Supplemental Digital Content is available in the text Adenoid cystic carcinoma (ACC) is an infrequent malignant neoplasm of the salivary glands. The clinical and pathological characteristics include slow growth, perineural invasion, and local recurrence. ACC of the mobile tongue is rarely reported in the literature.We describe a case of a 43-year-old man patient with numbness and growth in the tongue for 1 month duration. The patient reported a long-term smoking and drinking habit.A biopsy was carried out and histopathological analysis confirmed as diagnosis of adenoid cystic carcinoma. Computed tomography (CT) of the head and neck enhanced scanning revealed an ill-defined high density mass with altered enhanced signal entities involving the anterior 2/3rd of the tongue.The patient was treated with surgery and adjuvant radiotherapy. Incision was placed over the anteriorly till 2/3rd of tongue and then the femoral anterolateral free flap was used to repair and reconstruct the defect of tongue.The patient is currently under a postsurgical 29-month regular follow-up, showing good swallowing and speaking function without any recurrence and metastasis.This case report suggests that surgery and adjuvant radiotherapy are suitable procedures for patients with resectable ACC of the mobile tongue. ACC was described by Billroth as “cylindroma” and has explained its recurrent tendency in 1859. Foote and Frazell designated as “adenoid cystic carcinoma” in 1953. The sites of ACC include the minor or major salivary glands, and the most common minor salivary gland site is the hard palate, followed by the base of the tongue. Typical clinical performances include slow indolent growth, local recurrence, perineural invasion, and distant metastasis, mainly to the lungs and bones. We present a rare case of a 43-year-old male patient with a c-kit positive mobile tongue ACC treated with surgery and adjuvant radiotherapy and carried out a brief literature review on the subject.Adenoid cystic carcinoma (ACC) is a relatively uncommon malignant tumor that originates in the salivary glands. The peak incidence of ACC is between the fifth and seventh decades of life, which occurs predominantly among women.2A 43-year-old male patient presented to hospital, complaining of volume growth of the tongue with numbness for the past 1 month before consultation. The patient reported a long-term smoking and drinking habit.This study was conducted with the approval of Medical Ethics Committee of Chongqing Cancer Hospital, and was performed in accordance with the ethical standards of the Helsinki Declaration. Written informed consents for his data and images to be used for our study and publication were obtained from patient before operation.On intraoral clinical examination and palpation, an obvious smooth firm mass of about 2 cm in diameter on the mobile tongue with the same color as that of the surrounding mucosa was observed without other oral lesions. The cervical lymph nodes were not swollen on palpation. A biopsy was carried out and histopathological analysis demonstrated tissues formed by adenoid cystic carcinoma. Computed tomography (CT) of the head and neck enhanced scanning revealed an ill-defined measuring 23 mm × 19 mm high density mass with altered enhanced signal entities involving the anterior 2/3rd of the tongue. There was no obvious abnormality in the adjacent mandibular bone. No obvious enlarged lymph nodes were seen in both sides of the neck  was used to repair the defect of tongue and preserve the swallowing and speech function. During the intervention, a preventive tracheotomy was carried out to ensure breathing , CK8, epithelial membrane antigen (EMA), Ki-67(10%), and P63, while negative for carcinoembryonic antigen (CEA) and S-100  has been reported to grow slowly, with a high recurrence rate, and a high chance of metastases. Perineural invasion into perineural spaces, or within the nerve itself, is a typical feature of ACC. Distant metastases often occur in the lungs and bones, and lymph node metastasis and blood dissemination are rare. Among ACCs of the head and neck regions, the site of origin has been reported to be the tongue in between 3.4% and 17.1% of cases, with the mobile tongue reported to be the site of origin in 2.9% of cases. reported 10-year survival rates of 16.7% in ACCs with a solid histological pattern and of 47.4% in ACCs with cribriform or tubular patterns. Moreover, Sequeiros et al reported that patients with a solid histological pattern presented with the worst prognosis, with 10-year survival rates of 34%, in contrast to 76% for the cribriform and 100% for the tubular patterns, and they suggested that the solid pattern is associated with recurrences, early distant metastases, and a higher mortality rate.Histopathologically, ACC consists of a mixture of ductal epithelial cells and myoepithelial cells, and the 2 kinds of cells are arranged in 3 different patterns, namely, solid, tubular, and cribriform. Several studies have confirmed a relationship between histologic patterns and prognosis. Huang et al,5 Compared with other malignant neoplasms, ACCs are more difficult to completely remove, and positive surgical margins occur frequently. Tongue ACCs have rarely been reported. Luna-Ortiz et al found that the overall 5-year and 10-year survival rates for tongue ACCs were 51% and 34%, respectively, and that pulmonary metastases develop in 37% of patients.The primary treatment approach for ACC is local control, functional recovery, and distant metastasis prevention. Surgery is the mainstay of treatment, with long-term follow-up.Surgery for ACC of the tongue may comprise a partial glossectomy, a hemi-glossectomy, or a total glossectomy with or without reconstruction, with attention to margins and the presence of perineural invasion. Decisions concerning surgical excision should focus on patient quality-of-life outcomes, including preservation of speech and swallowing. Therefore, we suggest that large defects post-excision should be repaired with an appropriate free flap such as a radial forearm free flap or an anterolateral thigh flap (ALTF).Reconstruction of a tongue defect is challenging, given its comprehensive functions including articulation, deglutition, and taste. Since ALTF is used in 1984, tongue reconstruction has become increasingly popular and emerged as the “workhorse flap” for tongue defect reconstruction, as this form of flap is both reliable and versatile. Other advantages of this workhorse flap are that it is easy to raise, with relatively soft tissue volume and a long vascular pedicle. The ALTF can supply a large amount of soft tissue for complex tongue defects involving the tongue and the floor of the mouth that require accurate flap design and tailoring. When selecting a flap for tongue reconstruction, an ALTF is an excellent surgical option entailing less damage at the donor site than a radial forearm flap. In recent years, tyrosine kinase inhibitors have been used to treat ACC as an adjuvant therapeutic method in managing distant metastases. Andreadis et al found that most ACCs of the salivary glands stained positively for epidermal growth factor receptor, and patients with ACC might benefit from these agents, especially when surgery has failed or for those patients with recurrent or metastatic disease. Luna-Ortiz et al observed c-kit (CD-117) positivity in all 8 of their reported cases of ACC of the tongue.Radiotherapy is routinely recommended, involving adjuvant external radiation of the surgical bed following surgery. Some studies have recommended the use of postoperative radiation for patients with advanced T stages, positive microscopic margins, or perineural invasion to decrease the incidence of local recurrence. reported that the base of tongue, the mobile tongue, and the mouth floor were the 3 most frequent sites of lymph nodes metastasis, with incidence rates of 19.2%, 17.6%, and 15.3%, respectively, and that level Ib and II regions were the most frequently involved. The overall 5-year survival rate was significantly lower for patients with lymph node metastasis compared with those without lymph node metastasis (48% vs 77%). Selective neck dissection should be considered for patients presenting with floor-of-mouth and tongue lesions with cN0 neck and larger ACCs in these sites with lymph-vascular invasion. A combined method of radical surgery, including wide resection of the original tumor and radical neck dissection, followed by postoperative irradiation, is suitable for primary or recurrent cN+ patients. Factors influencing the choice of therapy are tumor site, tumor stage, histologic grade, and biologic behavior of the ACC.The probability of regional lymph node metastases in ACCs of the head and neck is rare. In a retrospective study of 616 patients with ACCs located in the head and neck regions, Carrasco et alThe ALTF technique for tongue reconstruction has some limitations. First, restoration of the bulk, mobility, and sensibility of the tongue are significant challenges. Preservation of tongue mobility and providing tongue bulk need to be considered. To restore the swallowing function, reconstruction of tongue tissue volume is essential, and providing sufficient tissue to cover the tongue defect and restore the physiological contact tongue-palate is crucial. However, restoring the sensation function with a flap repair is difficult. If a surgeon wants to restore sensation to the tongue, an attempt may be made to connect the lateral femoral cutaneous nerve to the lingual nerve. Furthermore, excess tongue volume could reduce the oral cavity space and limit the mobility of the tongue. Recently, surgeons have reported thinning the ALTF using microscopy, in which case a skin graft is not required and tendons are not damaged. Finally, our experience has shown that when the width of a flap exceeds 9 cm, it may difficult to suture it directly to the donor site, and skin grafting is required. For defects >10 cm in width, the design of a component flap may be considered to avoid the consequences of skin grafting, due to the risk of direct suture failure at the donor site.Conceptualization: Chengyao Zhang, Xiaohong Zhou, Yulian Zhang.Data curation: Chengyao Zhang, Yulian Zhang.Formal analysis: Chengyao Zhang.Funding acquisition: Chengyao Zhang.Investigation: Chengyao Zhang.Methodology: Xi Tang, Rui Chen, Xiaohong Zhou, Yulian Zhang.Project administration: Xi Tang, Xiaohong Zhou.Resources: Xi Tang, Rui Chen, Xiaohong Zhou.Software: Xi Tang.Supervision: Xi Tang.Validation: Xi Tang, Chengyao Zhang, Rui Chen.Visualization: Xi Tang, Chengyao Zhang, Rui Chen, Yulian Zhang.Writing – original draft: Xi Tang, Chengyao Zhang, Yulian Zhang.Writing – review & editing: Xi Tang, Chengyao Zhang.Chengyao Zhang orcid: 0000-0001-7044-6137.


