We report the case of a patient with recurrent pterygo-palatal angiofibroma and its treatment. A 21-year-old male patient had a long history of recurrent epistaxis with progressive nasal obstruction. He was diagnosed with an angiofibroma centered in the right pterygo-palatine fossa. Initially, he underwent surgical excision with removal of the entire tumor. The evolution was clinically good with no signs of recurrence on the cervico-facial scan of control (CT). Nine months after, he presented a reappearance of epistaxis. A cervico-facial MRI was performed and showed a recurrence of the tumor process, which this time was considered inextirpable, hence the decision to opt for radiotherapy with intensity modulated radiation therapy (IMRT). He has improved clinically with a clear reduction in tumor mass on CT scan. This technique represents an interesting alternative to overcome anatomical complexity of the region, cover the tumor and preserve the organs at risk. Pterygo-palatal angiofibroma or juvenile angiofibroma (JA) is a rare vascular and fibrous tumor with an incidence of 0.05-0.5% of all head and neck neoplasm, and affects young males aged within the range of 12-35 years [et al. (A 21-year-old male patient with a long history of recurrent epistaxis, with onset of progressive right unilateral nasal obstruction, motivated him to consult the Ear, Nose and Throat Department, where he initially benefited from a nasofibroscopy that showed a right pterygo-maxillary fossa process. On physical examination, there was no cervical adenopathy palpable. A cervico-facial CT scan and brain level was performed and showed a hypervascular tumor process centered on the right pterygo-maxillary fossa. The diagnosis of an angiofibroma was confirmed by biopsy with anatomopathological and immunohistochemical analysis. He was operated on with total removal of the tumor. The clinical course was marked by an improvement in the patient's clinical symptomatology with disappearance of epistaxis and nasal obstruction. CT scan of the control facial mass after surgery showed no sign of tumour recurrence. After a free interval of nine months after surgery, the patient presented a reappearance of the epistaxis and obstruction, which this time associated with a notion of right temporal headaches. In addition, it was refractory to usual analgesics, and was associated with decreased visual acuity in the right eye. The patient consulted again in the Ear, Nose and Throat Department. The physical examination found visual acuity at 6/10 of the right eye and 10/10 of the left eye. The fundus of both eyes was normal. There was no cervical lymphadenopathy. A nasofibroscopy done in the patient who showed a recurrence of his tumor process . The expet al. . The patet al. . The ophet al. .JA is a tumor that originates from the posterolateral wall of the arch of the nose, where the pterygoid process of the sphenoid bone and the sphenoid process of the palatal bone constitute the sphenopalatal foramen. From there, several extension patterns occur, mainly towards the nasopharynx, resulting in clinical signs that are often confusing to the uninformed clinician . For exaRadiotherapy and surgery are the main therapeutic modalities for pterygo-palatal angiofibroma. Although it is not indicated as a first-line treatment, and although pterygo-palatine angiofibroma is a benign tumor, radiotherapy represents an interesting alternative in cases of intracranial extension that cannot be surgically removed.


Parathyroid adenomas are most commonly diagnosed when symptoms consistent with primary hyperparathyroidism arise. However, certain parathyroid glands may enlarge without such symptoms. Described here is a case in which a patient presented with acute signs of unilateral cervical point tenderness, dysphagia, and odynophagia. Calcium and parathyroid hormone levels tested within normal range. Imaging revealed an enlarged right-sided mass, with compression of the trachea-esophageal groove and potentially the right recurrent laryngeal nerve. Surgical excision was performed, and final pathology revealed an infarcted parathyroid adenoma. Clinical symptoms promptly resolved thereafter. Current NIH criteria for parathyroidectomy include various symptoms of hyperparathyroidism but do not include the above findings. Nonsecreting parathyroid adenomas rarely cause laryngeal symptoms, as this has only been documented once before. Parathyroid adenomas (PAs) are by far the most common cause of primary hyperparathyroidism. The pathogenesis of their development is thought to arise from clonal expansion caused by genetic mutation . AlteredHere, we discuss a unique case in which a patient presented to our institution with acute unilateral cervical tenderness, dysphagia, odynophagia, globus pharyngeus, and dysphonia as her only symptoms. Lab values, including PTH and calcium, were within normal limits. Upon evaluation, she was found to have a mass posterior to the right thyroid lobe. Pathological examination revealed an infarcted parathyroid adenoma. There has been only one such case previously reported in the literature .A 42-year-old African American female presented