery ; Glare ; Humans ; Male ; Neodymium ; chemistry ; Yttrium ; chemistry

PURPOSE: The prevalence of supernumerary teeth has been reported to be between 0.1% and 3.8%. The aim of this study was to determine the prevalence, clinical significance, and associated pathologies of fourth molars based on a retrospective study and a literature review. MATERIALS AND METHODS: A 5-year retrospective prevalence study was conducted at the Department of Oral Diagnosis and Dentomaxillofacial Radiology of Okayama University Hospital, Okayama, Japan. The study involved extracting data from the digital records of patients from January 1, 2013 through December 31, 2017. The sampling frame included all patients who had panoramic radiographs, cone-beam computed tomography (CT), and multislice CT images during the period under review. RESULTS: A total of 26,721 cases were reviewed and 87 fourth molars were identified. The prevalence of fourth molars in the 5-year study at Okayama was calculated as 0.32%. The mean age of patients with a fourth molar was 30.43 years, and the male-to-female ratio was 1:0.98. The vast majority of cases were in the maxilla (92%) and had normal shapes (89.7%); furthermore, 82.8% of cases were unerupted. CONCLUSION: The prevalence of fourth molars in the study population was found to be 0.32%, and fourth molars occurred with approximately equal frequency in males and females. Fourth molars were more common in the maxilla and were predominantly unerupted and small.
Cone-Beam Computed Tomography ; Cross-Sectional Studies ; Diagnosis, Oral ; Female ; Humans ; Japan ; Male ; Maxilla ; Odontogenic Cysts ; Odontoma ; Pathology ; Prevalence ; Retrospective Studies ; Tooth, Supernumerary

BACKGROUND/AIMS: Previous studies from Korea have described chronic intestinal pseudo-obstruction (CIPO) patients with transition zone (TZ) in the colon. In this study, we evaluated the pathological characteristics and their association with long-term outcomes in Korean colonic pseudo-obstruction (CPO) patients with TZ. METHODS: We enrolled 39 CPO patients who were refractory to medical treatment and underwent colectomy between November 1989 and April 2016 (median age at symptoms onset: 45 [interquartile range, 29–57] years, males 46.2%). The TZ was defined as a colonic segment connecting a proximally dilated and distally non-dilated segment. Detailed pathologic analysis was performed. RESULTS: Among the 39 patients, 37 (94.9%) presented with TZ and 2 (5.1%) showed no definitive TZ. Median ganglion cell density in the TZ adjusted for the colonic circumference was significantly decreased compared to that in proximal dilated and distal non-dilated segments in TZ (+) patients (9.2 vs 254.3 and 150.5, P < 0.001). Among the TZ (+) patients, 6 showed additional pathologic findings including eosinophilic ganglionitis (n = 2), ulcers with combined cytomegalovirus infection (n = 2), diffuse ischemic changes (n = 1), and heterotropic myenteric plexus (n = 1). During follow-up (median, 61 months), 32 (82.1%) TZ (+) patients recovered without symptom recurrence after surgery. The presence of pathological features other than hypoganglionosis was an independent predictor of symptom recurrence after surgery (P = 0.046). CONCLUSIONS: Hypoganglionosis can be identified in the TZ of most Korean CPO patients. Detection of other pathological features in addition to TZ-associated hypoganglionosis was associated with poor post-operative outcomes.
Cell Count ; Colectomy ; Colon ; Colonic Pseudo-Obstruction ; Cytomegalovirus Infections ; Eosinophils ; Follow-Up Studies ; Ganglion Cysts ; Humans ; Intestinal Pseudo-Obstruction ; Korea ; Male ; Myenteric Plexus ; Pathology ; Recurrence ; Ulcer

ObjectiveTo investigate the diagnosis and treatment of ejaculatory duct cyst.

METHODSThis study included 2 male patients present at the hospital for hemospermia and abnormal sensation in the perineal region in July and August 2014. Both underwent transrectal ultrasonography, routine semen examination, CT, MRI, cystoscopy, and vesiculography before transurethral fenestration of the cysts and pathological examination of the cyst wall specimens. Analyses were made on the clinical presentations, imaging features, pathological characteristics, differential diagnosis and treatment of ejaculatory duct cyst and relevant literature was reviewed.

RESULTSThe cyst wall was mainly composed of smooth muscle, the inner wall lined with pseudostratified ciliated columnar epithelia, and with positive expressions of CD10 and Muc6 proteins on immunohistochemical staining, which indicated renal iatrogenic ejaculatory duct cyst. The patients were followed up for 18 and 20 months, respectively. All symptoms disappeared and no recurrence occurred after surgery. Routine semen examination for the two patients showed the semen volumes to be 3.5 and 3.1 ml, sperm concentrations 35 and 32 ×10⁶/ml, grade a sperm 32.0 and 26.0%, grade b sperm 18.0 and 31.0%, and semen liquidation time 30 and 34 minutes, respectively.