Intra-thymic bronchogenic cysts are a rare entity but should be considered in the differential of all non-invasive thymic masses.We describe a 50-year-old patient who was found to have an incidental thymic mass on computer tomography of the chest. Non-invasive thymoma was suspected and a thoracoscopic thymectomy was performed. Final pathology revealed a bronchogenic cyst.Intra-thymic bronchogenic cysts are extremely rare tumors of the anterior mediastinum. It should be considered in differential diagnosis of anterior mediastinal masses. Benign thymic pathologies are rare and they are not discussed fully in the published literature. One of these pathologies is intrathymic cysts that can be either congenital or acquired. Intrathymic bronchogenic cyst is an extremely rare congenital cystic thymic pathology. Bronchogenic cysts are rare congenital anomalies that develop due to a foregut abnormal budding. It is located either in the mediastinum namely the middle and posterior mediastinum or in the lung parenchyma . AnterioHerein we present a case in which an asymptomatic intrathymic bronchogenic cyst was discovered incidentally on the CT scan of the chest while evaluating a subcentimetric pulmonary nodule.2  was performed. After assessment of the whole chest cavity, complete thymectomy including the above-mentioned tumor as well as the adjacent mediastinal fat was performed. The specimen was retrieved and a 24-Fr chest drain was inserted. A digital palpation through the thoracoscopic incisions could not detect the nodule so resection was not performed.A 50-year-old female patient with a previous history of hypothyroidism and no past surgical history was transferred from Ear, Nose and Throat (ENT) outpatient clinic for further evaluation of a 7 mm solitary pulmonary nodule in the right upper lobe Fig. . This waThe post-operative course was uneventful and the patient was discharged home after chest tube removal on the first postoperative day.The histological examination revealed grossly a completely excised thymus that contained a unilocular cyst filled with white creamy material. Histologically the cyst was lined with ciliated columnar epithelium resembling respiratory epithelium that supported the diagnosis of intrathymic bronchogenic cyst Fig. .Fig. 4PhBenign thymic pathologies such as hyperplasia or thymic cysts are rarely studied. In the published literature there are few studies focused on the clinical and radiological manifestations of benign entities of the thymus. Usually they are grouped as benign thymic disorders .Thymic cysts are relatively rare pathology that can be seen in any age group. They represent 1–3% of all mediastinal masses . They maThe most common mediastinal cysts are the bronchogenic cyst, which usually occurs in the posterior and middle mediastinum. They account for 10 to 15% of all primary mediastinal masses . The sitInfrequently, the cysts have occurred in rare sites, including skin and subcutaneous tissues, neck, pericardium, diaphragm, mesentery of the bowel, and the intramedullary part of the spine. They have also been reported to extend from the mediastinum through the diaphragm into the abdomen as dumbbell cysts .Rarely, bronchogenic cyst can occur within the thymus gland. The first reported case was published by Taniwaki and colleagues in 1997 . After wThe definitive diagnosis of intrathymic bronchogenic cyst is usually made after complete excision of the lesion by illustration of the histological characteristics of the bronchogenic cyst like ciliated columnar epithelium with or without infiltration by chronic inflammatory cells, presence of cartilage, smooth muscle, bronchial glands and nerve tissue .Intra-thymic bronchogenic cysts are extremely rare tumors of the anterior mediastinum. The overlapping radiological characteristics with solid tumors, especially on the CT makes the preoperative radiological diagnosis very difficult. It should be considered in differential diagnosis of anterior mediastinal masses. The final diagnosis would be given after surgical excision and histopathological examination.


Epithelioid hemangioendothelioma is a malignant mesenchymal tumor of unknown etiology. They tend to be asymptomatic or with non-specific symptoms. The lesion is usually multiple and variable size.We describe a clinical case of a 23-years-old patient diagnosed with a pelvic mass, a possible uterine fibroid or adnexal mass, and multiple liver lesions that seemed an advanced ovarian cancer presentation and after liver biopsy turned out to be a hepatic epithelioid hemangioendothelioma.It may be confused with a metastatic process in diagnostic imaging. There have been described some possible risk factors but the etiology remains unknown. The prognosis is usually lethal in 50% of cases. The surgical removal of the lesion and liver transplant appear to be the only hope for these patients.Epithelioid hemangioendothelioma must be part of our differential diagnosis when we find a liver tumour, especially in young women. Treatment is excision of the tumour in limited disease. In the case of unresectable disease are candidates for liver transplantation. Its incidence is less than 1 case per million people per year. The age of presentation is very variable, from 12 to 93 years .2A 23-year-old woman, asymptomatic, presented in a gynaecological examination a pelvic tumour in the left iliac fosse. Physical examination showed a 9 cm mobile mass in the left iliac fosse compatible with fibroid or adnexal mass. She was a non-alcoholic, non-smoker with no co-morbid medical or surgical ailment.Transvaginal ultrasound shows an anteversion uterus with a proliferative endometrium, normal ovaries and a mass of about 9 cm, which does not seem to have a clear relationship with the uterus.Pelvic magnetic resonance imaging describes a 9 cm semi-solid mass compatible with pedunculated fibroid or primary retroperitoneal lesion. Cerebral MRI shows no abnormalities.Abdominal CT scan during the portal phase showed a pelvic mass of 6.5 x 5.5 cm, which seemed to originate from the left ovary, predominantly cystic with solid areas compatible with cystic ovarian neoplasm. Retroperitoneal or pelvic lymph nodes were not identified. There was no ascites. There were multiple bilocular masses informed as metastatic lesions in liver parenchyma. The rest was normal  A and B.CEA, Ca 12.5 and Ca 19.9 tumour markers were normal. Alpha-fetoprotein, testosterone, SDHEA and delta-4-androstenedione were also normal.After discovering these surprising findings, our gynaecologic Tumours Committee decided to perform a guided needle core biopsy of the liver lesions to discart an advanced ovarian cancer. Histologically these lesions corresponded to a vascular tumour compatible with EH.We decided to perform a diagnostic laparoscopy. During the exploration of the abdominal cavity, an enlarged liver with multiple intra-parenchymal hepatic lesions and a tumour with a large blood vessel originating from the greater omentum could be seen. Partial omentectomy resection, including the mass, was performed and was extracted in an endobag  A and B.Macroscopically the lump was fleshy, filled with blood and presented whitish areas. Anatomopathological study confirmed an EH. Immunohistochemical profile was CD31+, CD34+, CKAE1/AE3-, CK8/18-, C-kit-, EMA-, vimentin +, progesterone , estrogen-, mitotic activity <1 × 10 fields of large increased , Fig. 4.After the results, liver resection was discarded due to multicentre lesions, involving pretty much the totality of the liver; making the patient a candidate for liver transplant. Six months later the patient was subjected to a successful liver transplant.Consent was obtained for the publication of the case report and the images from the patient.3EH is a rare tumour that affects middle-aged women in a ratio of 3:2 with respect to men ,6. The sThere have been possible causal agents or risk factors which have been hypothesized, such as vinyl chloride, polyurethane/silica, oral contraceptives, primary biliary cirrhosis, viral hepatitis, asbestos and alcohol use, among others. However, based on the evidence, the etiology remains unknown ,8.In terms of the complementary examinations, more than 84% of the patients present altered liver function, a slightly elevated alpha-fetoprotein and a carcinoembryonic antigen (CEA), which tends to be negative . UltrasoHistopathological diagnosis with routine techniques is difficult, considering differential diagnosis of sclerosing hepatic tumours (hepatocellular carcinoma and cholangiocarcinoma), metastases of carcinomas, particularly those of signet ring cells and angiosarcoma .Histologically characterized for having variable of epithelioid cells and dendritic proportions in the bosom of a stroma with an infiltrate inflammatory joint (85%), and infiltrative nature, which often respects the liver acinar architecture, that is often calcified and sclerosed, and which is particularly prominent in the acinar area . ImmunohThe peculiar natural history of hepatic EH makes difficult the assessment of the effectiveness of the treatments. 50% of patients without treatments survive more than 5 years and the existence of metastasis does not influence the prolongation or shortening of the survival rate. In patients with single or few lesions, surgical excision is the treatment of choice. Patients with extensive, or multiple injuries which are the majority, must be transplanted and the existence of metastasis is not a contraindication . Chemoth4Therefore, the EH must be part of our differential diagnosis when we find a liver tumour, especially in young women. Treatment is excision of the tumour in limited disease. In the case of unresectable disease are candidates for liver transplantation. A multidisciplinary team and a good management of the Tumours Committee is the most important thing to handle this type of complex cases.The authors declare no conflict of interest.d Medtronic University Chair for Training and Surgical Research .This work received financial support from All procedures performed in studies involving human participants were in accordance with the ethical standards of the local ethics and research committee and followed the Declaration of Helsinki guidelines. Written informed consent was required for collecting data.Approval by the Ethics and research committee of our institution (CEIm) was obtained. Ref. 012/2017.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Study concepts: Antoni Llueca, Dolors Piquer.Study design:Antoni Llueca.Data acquisition: Anna Serra, Katty Delgado.Quality control of data and algorithms: Yasmin Maazouzi.Data analysis and interpretation: Yasmin Maazouzi.Manuscript preparation: Antoni Llueca, MC Medina.Manuscript editing: Antoni Llueca, Dolors Piquer.Manuscript review: Anna Serra, Javier Escrig.This is not a first-in-man case report and thus cannot be registered.Dr. Llueca is the guarantor of the paper.Not commissioned, externally peer reviewed.