to the emergency room with concerns of acute neck pain, dysphagia, odynophagia, globus pharyngeus, and dysphonia that started three days prior. Her symptoms progressively worsened over this time; she sought medical care chiefly for reduced cervical range of motion and strangled voice. She was able to localize the pain to her right anterior neck. She denied any previous episodes or alleviating/aggravating factors. The patient noted being under the care of an endocrinologist as she had a history of hyperthyroidism. She was currently euthyroid on 5 mg of methimazole. She had no history of elevated PTH or symptoms associated with hypercalcemia. Other contributory history included sick-cell trait and a tobacco abuse history of one-half packs per day for 28 years.A CT-neck with contrast was obtained as part of her work-up, which revealed a 3.1 × 1.2 × 1.7 cm solid mass posterior to the right thyroid lobe , and calcium—9.1 mg/dL (8.6–10.8). The operation was uneventful. The mass was encountered posterior to the right thyroid gland inferiorly. Fibrotic adhesions to the thyroid gland as well as the tracheoesophageal groove were noted during dissection. Grossly, the mass was oval, solid (not cystic), soft, and yellow-brown in color. It did not have a fibrofatty appearance. Intraoperatively, the right recurrent laryngeal nerve was identified in close approximation to the mass.Due to the patient's concerning symptoms associated with her neck mass, she was scheduled for surgery. Two days prior to surgery, her labs were as follows: PTH—68 pg/mL (12–88), total T3—84 ng/dL (87–178), free T4—0.6 ng/dL (0.7–1.7), TSH—1.44 The patient was kept under observation for pain control and management of the indwelling Jackson–Pratt drain. Postoperatively, her symptoms markedly improved. Her voice was intelligible without strangulation. Additionally, she admitted oral dietary tolerance without symptoms of dysphagia or pain.Grossly, the mass was described as a well-encapsulated dark pink-red soft tissue mass weighing 0.98 grams. Upon sectioning, a 1.8 × 0.8 × 0.6 cm well-circumscribed variegated tan-yellow to dark red focally softened nodule was present with all slices coming to within <0.1 cm of all margins. A rim of scant pink-tan grossly uninvolved soft tissue was located at the periphery. Microscopical sections revealed a well-demarcated nodule with encapsulated, cellular, homogenous cells composed of chief cells with some oxyphil cells in a delicate capillary network . There wPAs are the most common cause of primary hyperparathyroidism. The prevalence of primary hyperparathyroidism is 21 cases per 100,000 person-years, and this most likely is an underdiagnosed condition . There aThe incidence of nonfunctioning PAs is exceeding low. Whether this is due to a rare distinct genetic mutation, a rare inactivation event affecting PTH production or secretion in a previously functioning PA, or simply due to relative clinical silence is unclear . HoweverNonfunctioning parathyroid carcinomas (PCs) have also been described, whereas this entity usually presents with high levels of PTH; when nonfunctioning, they are more aggressive and portend a poorer prognosis . Due to In our case, serum intact-PTH was not elevated. Immunohistochemical staining demonstrated PTH within the adenoma. The most likely explanation for this clinical picture is that the infarction evidenced on pathologic evaluation caused a decrease in secretion of PTH into the bloodstream.The description of vocal fold dysfunction or acute dysphonia from parathyroid pathology is not novel. In as early as 1972, Lore Jr. et al. had described PAs causing vocal fold paralysis . The patSeveral causes of vocal fold paralysis from PAs have been described. Rapid expansion from spontaneous hemorrhage has been thought to apply pressure to the adjacent recurrent laryngeal nerve in some cases. In other cases, the sheer size of a large solid PA can cause a mass effect. The resulting stretching and pressure from either of these scenarios can cause neuropraxia or axonotmesis and subsequent impaired nerve conduction . One musIn our case, due to the acuity of the dysphonia and associated pain and in correlation with the final pathology, we do believe a recent hemorrhagic event occurred within the adenoma. This has been known to happen in the absence of a causative trauma. True vocal fold palsy may have been averted in our case by the promptness of her presentation (only three days after symptom onset), needle aspiration , and timely surgical excision.The unique sequela of vocal fold dysfunction due to a nonfunctioning PA has only been described once before. Recently, in 2016, Kamali et al. described a 49-year-old male with a 10-week history of dysphonia and globus pharyngeus . InteresThe management of parathyroid glands has been studied and, over time, adjusted. For the most part, the criteria for the surgical excision of parathyroid glands, initially proposed in 1990 by the NIH  14], fo, fo14], Unrecognized in much of the literature are the symptoms of a neck mass with cervical tenderness, dysphagia, and dysphonia associated with a parathyroid growth. These would be the only presenting