CONCLUSIONSPelvic cystic masses can be detected by transrectal ultrasonography, CT and MRI, but definite diagnosis relies on vesiculography, pathological examination and immunohistochemical staining. Transurethral fenestration is safe and effective for the treatment of ejaculation duct cyst.

Cysts ; diagnostic imaging ; pathology ; surgery ; Ejaculation ; Ejaculatory Ducts ; diagnostic imaging ; pathology ; surgery ; Genital Diseases, Male ; diagnostic imaging ; pathology ; surgery ; Hemospermia ; etiology ; Humans ; Magnetic Resonance Imaging ; Male ; Neoplasm Recurrence, Local ; Semen ; Semen Analysis ; Sperm Count ; Spermatozoa ; Tomography, X-Ray Computed ; Ultrasonography

OBJECTIVETo compare technique and clinical effect of arthroscopic and conventional sectional therapy for the treatment of ischial tuberosity cyst.

METHODSFrom May 2014 and September 2016, 49 patients with symptomatic ischial tuberosity cyst were randomized divided into arthroscopic group and conventional section group by envelope method. There were 24 patients in arthroscopic group, including 16 males and 8 females aged from 42 to 81years old with an average age of (64.1±9.3) years old;the courses of disease ranged from 2 to 36 months with an average of (17.0±9.1) months;treated with removing cyst wall under arthroscopic and build artificial lacuna around cyst. There were 25 patients in conventional section group, including 11 males and 14 females aged from 47 to 79 years old with an average of (61.2±10.6) years old; the courses of disease ranged from 4 to 36 months with an average of (17.5±8.5) months;treated with cutting off lump with transverse incision. Operative time, blood loss, fluid volume, hospital stays and postoperative complication were observed and compared, VAS score were applied to evaluate pain degree.

RESULTSForty-nine patients were followed up from 6 to 18 months with an average of (11.3±3.3) months. In conventional group, 2 patients occurred incision infection, 1 case reoccurred. All patients in arthroscopic group were healed at stage I. Operative time, blood loss, fluid volume, and hospital stays in arthroscopic group were (54.7±7.7) min, (20.8±3.5) ml, (20.3±5.6) ml, (2.8±0.6) d respectively and better than that of conventional group(71.8±8.8) min, (67.3±12.0) ml, (103.6±20.3) ml, (7.8±2.9) d. Postoperative VAS score in arthroscopic group on the first day, seventh days and first month were 2.6±0.7, 0.5±0.6, 0.3±0.5, and significantly lower than that of conventional group 6.0±0.7, 3.0±1.0, 1.1±1.0 respectively(<0.05). There were no statistical difference in postoperative complications between two groups(>0.05).

CONCLUSIONSCompared with conventional group, arthroscopic for ischial tuberosity cyst has advantages of minimal invasive, less blood loss during perioperative period, less pain degree after surgery, safety and rapid recovery. But arthroscopic skills are needed for surgeons. It should be recommended in clinical practice.

Adult ; Aged ; Aged, 80 and over ; Arthroscopy ; Bone Cysts ; surgery ; Case-Control Studies ; Female ; Humans ; Male ; Middle Aged ; Operative Time ; Pelvic Bones ; pathology ; Postoperative Period ; Prospective Studies ; Treatment Outcome

Aneurysmal bone cyst (ABC) is a rare non-neoplastic bone lesion that involves mostly the long bones and vertebrae and may occur very rarely in the craniofacial bones. ABCs may occur as secondary bony pathologies in association with various benign and malignant bone tumors and with fibrous dysplasia (FD). FD is a common non-neoplastic bony pathology mostly affecting craniofacial bones. Secondary ABC occurring in craniofacial FD is extremely rare, with only approximately 20 cases reported in the literature to date. Here, we report on a case of secondary ABC in a 25-year-old woman who has had a craniofacial deformity for over 10 years and who presented to us with a rapidly growing painful pulsatile mass in the right frontal region that began over 2 months prior to admission. On thorough examination of computed tomography and magnetic resonance imaging brain scans taken at two-month interval, an aggressive, rapidly enlarging ABC, arising from the right frontal FD, was diagnosed. The patient underwent preoperative embolization followed by gross total resection of the ABC and cranioplasty. The 6-month follow up showed no recurrence of the ABC, nor was any progression of the FD noticed.
Adult ; Aneurysm* ; Bone Cysts* ; Bone Cysts, Aneurysmal ; Brain ; Congenital Abnormalities ; Craniotomy ; Female ; Fibrous Dysplasia of Bone ; Follow-Up Studies ; Frontal Bone ; Humans ; Magnetic Resonance Imaging ; Pathology ; Recurrence ; Spine