Thymic abnormalities occur as hyperplasia and thymoma. Myasthenia gravis is commonly present in thymoma. Thymectomy possesses risk due to anatomical proximity with vital thoracic structures and myasthenia crisis.Forty five years female with complaints of difficulty swallowing and weakness of upper limb muscles upon investigation showed mass in mediastinum and antibody test for myasthenia gravis positive. Medical management was done for a month followed by thymectomy. There were no intra and postoperative complications. Medical management was stopped one month after surgery and she is symptom free.Thymectomy is the standard of care where median sternotomy is the mainstay approach to surgery. Various other surgical approaches and complications revolving around surgery has been discussed.Surgical removal of thymoma cured myasthenia gravis in our case. We focused on proper preoperative optimization of myasthenia gravis symptoms before thymectomy. Thymic 2Forty five years female from hilly region of Nepal presented to our Hospital, a community based hospital, with difficulty of swallowing for seven months initially for solid foods which gradually progressed to liquid foods. She had difficulty breathing since two months. There was weakness of upper limb muscles, more pronounced in the evening. There was no decrease in appetite, waterbrash, weight loss or cough. Chest X-ray was suggestive of mediastinal widening . Contras3Thymoma may present with a range of clinical features from asymptomatic detected incidentally upon investigations, presenting with local symptoms to features of myasthenia gravis or other neoplastic processes. One-third of the patient present with space occupying features while one-third to two-thirds have complaints of autoimmune condition, most commonly myasthenia gravis present in 15 to 20 percent of cases . Managem4Surgical removal of thymoma cured the problem of myasthenia gravis in our case. We focused on proper preoperative optimization of myasthenia gravis symptoms before thymectomy. During surgery, care was taken to prevent damage to adjacent vital organs. Vigilance to note myasthenia crisis and proper management in such scenario is vital for successful results.There is no external funding provided to the study.Since this is a case report, ethical approval was not required from Institutional Review Board.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Study Concept: S Dahal, N Bhandari, P Dhakal, RM Karmacharya.Data Collection: RM Karmacharya.Writing: S Dahal, N Bhandari, P Dhakal, RM Karmacharya, AK Singh.Revising and critical review: M Devbhandari, SM Tuladhar.This is a case report so registration was not required.All the authors are the guarantor of the study.Not commissioned or externally peer reviewed.There are no conflict of interests.


Acinonyx jubatus), living in a large enclosure on a private Namibian farm, developed a large exophytic nodular neoplasm in its skin at the height of the left shoulder blade. We describe the clinical appearance, the surgical removal, and histological examination of the tumor, which was diagnosed as a moderately pigmented benign basal cell tumor. A three-year follow-up showed no evidence of recurrence after the surgery. Although neoplasia is reported in nondomestic felids, only very few concern cheetahs. So far, no case of basal cell tumor was described in this species.A 3.5-year-old wild born cheetah ( The number of reports on neoplasms in exotic felids is continuously rising, particularly due to an increase in longevity in captive animals . SeveralNamibia hosts the worldwide largest free-ranging cheetah population with most of these animals roaming on privately owned commercial farmland , 9. WhilWe were contacted by a local farmer who kept a cheetah on his farm, which exhibited a slow growing alopecic dermal mass at the height of the left shoulder blade that began to grow approximately 1.5 years earlier . The aniThe cheetah was immobilized with a mixture of ketamine  and medetomidine  administered with a dart shot from a dartgun . The skin around the mass was shaved and the surgical field was washed and disinfected with 70% ethanol. The mass and covering skin were excised from the adjacent tissue. The neoplasia was well encapsulated and no infiltrative growth into the surrounding tissue was visible, but it had one large supportive blood vessel, which was ligated and cut. The wound was closed with a subcutaneous consecutive suture and several cutaneous suture stiches both with absorbable material. The animal was treated prophylactically with 2 mg/kg ketoprofen  as anti-inflammatory and pain medication and with a combination of 15.000 IU/kg procaine benzyl penicillin and 15.000 IU/kg benzathine benzyl penicillin  to prevent a wound infection. To keep the wound clean from dust and dirt, we dressed the animal with children's shirt, which was removed by the farmer seven days after surgery. The immobilization was reversed with Atipamezole . The wound remained uninfected and was nearly invisible at the annual medical check-up one year after the surgery and the cheetah was still alive 3.5 years after removal of the tumor without any signs of recurrence.The excised neoplasia was fixed in 10% buffered formalin and shipped to Germany in full compliance with the Convention on International Trade in Endangered Species (CITES) for histological examination at the Leibniz Institute for Zoo and Wildlife Research in Berlin.The tumor presented itself as an exophytic firm encapsulated broad based nodular dermal to subcutaneous mass measuring 5.5 cm × 4.5 cm × 2.5 cm, covered by intact sparsely haired dark pigmented skin. Cut surfaces revealed multiple small lobules separated by fine white strands of connective tissue . LobulesA panel of immunohistochemistry stains was applied and revealed tumor cells being positive for pancytokeratin but negative for MelanA, S100, and vimentin. Although the dark pigmentation and nests and islands of polygonal cells prompted the differential diagnosis of a melanocytoma, the immunohistochemistry results lead to the final diagnosis of a moderately pigmented basal cell tumor.Basal cell tumors are common cutaneous epithelial neoplasms in domestic dogs and cats  originatThe most common location of this neoplasia in domestic cats is the head or the trunk , while tPanthera pardus fusca) [P. leo) [P. tigris tigris), which had metastasized to most lymph nodes and the lung [In nondomestic felids, a similar solid basal cell tumor was described in a captive Indian leopard (s fusca)  and a ba[P. leo) . However[P. leo) , and thethe lung .In cheetahs, the most common neoplasia is myelolipoma, which was first described in 1968 . It occu


Fibrous hamartoma of infancy is a solid benign tumor of the subcutis, which usually occurs within the first 2 years of life. It predominantly occurs in males, and is clinically presented as a solid, painless, well-limited subcutaneous formation, tending to grow, and in most cases without any symptoms. It occurs in various locations. The aim of this case report was to present a case of a rare tumor of infancy in the scrotal region, in an 8-month-old male infant, with a nonspecific clinical picture, suggestive of a malignant formation, thus presenting a diagnostic challenge for a doctor. The tumor was completely removed. The diagnosis was confirmed by histopathological analysis. One year after the surgical procedure, a follow-up ultrasonography examination showed no relapse. Fibrous hamartoma of infancy (FHI) is a solid benign tumor of the subcutis, which usually occurs within the first 2 years of life. It was first described by Reye in 1956, as the dermal fibromatous tumor of infancy, and later, within a larger study, Enzinger in 1965 renamed it into fibrous hamartoma of infancy. It accounts for 0.02% of all benign soft tissue tumors.i-67, Bcl-2, CD 34) confirmed the diagnosis of an FHI  scan, magnetic resonance imaging (MRI), and tumor markers were not done. According to the guidelines of our hospital, the follow-up was after 3 months. However, during a follow-up examination 3 months later, progression was verified. A solid formation was palpated, still located in the upper half of the right hemiscrotum, painless, without symptoms, not connected to the testicle, now ∼2 × 2.5 cm in diameter . After  an FHI and6. This case demonstrates a rare tumor of the scrotal region which, due to its nonspecific clinical picture, presents a diagnostic and therapeutic challenge in clinical practice. Considering the nonspecific clinical picture, the consistency of the formation, the area it affects, and its tendency to grow, in terms of differential diagnosis, several conditions can be taken into accountIn most cases, the microscopic finding has three characteristic components: well-differentiated fibrous connective tissue, primitive mesenchymal stroma, and islands of mature fat cells.FHI is a benign and rare formation in the subcutis with a good prognosis. Considering the nonspecific clinical picture, this formation deserves wider diagnostic tests to exclude malignant disease. The method of treatment is complete excision. The final diagnosis is confirmed by histopathological analysis.


Small bowel intussusception in adults is rarely encountered. In most cases small bowel intussusception is caused by benign neoplastic lesions, but metastasis of cutaneous malignant melanoma causing small bowel intussusception is rare. We present such a case of jejuno-jejunal intussusception with an intraluminal metastatic lesion acting as a lead point.We present a case of a 71-year-old Caucasian man who presented with small bowel obstruction. His medical history revealed that he had had a cutaneous malignant melanoma excised 7 years earlier and underwent total laryngectomy due to a metastasis 6 years later. The disease was classified as stage IV and he was receiving immunotherapy. An emergency abdominal computed tomography scan demonstrated small bowel obstruction, most probably caused by an intraluminal lesion. An emergency laparotomy revealed an intraluminal metastatic lesion causing jejuno-jejunal intussusception. Metastasectomy of the lesion was performed and 13 days later he was discharged.Jejuno-jejunal intussusception with a malignant melanoma metastasis acting as a lead point is very rare. With the gastrointestinal tract being a common location of distal metastases, a medical history of malignant melanoma treatment in cases of small bowel obstruction should raise a suspicion of possible metastatic disease. A computed tomography scan is the diagnostic modality of choice and surgery still remains the standard of care. Small bowel intussusception in adults is very uncommon. It accounts for only 1% of cases of bowel obstruction in adults; only 5% of all intussusceptions are found in adults . It is uA 71-year-old Caucasian man presented to our emergency room with vomiting and abdominal pain. He had been experiencing constipation and abdominal discomfort for a few weeks and had heard borborygmi in his intestine. He had noticed an occasional black stool during defecation. Due to persistent normocytic anemia, with hemoglobin levels below 100 g/L, he had had a gastroscopy, which revealed no abnormal conditions. He was also scheduled for a colonoscopy.He had a history of arterial hypertension. Seven years earlier, he had malignant melanoma excised from his abdominal skin. Left-sided axillary lymphadenectomy was performed later due to positive sentinel node. For 6 years his clinical condition was stable. Then, a metastasis on his vocal cord and in his sternum was found, along with a suspicious lesion in his left breast. He underwent total laryngectomy and started receiving immunotherapy with vemurafenib and cobimetinib. Due to adverse side effects, including vomiting, weight loss, and phototoxicity, his therapy was adjusted to reduced dosages, which he was still receiving at the time of our encounter. A head, neck, and chest computed tomography (CT) scan performed for follow-up in another institution 1 month before admission to our department, demonstrated a stable disease.In our emergency room he was stable. His abdomen was distended and diffusely tender on palpation, but without any signs of peritoneal irritation. An absence of bowel sounds was discovered on auscultation. An emergency CT scan was performed, demonstrating a 10 cm long segment of small bowel intussusception , all positive. R0 resection was achieved. Further follow-up visits were scheduled with our patient’s treating oncologist at another institution. At the last visit, his clinical condition was stable and he resumed immunotherapy.Malignant melanoma has a high metastatic potential. The gastrointestinal tract (GI) is the third most common site of distant metastases; among these, the small intestine is most commonly affected . The smaAmong all causes of bowel obstruction in adults, intussusception accounts for only 1% . It is mReported cases of jejuno-jejunal intussusception due to malignant melanoma metastases acting as a lead point are scarce. The clinical presentation of such cases is often unspecific, with abdominal pain, nausea, and vomiting being the most common symptoms. Weight loss, melena, or clinically relevant anemia are rarely seen in adults , 2. HoweAn emergency CT scan was performed at admission of our patient. An abdominal ultrasound demonstrating a donut sign can be helpful in diagnosing an intussusception due to intraluminal malignancy, but CT remains the modality of choice , 11. CT Surgery remains the standard of care of small bowel intussusception with a malignancy acting as a lead point. Usually the operation is urgent and lifesaving, considering that most of the cases are discovered only after symptoms of obstruction become obvious. Elective surgery is considered only in selected cases . WhetherMalignant melanoma with distal metastases is considered a stage IV disease and such patients are subject to systemic therapy. There is no clear consensus on the optimal surgical approach. Although most authors propose resection of the affected small bowel segment, metastasectomy in stage IV disease is also adequate . The priThe average life span of stage IV malignant melanoma is 2 months to 15 years after diagnosis . The timMalignant melanoma metastasizing to the GI tract is not uncommon, yet it rarely causes bowel obstruction. Jejuno-jejunal intussusception with a malignant melanoma metastasis acting as a lead point is seldom encountered and only a few cases have been described in the literature. However, in cases of chronic or acute small bowel obstruction a suspicion of possible metastatic disease should be raised. In such cases, a CT scan is the diagnostic modality of choice and surgery still remains the standard of care.