symptoms of a nonfunctioning parathyroid growth and indeed some functioning growths in asymptomatic HPT patients. We believe these masses should be excised, and as such, we propose that the symptoms of cervical tenderness, dysphagia, and dysphonia be added to the guidelines for completeness.This case report is not immune to limitations. Fiberoptic laryngoscopy was performed only after needle aspiration due to scope availability. The needle aspiration may have decompressed the recurrent laryngeal nerve allowing for return of function. We assume the infarction event was acute and likely causal to the PAs nonfunction, yet the intact-PTH level drawn prior to any symptoms was within normal limits. This lab draw may predate the presence of the PA, as there was no evidence of PA on an ultrasound performed around the same time as the lab draw. Additionally, elevated serum calcium was noted in that same routine endocrine lab draw. Although the patient denied any hematuria or flank pain and creatinine clearance showed no decay of renal function, no urine calcium was on refold to disprove familial hypocalciuric hypercalcemia.In conclusion, there have been many instances in the literature that have well described parathyroid enlargement and its proximal effect on the recurrent laryngeal nerve. However, in the absence of hyperparathyroidism, a diagnosis of a PA is highly unlikely. Our patient presented with acute neck pain, dysphonia, and odynophagia, which ultimately was caused by a nonfunctioning PA. It is only the second time in literature that this has been described. Although not addressed in the current guidelines as indications for removal, we recommend that parathyroid glands presenting with the symptoms of neck mass with cervical tenderness, dysphagia, and dysphonia be surgically removed.


We report the case of a patient diagnosed with primary umbilical endometriosis intending to discuss the diagnostic and therapeutic management of this rare disease. A 45-year-old woman suffering from a painful swelling located in the umbilical region, with intact and normal cutaneous aspect, came to our attention. Ultrasonography of the umbilical region showed a nodule with a nonhomogeneous echotexture pattern. Partial omphalectomy was performed under local anesthesia in day care setting surgery. Histology confirmed the diagnosis of umbilical endometriosis. Pre- and postoperative clinical controls showed no evidence for other endometriosis localization. No medical treatment was administered. No signs of recurrence were observed after 5 years from surgery. A review of the literature of the last 10 years was generated based on MEDLINE research, selecting some specific keywords. Several lesions can occur in the umbilical region, and endometriosis has to be ruled out even in patients without any surgery in their medical history. Surgery is the gold standard treatment for this condition: partial and radical omphalectomy are the two treatment options. We believe that given the significant psychological and aesthetical value of the umbilicus, surgical treatment has to be tailored and in case of a small endometrial umbilical nodule, partial omphalectomy  with a 3 mm free border, even without adjuvant hormonal treatment, could ensure adequate and effective treatment. Endometriosis is a benign condition characterized by endometrial tissue found in anatomical localizations other than the uterus. It has been estimated that the prevalence of endometriosis ranges up to 10% in the general female population: in particular, this condition mostly affects women in fertile age (up to 40% during reproductive years) , 3. EndoA good-looking 45-year-old woman was admitted to our hospital, suffering from pain located at the umbilicus where a solid, well-circumscribed subcutaneous nodule was palpable on physical examination. The lesion size was approximately 1 × 1 cm, increasing during the menstrual cycle. The skin above the swelling appeared normal and intact. Medical history revealed a few years of cyclical pain during menstrual period, no surgical intervention nor caesarian section, and two vaginal deliveries.To better investigate the umbilical swelling, we decided to perform ultrasonography (US) of the periumbilical region, which allowed the clinical diagnostic hypothesis of PUE. In particular, US showed, in the subcutaneous layer, a roundish well-circumscribed nodule with a nonhomogeneous echotexture in the umbilicus, located close (but not attached) to the underlying muscular fascia, with no connection to intraperitoneal structures or organs. Because of the characteristic clinical and US presentation, no further imaging was needed. Then, the patient underwent surgical excision of the subcutaneous tumefaction. The procedure was accomplished under local anesthesia; after infiltration of 5 mL of 1% mepivacaine, a periumbilical semilunar incision of about 2 cm is performed around the clinically appreciable swelling (lower right quadrant). With skin dethatched, a subcutaneous nodule of about 1 cm in diameter with a typical brownish red color was detected. This nodule appeared indissociable from the overlying skin but easily dissociable