Malignant melanoma is a nonepithelial neoplasm of melanocytes. It is tremendously rare for this condition to primarily involve the respiratory tract, accounting only for 0.01% of the lung malignancies. It often presents as a solitary nodule provoking mass effect and/or obstructive symptom. It most commonly affects patients 50 years old and older, with no gender predilection. Complete surgical excision is the treatment of choice; nevertheless, chemotherapy or radiation might be necessary depending on tumor location and/or metastasis status. Recently, biochemotherapy and immunotherapy have emerged as promising treatment modalities. We present a case of Primary Pulmonary Malignant Melanoma (PPMM) in a 76-year-old male with no previous personal or family history of cancer who presented with new onset nonproductive cough. We also present an analysis with high yield points summarizing clinical features, diagnostic workup, and management of PPMM. Finally, we post a table summarizing all the cases ever reported in English literature. There are approximately 41,000 melanoma-related deaths in the world yearly and 16,000 new diagnoses every year; nevertheless there are only 41 cases of PPMM reported in the literature since 1916 . Its preA 76-year-old nonsmoker male with history of Obstructive Sleep Apnea presented for elective a Left Knee Total Arthroplasty. Next day, after a successful intervention the patient developed a nonproductive continuous cough. A Chest X-Ray (CXR) was obtained and showed a nodular, irregular opacity in the right lung . ComputeMalignant melanoma of the skin and mucosae is routinely described as a dark, irregular, and asymmetric lesion, which in its most common form leads to a straight forward diagnosis. In the respiratory tract, this malignancy is easily confused with more conventional neoplasms . AlthougDue to its endobronchial location, PPMM frequently manifest with symptoms of cough, hemoptysis, dull chest pain, obstructive symptoms such as pneumonia, lobar collapse, or atelectasis. Symptoms of fatigue, malaise, and nighttime diaphoresis and signs of weight loss or lymphadenopathy might accompany the clinical picture as well. In 20-30% of the cases, it will be incidentally found on imaging . ProGender distribution was fairly equal with 52% of the patients being males and 48% females.The median age at time of diagnosis was 59.1 years (29 to 90).The average tumor size was 4.6 cm (1.0 to 10 cm).67.5% of the patients had metastasis at the time of diagnosis. The most common sites were contralateral lung, liver, brain, and bones as detailed above.Given the broad period in which these cases were published, as well as new advances in diagnostic and therapeutic technology, conclusions regarding treatment modalities or the variables that impact survival and mortality are precluded. There are only a few facts that can be stated upon review of the cases above:With this case report and literature review we aim to add critical data to the current medical literature regarding this highly unusual and rare disease. Reliable data regarding clinical presentation, diagnosis, and potential treatments of PPMM is becoming more available to health care workers making this a promising era in the identification and management of this condition, specifically with the establishment of unequivocal diagnostic criteria and the advent of new treatment modalities. Additionally, our findings of disseminated disease and prompt surgical intervention being the strongest factors influencing survival are perfectly exemplified by our case report. At this time randomized studies are univariably defiant as with any rare disorder.


Extrauterine leiomyomas are very rare and present a clinical and diagnostic challenge due to their unusual growth patterns and behaviours. A 47-year-old woman was transferred to our tertiary specialist obstetrics and gynaecology hospital with acute abdominal pain and a palpable abdominal mass. She was taken immediately to theatre with the presumptive diagnosis of an ovarian torsion. Intraoperatively, a large necrotic mass originating from the mesentery and attachments to the bowel at the ileocaecal junction was noted. When converted to laparotomy due to limited access and poor visualisation, the uterus, ovaries, and tubes were found to be normal. A right partial hemicolectomy was performed with the assistance of the colorectal surgeon due to suspicion of bowel malignancy. Histology revealed a benign infarcted leiomyoma with adhesions to the adjacent ileum. The diagnosis of a primary torted mesenteric fibroid was made. Leiomyomas (fibroids) are histologically distorted smooth muscle tumours, which are thought to arise from the genitourinary tract and are almost always benign . They arLittle is known about these rare extrauterine growths. In fact, it is often only after histological assessment of the resected specimen that a diagnosis of a leiomyoma is made. It has been postulated that these atypical leiomyomas develop secondary to cell spillage and seeding during morcellation at hysterectomy or myomectomy , 10. RarWe present a case of a 46-year-old woman with a torted mesenteric fibroid; her puzzling presentation, intraoperative findings, and suggested theories behind the possible development are also discussed.A 47-year-old para 2 presented to the general hospital emergency department with a 3-day history of severe abdominal pain, nausea, and vomiting. This was her first hospital presentation with abdominal pain. Her past medical history included two previous caesarean sections but no other abdominal surgeries. A month prior to this presentation, she had visited her primary healthcare provider complaining of a distended abdomen. An ultrasound performed at the time had reported a large pedunculated uterine fibroid measuring 126x104x108mm. The left ovary was normal but the right ovary was not visualised. Unfortunately, this information was not readily available to the treating team at the time of her acute presentation.A FAST scan (focused assessment with sonography for trauma) done in the general hospital had reported a moderate amount of free fluid in the right iliac fossa and a large pelvic mass. The presumed diagnosis was an ovarian cyst torsion and formed the premise for transfer to our tertiary specialist obstetrics and gynaecology hospital.On arrival to our specialist hospital, the patient was haemodynamically stable and afebrile. On palpation of her abdomen, a large, firm, mobile, and tender mass was noted, extending to her umbilicus. She had a negative pregnancy test. Besides a raised CRP of 77ml/L, the rest of her blood profile was normal. en bloc. Postoperatively, she had a five-day hospital stay complicated by an ileus requiring nasogastric tube placement. She made a full recovery and was discharged home with outpatient clinic follow-up.Given the acute nature of her presentation and the ultrasound findings, she was transferred to theatre for laparoscopic management of a torted ovarian cyst. At laparoscopy, performed through Palmers point entry due to the large mass extending to the umbilicus, a large 25cm multilobulated necrotic mass occupying the entire lower abdomen and pelvis was found. The mass was mobile with attachments to the bowel at the right iliac fossa. Due to limited laparoscopic accessibility, it was impossible to fully explore the rest of the pelvic organs safely. Laparoscopy was converted to a laparotomy and a transverse lower abdominal incision was made for a safer inspection. A large necrotic mass originating from the mesentery of the ileocaecal junction was noted . Its borOn macroscopic histopathology, a large mass attached to the serosal surface of the ileocaecal junction with a 10mm vascular stalk was reported. Microscopically, a circumscribed mass composed of interlacing bundles of smooth muscle with large areas of necrosis and infarction was noted. Adhesions made up of fibrofatty tissue, between the mass and the caecum, were present. The smooth muscle cells stained positive for desmin and actin and showed a low Ki-67 staining pattern. Features of a benign leiomyoma with extensive infarction were confirmed. Adhesions between the leiomyoma and the adjacent ileum and caecum were extensively present, supporting the diagnosis of a torted extrauterine mesenteric fibroid.Extrauterine fibroids are extremely rare and poorly understood. They develop outside the uterus and arise as proliferation of smooth muscle cells with behaviours and histological characteristics identical to their uterine leiomyoma counterparts . Their dUnusual extrauterine fibroid growth patterns described in the literature to date include peritoneal leiomyomatosis, intravenous leiomyomas, benign metastasizing leiomyomas, retroperitoneal leiomyomas, and parasitic leiomyomas. Although various theories and hypothesis exist regarding the mechanism behind these manifestations, no established conclusive pathophysiology has been made. It is important however to consider the heterogeneous theories that do exist regarding the bizarre manifestations of these extrauterine leiomyomas, before considering the diagnosis of our case.Disseminated peritoneal leiomyomatosis, first described by Taubert et al. in 1965, is a rare condition characterized by innumerable diffuse vascular peritoneal nodules that grow along submesothelial tissues in the abdominopelvic peritoneum. It is thought that these multiple growths arise from smooth muscle metaplasia of submesothelial mesenchymal cells, the embryological origins of the peritoneum that line the female reproductive tract . These aAnother rare extrauterine pathology exists in the form of Intravenous leiomyomas. These manifest as intramural proliferation of leiomyomas that occur within intrauterine and systemic veins . They arBenign metastasizing leiomyomas, as first described in 1939, manifest as nodules of well-differentiated benign leiomyomatous tissue located at distal sites to the uterus, mimicking metastatic disease . Most coAs first described in the literature by Kelly et al. in 1909, parasitic leiomyomas are a rare type of migratory leiomyoma. It was first hypothesised that pedunculated subserosal leiomyomas could adhere to surrounding structures, detach from the uterus, and develop axillary blood supplies, thus explaining the diagnoses of leiomyomas at sites distal to a myomatous uterus . AlthougOur case presented a diagnostic dilemma and left us puzzled as to the possible pathophysiological explanation. The most probable mechanism for her diagnosis would be that of a parasitic myoma. This is reinforced by the fact that she had had two previous caesarean sections and an ultrasound scan a month prior to her presentation reporting the presence of a subserosal pedunculated fibroid. However, caesarean sections have not yet been postulated as a potential source for seeding. Also, at the time of surgery, the uterus appeared normal with no area of detachment noted. Given the degree of dense adhesion to bowel and mesentery, and the short length of time that had elapsed between her scan and presentation, it is possible that the migration theory could not be the sole explanation. This case could therefore represent a solitary primary mesenteric fibroid.In the literature to date, there is little evidence of primary mesenteric fibroids in an unmorcellated abdomen. In a case series published by Dan et al. in 2012 of two patients who required right hemicolectomies for extrauterine fibroids, one patient had no prior history of abdominal surgery, whereas the other had a history of a laparoscopic myomectomy just eleven days prior . WhetherWhat is even rarer is the presence of a torted extrauterine leiomyoma. In our case, the fibroid had rotated along a stalk morbidly adherent to underlying mesentery and bowel. There are few reported cases in the literature describing torted parasitic fibroids in the absence of having had previous abdominal surgeries –28. SimiIt is important to consider the possibility of an extrauterine fibroid as a differential diagnosis in any female presenting with an intra-abdominal and pelvic mass, especially with a history of previous surgery for fibroids. It is also important to recognise the need for prompt exploratory surgery in any premenopausal patient presenting with acute abdominal pain and the clinical suspicion of torsion.Our case highlights the complexity of the diagnosis of extrauterine fibroids and adds to the body of literature describing and reporting extrauterine fibroids. As they remain rare and grossly misunderstood, it is prudent to continue reporting cases in order to create a database which could be used to formulate a better understanding of the clinical presentation, diagnosis, and management.


Spigelian hernia is an uncommon presentation of abdominal hernias with 0.1-2%. We report a case of a large strangulated Spigelian hernia, an uncommon presentation of abdominal hernias, and its management in a health facility in Central Uganda.  A 76-year-old female presented with a 2-day history of colicky abdominal pain, bilious vomiting, and abdominal distension. On abdominal ultrasound scan, an abdominal wall defect measuring 4.45 cm with herniated bowel loops in the left anterior abdominal region with mild fluid collection in the hernia sac was seen. Conservative management for intestinal obstruction which included putting the patient on nil per os, NG tube decompression, and soapy enema was instituted, and surgery was done on the second day of admission. Intraoperatively, using a Rutherford-Morrison incision, we found a large defect at the Spigelian aponeurosis, with an inflamed sac protruding. The Spigelian hernia was repaired with a mesh under layers. The patient recovered uneventfully and was discharged 10 days after surgery.  Clinicians and especially general surgeons might be aware of this rare condition in most of the anterior abdominal swellings. Strangulation is the commonest complication of Spigelian hernia, and surgical management remains the mainstay of its treatment. In all abdominal wall hernias, Spigelian hernia (SH) represents a rare surgical condition with 0.1-2%, frequent in patients aged between 40 and 70 years with a predominance in the female gender than male with a sex ratio of 1.18 : 1 .The Spigelian hernia is clinically asymptomatic in 90% cases and has nonspecific clinical findings. However, vague abdominal pain can be associated with it. Severe complications marked its evolution such as strangulation in up to 24%, and patients should be offered an immediate surgical management .In this paper, we need to report a case of a large strangulated Spigelian hernia, an uncommon presentation of abdominal hernias, and its management in a health facility in Central Uganda.A 76-year-old female, known to be hypertensive on treatment, presented with a 2-day history of colicky abdominal pain, bilious vomiting, and abdominal distension. On examination, she was sick looking and afebrile.On physical examination, she had a moderately distended abdomen, with a moderately tender swelling in the left anterior abdominal region which according to the patient had been there for over 20 years. The rest of the abdomen was mildly tender but no rebound tenderness, and bowel sounds were of increased frequency and pitch. A digital rectal exam revealed an empty rectum. Notably from the cardiovascular system exam, she was in hypertensive urgency with a blood pressure of 200/100 mmHg.Investigation results show the following data: full blood count: total white cell count 13.9 g/L, hemoglobin 11.9 g/dL, and platelets 563 g/L; serum electrolytes: chloride 99.9 mmol/L, sodium 134.9 mmol/L, and potassium 5.40 mmol/L generally normal; and abdominal ultrasound scan: bowel loops were distended  with conCT scan was requested but not done due to financial constraints. Working impression was intestinal obstruction due to sigmoid volvulus, also querying an obstructed hernia in the left anterior abdominal wall and hypertensive urgency.Conservative management for intestinal obstruction which included nil per os, nasogastric tube decompression of the stomach, soapy enema, maintenance intravenous fluids, and antibiotic cover was started plus blood pressure management, and 24 hours later, she had improved as the abdominal pain, distension, and vomiting had stopped. She was able to pass stool normally. The blood pressure had also normalized. Decision was made to repair the hernia; and an explanation was given to the patient, and informed consent was obtained.Intraoperatively, using a Rutherford-Morrison incision in the left anterior abdominal region, the abdomen was opened in layers; a large defect was found (6 × 5 cm) at the Spigelian aponeurosis , with a Spigelian hernia is an uncommon abdominal hernia with 0.1 to 2% of all abdominal defects . Even ifIn 24% of the cases, the strangulation is a major complication of Spigelian hernia as seen in our case report . The conIn Spigelian hernia, mesh repair associated with laparoscopy correction is the best procedure in developed countries , but in A Spigelian hernia is really an uncommon presentation of abdominal hernias, but it exists. Its presentation is quite asymptomatic, and its diagnosis is shown by the signs of its complications. Clinicians and especially general surgeons might be aware of this rare condition in most of the anterior abdominal swellings. Findings from supportive investigations play a major role in its diagnosis. Strangulation is the commonest complication of Spigelian hernia, and surgical management remains the mainstay of its treatment.


The purpose of this paper is to serve as a reference to aid in the management of this poorly understood intracranial malignancy. The authors report their experience treating the eighth ostensible case of a primary intracranial alveolar soft part sarcoma (ASPS). A 21-year-old man presented to hospital after collapsing. He gave a 1-year history of headache, a 2-month history of reduced visual acuity and on examination had left facial paraesthesia with left-sided incoordination. MRI of the brain revealed a large left posterior fossa mass. The patient underwent resection of the tumour with good recovery in function. Immunohistochemical analysis of the tumour specimen confirmed an ASPS, and multimodal imaging in search of an extra-cranial disease primary was negative. A review of the literature yielded only seven other cases of primary intracranial ASPS. A variety of diagnostic imaging modalities were employed in search of a disease primary, as were various combinations of surgical resection, chemotherapy and radiotherapy as treatment. Half of the cases documented delayed disease recurrence. The authors discuss the following: the unique radiological and immunohistological characteristics of this disease including the potential for its misdiagnosis; the investigations required to diagnose a primary intracranial ASPS; the efficacy of current medical and surgical treatment options and the factors that will aid in prognostication. This is the first review of this new primary intracranial malignancy. From our analysis, we offer a joint radiological and immunohistochemical algorithm for the diagnosis of primary intracranial ASPS and specific operative considerations prior to resection. Alveolar soft part sarcoma (ASPS) is an extremely rare malignancy of unknown cellular origin accounting for 1% of all soft tissue sarcomas. ASPS effects a younger patient population with a peak incidence between 15 and 35 years . The priHere, we present the eighth ostensible case of primary intracranial ASPS and the second case of ASPS arising in the posterior fossa . In conjA 21-year-old man presented to his local hospital after collapsing at home due to leg weakness. He gave a 1-year history of persistent headache accompanied by a 2-month history of blurred vision, reduced sensation on the left side of his face and occasional difficulty in walking. He had a history of learning difficulties and anxiety with no other medical problems. On admission, he was fully conscious with a Glasgow Coma Score of 15/15. Cranial nerve examination revealed a left 4th and 6th nerve palsy causing diplopia and reduced sensation in all distributions of the left trigeminal nerve. There was left-sided nystagmus in conjunction with left cerebellar signs causing a broad-based gait. Fundoscopy revealed papilloedema. Peripheral neurological examination was unremarkable.MRI brain with contrast revealed a broad-based and extra-axial mass measuring 4 × 4 × 5 cm in the left posterior fossa. It was isointense to grey matter on T1-weighted imaging (WI) and heterogeneous on T2WI imaging with avid heterogeneous T1WI enhancement post-gadolinium administration. Diffusion-weighted imaging showed no restriction with some small cystic components. Evidence of mass effect was noted on the left cerebellar lobe and the midbrain with evidence of hydrocephalus. Multiple small vessels were intimately associated with the lesion. An initial radiological diagnosis of meningioma was made  staining was performed and showed strong nuclear positivity confirming an alveolar soft part sarcoma.Immediately post-operation, the patient had worsening of his cranial nerve dysfunction with a new left facial weakness. During the next few weeks, his cranial nerve palsies gradually improved, and he was eventually able to ambulate independently. At this point, he was referred to the oncology team for further investigation.Extensive imaging in search of a primary lesion site was all negative, including repeat whole-body FDG-PET scans over a 2-month period. At 10-month follow-up, he is living at home requiring no formal package of care. He is currently receiving regular follow-up as part of surveillance by his oncology team. A repeat MRI brain at this time showed no evidence of disease recurrence, and CT of the chest, abdomen and pelvis showed no disease primary  fusion gene can be employed. Recently, an antibody directed against the C-terminus of the TFE3 fusion gene has proven a highly sensitive and specific marker for ASPS. Despite this, caution must be exercised as the TFE3 gene is also present, but in a different configuration, in paediatric RCC and also in granular cell tumours . TherefoThe cellular origin of ASPS remains an issue of debate and therefore makes investigation for the origin of primary disease difficult. Originally, it was thought that ASPS was a variant of granular cell tumour, but more recent immunohistochemical studies point towards a muscular origin . Given tChemotherapy, radiotherapy and surgical resection have been employed individually and in combination in the management of primary ASPS. Given the rarity of the disease, reports into the outcomes of these treatment modalities are