with the electrocautery from the underlying fascia. En bloc excision of the nodule along with the overlying skin was performed, with a free round margin of 0.3 cm. Closure of the muscular fascia was achieved with an absorbable running suture (2/0 Polyglactin). Skin closure was completed with a subcuticular running suture (3/0 Polyamide), as for reductive onphaloplasty, ensuring an excellent overall aesthetic results. The patient was discharged on the same day. Histology confirmed the diagnosis of PUE. The patient underwent a 6-month follow-up schedule. No medical treatment was administered before and after surgery. Five years after surgery, no signs of local recurrence were observed.PUE is an unusual manifestation of extragenital endometriosis and accounts for 30-40% of all cutaneous forms of endometriosis , 9. IndeThe mean age at the time of PUE diagnosis is estimated around 35-38 years, reflecting the fact that it takes an extensive exposure to hormonal, metaplastic, and/or environmental factors before this condition reaches the clinical level. The mean elapse of time from the onset of symptoms to the final treatment has been estimated as 13.3 months . In our The clinical presentation of PUE can be variable, mainly depending on its localization in the cutaneous or subcutaneous plane. Classic clinical features include umbilical swelling with associated cyclical pain. Patients can even show a bluish-purple mass, accompanied by catamenial bleeding from the umbilicus with or without associated pain or tenderness concomitantly with the menstrual cycle. Besides, the condition can be completely asymptomatic in some cases, while in literature, cases with continuous chronic pain have been described .In patients with UE, a clinical scenario and physical examination are mainstays for diagnosis. US, MRI, and CT scan can be helpful, in particular to investigate the anatomical relationships of the nodule with the surrounding tissues. A recent Japanese retrospective study has shown that for UE, none of the imaging technique was superior in sensitivity when compared to physical examination and that US, MRI, and CT were almost equivalent . HoweverHistological confirmation is the current golden standard for PUE diagnosis, while initial assessment for clinical workup is primarily clinical. Fine-needle aspiration cytology (FNAC) can be supplementary, but inconclusive results have been reported as high as 75% . MoreoveIn our case, the clinical presentation, along with the US pattern revealing no invasion of the underlying structures, was considered sufficient to establish a presumptive diagnosis to initiate treatment.The management of UE is not standardized. Generally, medical treatment using progesterone, danazol, norethisterone, and GnRH analogues is still under debate. Indeed, medical treatment can be effective in relieving symptoms temporarily, but recurrences are frequent after treatment cessation . Thus, dLocal resection of the umbilical endometrial nodule should be achieved with adequate borders of healthy tissue. Respecting sufficient borders and the integrity of the nodule itself is vital to minimize local recurrences after surgery . The litTo provide the reader with a comprehensive and detailed picture of the state of the art and to compare therapeutic choices with prognosis, the MEDLINE database was searched using the keywords “primary umbilical endometriosis”, “primary endometriosis of umbilicus”, “spontaneous umbilical endometriosis”, and “Villar's nodule”. Open and English-written articles of the last 10 years discussing cases of PUE with a reported follow-up were selected. The results are shown in The significance of the aesthetic and psychological value of the umbilicus requires tailored and therapeutic appropriateness. Since ancient Greece and in several old cultures, seductive power has been attributed to the umbilicus . The Greek myth tells that Heracles, fascinated by the vision of the Queen of Lidia Omphale, bent to the will of the latter to the point that not only did he give her his club and lion skin but also he had himself reduced to a humble servant, carding her wool .Nodule size and relationship with the musculoaponeurotic fascia and the peritoneumOther locationsAge of the patientDesire for pregnanciesAesthetic expectationsFor each patient with a PUE diagnosis, the following factors should be considered:The clinical case we treated meets the efficacy and efficiency criteria because no recurrence was noticed during a 5-year follow-up period, and the patient kept a satisfactory aspect of the umbilicus. Before surgery, the patient only underwent an ultrasound examination which strengthened the diagnostic hypothesis of an endometrial nodule and specified its suprafascial position, allowing us to plan and carry out the intervention under local anesthesia and in the day care setting.Based on our case and literature review, we believe that for small PUE nodules, without involvement of the musculoaponeurotic plane and other endometriosis localizations, partial omphalectomy  with a 3 mm free border, even without adjuvant hormonal treatment, could ensure an adequate and effective treatment.