limited to large case series and do not address ASPS effecting one specific anatomical region.Surgery may be considered the first-line treatment in localized ASPS and may potentially increase long-term survival . PrognosA favorable outcome from complete microscopic resection of localized ASPS has been reported from several series. A study by the Italian Soft-Tissue Sarcoma Cooperative Group found that 100% of patients who underwent total surgical resection of localized ASPS showed no evidence of disease at follow-up ranging from 11 to 243 months, often with surgery being the only treatment modality employed . SimilarIn our review of primary intracranial ASPS, total surgical resection was achieved in six of eight cases. Total surgical resection was deemed unsafe in two cases due to difficult to control large-volume haemorrhage. Three out of the seven cases were detailed experienced localized disease recurrence, with two of these patients having undergone total surgical resection and the other subtotal resection. In two instances, patients received adjuvant chemo- and radiotherapy and in the other radiotherapy alone. Of the four patients who remain disease-free, two underwent total surgical resection alone, one underwent total surgical resection plus adjuvant chemotherapy and one total surgical resection plus radiotherapy.It is of note that in the three instances of disease recurrence, the follow-up time is significantly longer than in those who remain disease-free. Also, contrary to the association between younger age at presentation and favorable prognosis reported from extra-cranial primary ASPS, the only three patients under 17 years of age in this review were the ones to demonstrate disease recurrence.ASPS appears to be a chemotherapy-insensitive pathology. Eleven patients with both localized and metastatic ASPS who received chemotherapy failed to show any response to treatment. Chemotherapeutic agents used were varied including antimetabolites, alkylating agents, mitotic inhibitors, anthracyclines, antimicrobial agents, antineoplastics, antifolates, COX-2 inhibitors, interleukin-2, interferon-alpha and TNP-470 . The larThe role of radiotherapy in the treatment of ASPS remains unknown. The few case series documenting the response to radiotherapy are unable to demonstrate any clinical benefit due to small study numbers , 20. RadAs the long-term prognosis for localized ASPS is favorable, patients will require extended periods of follow-up with appropriate cranial imaging. Cases 3 and 4 highlight the need for extended follow-up as disease recurrence can occur in a delayed fashion. Also, extended follow-up is recommended to ensure that no initially occult primary disease focus renders itself detectable. Furthermore, even in patients with widespread metastatic disease, there exists an unexplained subset of long-term survivors beyond 5 years from initial treatment. These patients highlight both the potential indolent nature of even metastatic ASPS and our incomplete understanding of its natural history. In time, molecular analysis of these patient subsets may provide an explanation for this variation in survival.Based upon the clinical features of the reported cases of primary intracranial ASPS in conjunction with literature to date, there are several implications for consideration in the management of this disease. First, our case gives further evidence that ASPS may arise as a primary intracranial malignancy within the posterior fossa. Second, to confidently demonstrate a primary intracranial ASPS, whole-body FDG-PET scanning must be performed to exclude a soft tissue primary as well as CT imaging of the thorax to rule out pulmonary involvement. If unable to test for the TFE3 fusion gene, then imaging of the kidneys should also be performed to exclude a renal mass. Third, primary intracranial ASPS should be borne in the mind of the neurosurgeon when confronted with what radiologically appears to be an atypical meningioma, haemangiopericytoma or medullablastoma in the younger patient. Fourth, total surgical resection of primary disease is the main treatment option as neither chemotherapy nor radiotherapy has proven benefit in curative management in extra-cranial disease. Furthermore, where the benefit of a diagnostic biopsy is available prior to resection, pre-operative preparation for large-volume haemorrhage is advised. Fifth, contrary to the trend in extra-cranial primary ASPS, younger patients with primary intracranial ASPS may have a worse prognosis.At this moment, we know neither the natural history nor the cellular origin of this primary intracranial malignancy. Our case makes part of this review which is the most comprehensive to date of this disease. Still, we cannot strongly conclude what may be the best treatment options for primary intracranial ASPS from these few cases, despite significant literature documenting experience with extra-cranial disease. We therefore encourage other clinicians to report their encounters with this new primary intracranial malignancy in the endeavor to create a greater understanding of this disease and its best management.


Pulmonary carcinoids are included in the group of neuroendocrine tumors (NET) and derive from pulmonary neuroendocrine cells. The incidence of these tumors is increasing, but disease awareness remains low among clinicians. The synchronous presentation of lung cancer and mycobacterial infection is well known but the combination of pulmonary carcinoid and mycobacterial infection is rare.We treated a 45-year-old female who presented with recurrent pneumonia. Chest X-ray showed a consolidation in the left upper lobe. The patient was treated with various courses of antibiotics without full recovery after six months. Computed tomography (CT) scan demonstrated a central mass in the left upper lobe. Bronchoscopy revealed an endobronchial, well-defined lesion that totally obstructed the left upper lobe bronchus. Bronchial biopsy showed typical carcinoid tumor. Rigid bronchoscopy with electrocautery was attempted, but we were unable to radically remove the tumor. Therefore lobectomy was performed. The surgical pathology specimen showed atypical bronchial carcinoid and consolidations in the lung parenchyma with granulomatous inflammation distally of the bronchial obstruction. Ziehl-Neelsen staining demonstrated acid fast bacilli indicative of mycobacterial infection.This case history illustrates the importance of careful surgical pathologic examination, not only of the resected tumor, but also of the postobstructive lung parenchyma. Specific postobstructive infections such as tuberculosis or nontuberculous mycobacteria (NTM) can have clinical implications. Pulmonary carcinoids are NET arising from Kultchitzsky cells and 25% occur in the respiratory tract . PulmonaCarcinoid tumors are divided into low-grade  and intermediate-grade , based on mitotic activity and presence of necrosis. The location of pulmonary carcinoids can be central or peripheral, although the majority is located centrally , 3. RespThe cause of pulmonary carcinoid is unknown and there is no strong association with smoking or environmental carcinogens , 2.A 45-year-old female, never smoker and without comorbidity, presented with cough, low grade fever and mild weight loss. There was no haemoptysis. She had no contact with tuberculosis patients but she had travelled to endemic countries in Asia and Africa. There was no history of recurrent infections in the past. She was diagnosed with pneumonia and treated with various courses of antibiotics but without resolution of symptoms. Physical examination revealed decreased breath sounds in the left upper lobe. Chest X-ray revealed a consolidation in the upper left hilum and left upper lobe Fig. . CT scanPulmonary carcinoids are classified according to the WHO 2015 classification as typical or atypical . TypicalPatients with typical carcinoid have a favorable prognosis with a 10-year survival of up to 80–90%, compared to 40–60% in patients with atypical carcinoids .The most frequent symptoms in patients with bronchial carcinoids are cough, dyspnea and hemoptysis. Lobar obstruction often occurs in bronchial carcinoids and causes postobstructive pneumonia , 7. The The coexistence of lung cancer with tuberculosis and NTM is well known but the coexistence of bronchial carcinoid with tuberculosis or NTM is rare and has been reported previously in a few cases in the English literature.Agave et al. reported about 9 cases of pulmonary carcinoids with MTB  and DixiTuberculosis has been described in association with various forms of lung malignancies. However, its association with carcinoids has been rarely reported possibly because the pulmonary carcinoid tumors are rare tumors .Unfortunately, the mycobacterial culture remained negative. Several explanations are possible. First, there could be a sampling error due to uneven distribution of mycobacteria in tissue samples and/or the presence of extensive necrosis in the tissues . Second,There are some next generation sequencing techniques emerging for the diagnosis of microorganisms in the tissue. However, a major concern is the abundance of the microorganism DNA in the tissue. Advantages of such techniques are mainly the possibility of testing multiple microorganisms in one run (multiplex testing). In our case, the low amount of the mycobacterial DNA in the necrosis likely led to false negative results using rtPCR, a lot more sensitive technique.This case illustrates the importance of systematic examination of the resection specimen with careful attention of the lung parenchyma distal to the bronchial carcinoid. Findings such as mycobacterial infection may require additional treatment and should not be missed.Bronchial carcinoid is often associated with postobstructive bacterial pneumonia, but can sometimes also coexist with tuberculosis or NTM infection. It is therefore important not only to investigate the resected tumor, but also the obstructed lung parenchyma in the surgical resection specimen.


Background: Solid pseudopapillary tumors (SPTs) of the pancreas are uncommon neoplasms, first reported in 1934, well described by Frantz in 1959, and later further characterized by Hamoudi in 1970. Ninety percent of these tumors occur in young females in their second to third decade of life. An interesting case of a persistent solid pseudopapillary neoplasm is described in this report.Case presentation: A 24-year-old woman from a Middle Eastern country presented with an 8.2 × 7.6 cm heterogeneous-enhancing lesion growing within the uncinate process of the pancreas. She had first experienced symptoms at the age of 12 years. Imaging studies showed that the mass closely abutted the superior mesenteric vein as well as the superior mesenteric artery (SMA). The patient underwent an open cholecystectomy and a classic pancreaticoduodenectomy. During the resection, the SMA was transected due to tumor adherence. The vessel was subsequently reapproximated in an end-to-end manner. On the first postoperative day, thrombosis of the SMA occurred and a bile leak developed. The patient returned to the operating room for SMA embolectomy and for repair of a hepaticojejunostomy leak, with redo of the biliary-enteric anastomosis. Histopathological examination showed solid pseudopapillary-arranged cells and cystic areas, showing strong cellular immunoreactivity for CD56, CD10, vimentin, and β-catenin, and weak diffuse staining for synaptophysin. The tumor stained negative for chromogranin A, trypsin, AE1/AE3, and E-cadherin. Molecular genetic analysis was negative for the MYB gene deletion. At nearly 1 year of follow-up, the patient is well with no evidence of tumor recurrence.Conclusion: SPTs of the pancreas should be included in the differential diagnosis of pancreatic tumors, especially in young women. Ninety percent of SPTs occur in young women in their second to third decade of life.6 One hypothesis posits that the tumor is related to sex hormones, although this theory has not been confirmed. Surgical resection is the mainstay of therapy and an adequate surgical resection generally results in long-term tumor-free survival. SPTs can be quite different from other pancreatic tumors in radiological and pathological findings. The cellular origin of SPTs and tumorigenesis, while now thought to be embryonic in origin, remains an enigma.Solid pseudopapillary tumors (SPTs) of the pancreas are a rare neoplasm accounting for 0.13–2.76% of all pancreatic tumors.A 24-year-old woman from a Middle Eastern country presented to the Jefferson Pancreas, Biliary and Related Cancer Center for evaluation of a recurrent pancreatic mass. She complained of right upper quadrant fullness, and physical examination revealed a remote right subcostal incision. At the age of 12 years, she had first developed decreased appetite, weight loss, fatigue, pruritus, and subsequently became jaundiced. Medical records from that episode revealed that an endoscopic biliary stent was placed with surgical exploration through a right subcostal incision and partial resection/enucleation of a pancreatic mass. In the intervening 12 years, the mass had persisted and enlarged, although the patient was asymptomatic, having neither anorexia, pruritus, nor jaundice.Routine hematology and basic chemistry panels were normal. The tumor marker cancer antigen 19-9 was mildly elevated at 89 U/mL (<35 U/mL). An abdominal computed tomography (CT) scan with contrast revealed an 8.2 × 7.6 cm heterogeneous-enhancing lesion, prominently involving the uncinate process of the pancreas . The panThe patient underwent an open cholecystectomy and a difficult classic pancreaticoduodenectomy. The operative time was 12 h and the estimated intraoperative blood loss was 1500 mL. There was no evidence of metastasis, but the tumor had adhered extensively to the SMV and portal vein and surrounded the SMA. We were able to accomplish the separation of the tumor from the venous structures without incident; however, separating the tumor from the SMA proved challenging. At one point, the SMA was transected due to adherence of the tumor. The SMA was subsequently reapproximated in an end-to-end manner with good arterial Doppler signals distal to the anastomosis.MYB gene deletion.Pathological analysis of the surgical specimen revealed the tumor to be a solid pseudopapillary neoplasm . All surOn the first postoperative day, the patient had a small amount of bile visible in the operatively placed drains, she was fluid seeking, and her abdomen was somewhat distended. Due to the suspicion of a vascular insult related to the SMA reconstruction, an abdominal CT with intravenous contrast was obtained and revealed an intraluminal thrombus in the proximal SMA, ∼1.5 cm from its origin off the aorta, causing near complete occlusion of the SMA . She wasOn the fourth postoperative day, an upper gastrointestinal series with water-soluble contrast instilled into the stomach through a nasogastric tube revealed no contrast extravasation, and both the afferent and efferent limbs of the duodenojejunostomy were grossly patent.The patient and her family were instructed on the home management of the large abdominal incision and superficial wound infection. Healing occurred over the next 4 months. Telehealth monitoring was used by our nursing experts to communicate with the patient on a regular basis, with mobile phone images documenting the status of the wound.She returned to Philadelphia for a follow-up visit after 6 months. At that time, the patient appeared well, her wound was completely healed, and an abdominal CT scan with contrast showed normal after pancreaticoduodenectomy anatomy, without any evidence of recurrent or persistent tumor.7 as in the case herein reported. SPTs have a low-grade malignant potential and a good prognosis.8 Interestingly, SPTs can mimic malignant pancreatic tumors with hypermetabolism of FDG on positron emission tomography (PET) scanning.9 Typically, the radiographical appearance of SPTs is a well-defined encapsulated tumor that contains a mixture of solid and cystic components. In fact, most patients are asymptomatic with no abnormalities or physical signs, and the lesion is generally detected incidentally on imaging or physical examination. The size of the mass and its location (compressing adjacent organs) may lead to vague abdominal discomfort. Obstruction of the bile duct or intestine, jaundice, pancreatitis, or spontaneous tumor rupture are rare occurrences. Nakamura et al.10 described a rare case of SPT, which resulted in occlusion of the splenic vein, leading to the development of gastric varices and left-sided extrahepatic portal hypertension.SPTs are most often located at the head of the pancreas,11 Interestingly, Nakahara et al. described an unusual case of SPT in a young woman refusing surgery.12 During a 10-year follow-up period, the maximum diameter of the tumor gradually decreased from 4.5 to 1.5 cm, and the authors posited that some degenerative changes such as hemorrhage and necrosis, followed by absorption, resulted in the natural shrinkage of the tumor. Goh et al.13 suggest that the malignant potential of SPTs is difficult to predict by gender, age, or tumor size without surgery or biopsy, but that the diagnosis of SPTs should be suspected when a young female has a mass of pancreatic origin. In addition, if one finds a large, centrally calcified, well-encapsulated, mixed cystic/solid mass by CT/MRI, the diagnosis of an SPT should be suspected.Surgical resection of SPTs is the mainstay of therapy, and a complete surgical extirpation most often results in long-term survival. Almost 95% of SPTs can be cured by complete excision with margin-negative resection.14 In general, the neoplastic cells in SPTs are negative for chromogranin A. Occasionally, as in our case, immunoreactivity for synaptophysin is seen. In our case, the immunohistochemical findings were positive for CD56, CD10, vimentin, and β-catenin, and weakly positive for synaptophysin. However, the tumor was negative for chromogranin A and trypsin and also negative for AE1/AE3 and E-cadherin. In addition, the molecular genetics showed the tumor to be negative for the MYB gene deletion . Chromogranin A and synaptophysin are both general markers for neuroendocrine tumors and are typically reported together. In our case, chromogranin A was negative, and there was only weak diffuse staining for synaptophysin, indicating that the tumor contained scattered neuroendocrine cells. CD56 is also a marker for neuroendocrine lesions, but synaptophysin is more specific and less sensitive to neuroendocrine differentiation. Burford et al.15 suggest that fine-needle aspiration samples tested for expression of CD10 and the characteristic expression of E-cadherin/β-catenin are highly specific for distinguishing SPTs from pancreatic endocrine neoplasms. Cytokeratins (AE1/AE3) are intermediate filament (IF) proteins used for the cytoskeleton. AE1/AE3 are expressed and regulated in most epithelial cells. These markers are expressed in many tumors and may be helpful in identifying the origin of a neoplasm. In certain situations, IF vimentin is a useful marker to assess the primary site of a tumor and it has a relationship with dedifferentiation and carcinogenesis in the epithelial cells, suggesting that it may be a useful marker for pancreatic SPT precursor cells.On hematoxylin–eosin staining, SPTs are histologically characterized by solid pseudopapillary-arranged cells and cystic areas. SPTs may often mimic endocrine tumors of the pancreas, with morphological features including nest formation and tumor cells with eccentric nuclei. The characteristic immunohistochemical features of SPTs are CD10, CD56, and vimentin positivity. Importantly, beta-catenin has nuclear expression in all reported cases of SPTs.In conclusion, SPTs have characteristic pathological and radiological features and should be included in the differential diagnosis of pancreatic tumors, especially in young women.