Primary tubal cancer is very rare, most are diagnosed intra and post operatively. Histopathology is vital in determining the cancer origin. Here we present a case of fallopian tube cancer with clinical presentation mimicking endometrial origin.A 74-year old patient came with complaints of intermittent post-menopausal bleeding and pelvic pain. The patient had several investigations using Ultrasonography, Hysteroscopy-guided biopsy, and Magnetic Resonance Imaging. Pre-operative diagnosis was endometrial cancer based on histopathology of endometrial biopsy during hysteroscopy. Explorative laparotomy, total abdominal hysterectomy, bilateral salphingo-oophorectomy, pelvic and para-aortic lymph node dissection were then performed, and the tumor samples were sent to the histopathology laboratory. It was found that the post-operative diagnosis was in fact primary fallopian tube cancer stage IIB.For patients with gynecological malignancies, rare cases such as fallopian tube cancer should never be overlooked as a differential diagnosis. •74 year old female, originally diagnosed as endometrial cancer.•USG, Hysteroscopy biopsy, and MRI suggested endometrial cancer.•The endometrial tumor is spillage of fallopian tumor.•Final diagnosis was found to be Primary Fallopian Tube Cancer. Vaginal bleeding is the most commonly reported symptom of FTC and is present in approximately 50% of the patients. Other clinical symptoms suggestive of FTC include pelvic pain, watery vaginal discharge, and pelvic mass [Primary fallopian tube carcinoma (FTC) is a very rare gynecological malignancy. Despite being rare, most high-grade serous carcinomas arise from the fallopian . Classicvic mass . Howevervic mass . These nHere we report a rare case of primary fallopian tube carcinoma supported with intraoperative findings and histopathological examination. We hope to highlight the clinical presentation of FTC that is commonly underdiagnosed.2This Evidence Based Case-Report is made in line with the SCARE criteria .A 74-year-old female came with post-menopausal bleeding lasting for a month. The bleeding occurred intermittently, approximately 1–2 pads each day. The patient also complained of intermittent pelvic tenderness which was more severe on the right side. Patient had 2 living children via spontaneous vaginal delivery with no abortion history, she had menopause 22 years ago. Past personal and familial medical history were unremarkable. Normal findings were found upon physical and gynecological examinations.Initial ultrasonography  findings suggested that the uterus cavity was filled with fluid due to blockage at the endocervix. However, the cause could not be determined. Patient was then referred to a tertiary hospital for further investigation. Upon hysteroscopic examination by a gynecology oncology consultant with more than 10 years of experience, several glomerular mass with atypical vessels resembling malignant endometrial lesion were found. The appearance from the biopsy was suggestive for serous Endometrial Carcinoma grade II presumably from the endometrium. There were neither normal endometrial tissue nor hyperplastic zone to be identified .Fig. 1PrHistopathologic specimen showing cancerous cells with no normal tissue (A) 40x zoom and (B) 100x zoom. (C). Glomerular growing mass with atypical vessels.the uterus with suspicion towards ovarian cyst with of 34/37/42 mm, which is not part of the discussion. Other findings were within normal limit  found two masses: at the tube and endometrium. Endometrial mass was prominent to anterior junctional zone with 13 mm thickness, did not invade the myometrium, and covered <50% of the endometrial surface in accordance to T1A-N0-M0 staging . There wal limit .Fig. 2MRFollow-up USG on September 25th, 2018 revealed collapsed uterine cavity. Presence of intra-cavitary malignancy could not be ruled out. There was no widening of parailiac and bilateral of paraaortic lymph nodes.On October 4th, 2018 surgery was performed by a senior gynecology oncology consultant with more than 15 years of experience. Adhesiolysis and surgical staging laparotomy  were conducted.-grade serous carcinoma from the right fallopian tube  carcinoma for both endometrium and fallopian tubes are very similar . Thus, i4Despite rigorous examination, the diagnosis of FTC was missed. It was only at the latest stage that the final diagnosis was found. Regardless, a lesson can be learned from this case that due to its unpredictable nature, FTC should not be forgotten as a differential diagnosis. Therefore, the surgeon should always be prepared to adjust the operating procedure in accordance with intra-operative findings.Written informed consent was obtained from the patient for publication of this case report and accompanying images.No personal detail of the patient has been stated in the manuscript.No external funding received. The study is self-funded.Sigit Purbadi, Victor Prana Andika Santawi, and Laila Nuranna designed the case report.Sigit Purbadi and Victor Prana Andika Santawi wrote the manuscript.Laila Nuranna, Sahat Matondang, and Hartono Tjahjadi supervised and provided their expertise in this case report.The manuscript is a case report that does not involve experiments to human participants.Written informed consent was obtained from the patient for publication of this case report and accompanying images.No personal detail of the patient has been stated in the manuscript.Sigit Purbadi.All authors declare that there is no conflict of interest.