Volvulus of the mobile ascending colon is a serious complication of a subtle embryological abnormality that frequently goes unrecognised. There has been renewed interest in the development of the mesentery because of its relevance to the contemporary management of surgical diseases. This case is presented to illustrate the dire consequences of missing this diagnosis and to review the fascinating embryology of the condition as well as its clinical implications.We report on a 23-year-old male who presented with signs and symptoms of distal small bowel obstruction after a long prior history of intermittent right lower quadrant pain. At laparotomy, a 360-degree counter-clockwise volvulus of the entire right colon was noted as the result of an excessively long ascending mesocolon and unattached hepatic flexure. All gangrenous bowel was resected with primary ileocolic anastomosis performed thereafter. The patient did well post-operatively and was subsequently discharged.Interruption of the in-utero events of fixation known as peritoneal zygosis lead to a persistence of the mesocolon in parts of the bowel that ordinarily are retroperitoneal. The events that lead to this anomaly are poorly understood and have been subject to controversy for centuries. New insights have challenged surgical dogma and informed new surgical techniques. Its true incidence is probably underestimated because of its indolent clinical prodrome, and it requires a high index of suspicion.Failure of peritoneal zygosis is implicated in a myriad of clinical conditions. Expeditious recognition and intervention in the prodromal period can avert potentially disastrous complications. Volvulu2A 23-year-old male patient was referred to our public teaching hospital with two days history of colicky periumbilical abdominal pain, feculent vomiting and obstipation for one day. He had not had prior surgery, was HIV negative and had been generally well prior to this illness. He did however, admit to recurrent right lower quadrant abdominal pain throughout his life which had never been previously investigated. He had no relevant drug, family, psychosocial or genetic history.Upon examination, he appeared dehydrated, lethargic and in mild respiratory distress. He had a temperature of 36.9 °C, a respiratory rate of 40 breaths per minute and a saturation of 82%. He had moderate abdominal distension, visible peristaltic movements as well as peritonitis with rigidity and rebound tenderness. Digital rectal examination and assessment of other systems were unremarkable.3 (4–11/mm3), haemoglobin of 19.9 g/dl (14–17 g/dl), reflecting dehydration. He also had a high urea: 97 mMol/L (2–6.7 mMol/L) and serum sodium was 131 mMol/L (133–146 mMol/L).Blood investigations revealed leucocytosis of 13.11/mmAn acute abdominal series of x-rays was performed which showed distended loops of small bowel with multiple air fluid levels.A diagnosis of distal small bowel obstruction was made. The patient was immediately admitted and catheterised; resuscitation was instituted with crystalloids, intravenous antibiotics and nasogastric decompression. Oxygen was administered per face mask and he was taken to the operating room.At laparotomy, haemorrhagic fluid was noted on peritoneal entry. An axial 360° counter-clockwise volvulus of the midgut from terminal ileum to mid transverse colon was encountered around a very narrow mesenteric base . There w3Volvulus of the ascending colon and caecum is an infrequently encountered surgical emergency that comprises 1% of mechanical bowel obstructions . It occu10-20% of people are thought to have a mobile ascending colon , howeverAbnormalities of intestinal fixation that give rise to a persistent ascending mesocolon have their origin in in-utero events during embryogenesis, what Popky called “the gyrations and migrations of the gastrointestinal tract”. These are intricately choreographed sequential events that result in elongation, rotation, migration and fixation of the foetal bowel as well as some post-natal changes until it adopts its final normal configuration. Frazer and Robbins originally used a three-stage process to describe these events . SchwabePeritoneal zygosis is an abstruse process that involves adhesion of the primitive dorsal mesentery of the foetal gut with the peritoneum of the posterior abdominal wall resulting in “retroperitonealisation” of certain intestinal segments. This process of fixation occurs only in anthropoid apes and man and appears to be related to upright posture . For cenBalthazar postulated that excessive mobility on its own was not enough to cause volvulus of the ascending colon and that other mechanical factors were required . He suggExcessive mobility of the right colon has been linked with several clinical problems. Volvulus, as exhibited in our case, represents the extreme in terms of severity. However, patients may also present with a spectrum of non-specific chronic right lower abdominal pain that has been called the “mobile right colon syndrome”  thought 4Persistent ascending mesocolon represents a failure of zygosis during embryological development that may present with volvulus of the midgut in adulthood. General surgeons must keep this condition in their differential when a patient has recurrent abdominal pain. Prompt diagnosis and management may allow for preventative manoeuvres to salvage bowel.Nil.Nil.Ethical approval for this publication has been exempted by our institution.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by The Editor-in-Chief of this journal on request.Dennis Mazingi - case report design, subject research, consent and writing.Chenesa Mbanje - case report design, writing, research and editing.Godfrey Muguti - case report design and editing.Taurai Zimunhu - case report design, writing, research and editing.Bothwell Mbuwayesango - case report design.N/A.Not commissioned, externally peer-reviewed.


We describe a case of severe erosive oral lichen planus that led to nasopharyngeal stenosis. This is a rare clinical presentation that was ultimately, successfully treated by surgery combined with post-operative ‘nasal flossing’: a novel therapeutic intervention.A 76-year-old male suffering from a rare case of severe oral lichen planus that was resistant to conservative measures is described. Initial surgery was complicated by recurrence of nasopharyngeal stenosis. Definitive surgery required revision of nasopharyngeal stenosis release combined with a course of post- operative ‘nasal flossing’. The technique for ‘nasal flossing’ is described and demonstrated in photographs. The patient remained asymptomatic at 3 years using this combined approach, with restoration of olfaction, taste perception and voice quality, significantly enhancing quality of life.Erosive oral lichen planus is a rare but important presentation in oral medicine. We found ‘nasal flossing’ to be a successful treatment to maintain nasopharyngeal patency following surgical repair of this uncommon condition. We are not aware that this combined approach has previously been described in the published literature.Severe erosive oral lichen planus can lead to nasopharyngeal stenosis. Nasopharyngeal stenosis in these patients may be refractive to conventional surgical approaches. ‘Nasal flossing’ is demonstrated to be both practical and acceptable as a surgical adjunct in these difficult to treat cases of recurrent nasopharyngeal stenosis. This report has relevance for all those practicing oral and maxillofacial surgery, ear nose and throat surgery and oral medicine. The precells, leading to basal cell degeneration and sub-epithelial band like infiltration by T- lymphocytes . The aetThere are six different clinical subtypes; reticular, papular, plaque-like, atrophic (erythematous), erosive (ulcerative) and bullous . The mosThe most frequently cited complication of OLP is malignant transformation and 1.1% of patients with OLP develop oral squamous cell carcinoma . Lichen To our knowledge, there have been no reported cases of nasopharyngeal stenosis (NPS) occurring as a complication of OLP. NPS is an obliteration of the normal communication between the nasopharynx and the oropharynx resulting from the fusion of the tonsillar pillars and soft palate to the posterior pharyngeal wall . NPS is We present a case of NPS caused by OLP, and we also describe a novel technique for prevention of its recurrence following surgical intervention. This work is reported in line with SCARE criteria . The pat2A 76-year-old retired Caucasian male was referred for an oral and maxillofacial surgery opinion by his medical practitioner complaining of severe oral discomfort, altered phonation and impaired olfaction and gustation. Clinical examination indicated severe erosive lichen planus affecting most surfaces of the oropharynx . EndoscoIncisional biopsy of a representative area was obtained to confirm the clinical diagnosis. An initial three-month period of medical therapy included fluticasone propionate aqueous nasal spray (50 μg three times daily) and betamethasone oral rinse .Following three months of topical corticosteroid use the patient reported compete resolution of oral discomfort but still experienced impaired olfaction and gustation and still perceived changes to voice pitch and resonance.On examination, the oral mucosa appeared macroscopically healthy  but the Four months following the above procedure the patient reported improved olfaction and gustation, and improved tone of voice. Clinical examination confirmed that there was no recurrence of the previously observed NPS.However, by six months a relapse of erosive OLP had resulted in the recurrence of adhesion between the soft palate and posterior pharyngeal wall. This resulted in NPS and a return of symptoms. The relapse of NPS occurred despite continued use of the topical corticosteroid regimen.Following informed consent, the patient underwent a second surgical division as described above. Nasopharyngeal airways were used as a stent to allow epithelialisation. Following removal of the nasopharyngeal airways, a novel transnasal-transoral ‘flossing technique’ was employed to maintain separation of the inflamed tissues.2.1A 3.5 by 450 mm oval silicone sling  Ltd, Leeds) was introduced through the anterior nares and advanced along the floor of the nasal cavity until it emerged into the oropharynx so that it could be retrieved and pulled out through the mouth. Gentle traction was applied to both ends of the sling so that the soft palatewas pulled away from the posterior pharyngeal wall. The sling was manoeuvred medially and laterally by angling the oral part of the sling. The procedure was then repeated on the opposite side A–B.Fig. At outpatient review, two-weeks following surgery, the patient was taught how to insert the sling until it reached the nasopharynx, and then to retrieve the free end by flexing the neck so that the head was in face down position. He then used an explosive cough to expel the free end into the oral cavity where he could retrieve it and manoeuvre it as described above.There was no recurrence of NPS at one year following the second surgical release. The patient reported normal olfaction, gustation and speech. Nasal flossing was performed daily for a duration of approximately one minute each day. It was well tolerated by the patient, and there were no observed complications during follow-up and preparation of this report. No further surgical intervention has been required at three years3Historically, the main cause of NPS was infection, with the majority of cases being due to tertiary oropharyngeal syphilis . In contPatients with NPS frequently suffer significant morbidities, including phonatory changes, sleep disordered breathing, and otologic disturbances .Steroid therapy is the mainstay of treatment of OLP . A CochrThe treatment of NPS is challenging as there is a high rate of recurrence . TreatmeIn this case, despite an initially successful result achieved by surgical release and stenting, restenosis occurred due to the inherent tendency for the inflamed tissue to reattach.The multitude of both local and regional methods described in the literature for surgical correction of NPS rely on the availability of healthy tissue in proximity to the site of repair. This was not available in this case due to extensive oro-nasopharyngeal involvement.In this case the primary repair involved separation of the soft palate from the posterior pharyngeal wall, with minimal excision of scar tissue. Nasopharyngeal airways were then used as stents to maintain position of the soft palate while epithelial migration occurred.The use of silicone slings and the ‘nasal flossing’ technique described above is a novel approach to maintaining patency of the repair in selected patients. ‘Nasal flossing’ was successful in this case despite the increased tendency for restenosis. The choice of silastic slings (smooth surface) and a gentle technique avoided the effect of the Koebner phenomenon, which is where new lesions occur at sites of trauma.4We have described a case of severe erosive oral lichen planus where treatment required surgical release and ‘nasal flossing’ to maintain a patent nasopharyngeal airway. To our knowledge, this is the first case of oral lichen planus causing nasopharyngeal stenosis to be reported. This is also the first report of ‘nasal flossing’ as a treatment for this condition.‘Nasal flossing’ has potential application as an adjunct in the management of any of these causes for nasopharyngeal stenosis, and as such this report has relevance for all those practicing oral and maxillofacial surgery, ear nose and throat surgery and oral medicine.Use of ‘nasal flossing’ may allow for the utilisation of less complex primary surgical interventions in cases of nasopharyngeal stenosis, as clinicians may have confidence that the patency of the repair can be maintained in the longer term in suitable patients.None.This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.This study is a case report and does not require ethical approval in its current form.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.AH analysed and interpreted data, drafted the article and approved final version. JB analysed and interpreted data, revised drafted article and approved final version. JM conceived study and acquired data; critically reviewed article and approved final version.This case report does not require registration as a research studyThe Guarantor is Professor James McCaul.Not commissioned, externally peer reviewed.