Solitary pulmonary capillary hemangioma (SPCH) is a rare benign lung tumor that clinically resembles early lung cancer and precancerous pulmonary lesions that present with similar imaging manifestations.The patient was a 54-year-old Japanese man who was referred to Nagara Medical Center with a ground glass opacity (GGO) lesion within the right upper lung that was incidentally detected on computed tomography. After 8 months of follow-up, video-assisted thoracoscopic segmental resection of the right upper lobe was performed with diagnostic and therapeutic intent. Pathologic examination of the resected specimen demonstrated thickening of the alveolar septum caused by the proliferation of capillary vessels. This lesion was positive for CD31 and CD34 and negative for thyroid transcription factor-1 and cytokeratin on immunohistochemical staining. The tumor was diagnosed as SPCH pathologically.When radiological examination demonstrates a GGO in the lung, SPCH must be considered as one of the differential diagnoses. For a definitive diagnosis, pathological examination of a surgically resected specimen must be conducted.This study describes a case of SPCH and a review of the literature. PreoperThis case is reported in line with the SCARE criteria .2A 54-year-old Japanese man was referred to Nagara Medical Center for the evaluation of a GGO, which was found incidentally within the right upper lung on computed tomography (CT). Chest CT showed a pure GGO lesion measuring 8 mm in diameter in the anterior segment of the right upper lobe . The pat3The ﬁrst report of a surgically resected case of SPCH was published by Fugo et al. in 2006 . The numSPCH is difficult to diagnose because of a lack of typical symptoms. Radiographic findings often include pure or mixed GGOs, which may lead to suspicions of adenocarcinoma in situ (AIS), atypical adenomatous hyperplasia, and focal inflammation ,6. In thMicroscopically, an SPCH typically appears as a solitary lesion with densely proliferating and dilated capillaries within the alveolar septum, which is composed of a single layer of ﬂattened or cuboidal endothelial cells without cytological atypia ,3. The iThe characteristic immunohistochemical findings of SPCH have been reported. The expression of epithelial cell and histiocyte markers, such as cytokeratin, TTF-1, and CD68 (KP-1), is negative. Cells of the alveolar septa are positive for CD31 and CD34 ,3.All reported patients with SPCH were asymptomatic. No patients have died from the disease . However4GGO lesions may represent an SPCH or AIS/early lung cancer. Because the prognosis of each of these diseases is quite different, a pathological examination involving immunohistochemistry must be conducted to ensure the establishment of the correct diagnosis.The authors declare that they have no competing interests.No sources of funding.We have reported a single case, not a clinical study, with no requirement for ethical approval.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.Dr Teruya Komatsu: Investigation, Writing – original draft, Writing – Review and Editing, Conceptualization, Visualisation.Dr Naoki Date and Dr Takuji Fujinaga: Conceptualization, Writing – Review and Editing.Dr Akira Hara and Dr Tatsuo Kato: Writing – Review and Editing, Supervision.Not applicable.Teruya Komatsu and Takuji Fujinaga are the guarantors of this work. Thus, they have full access to all study data and take responsibility for the integrity and accuracy of such data.Not commissioned, externally peer-reviewed.


Microsporum spp. or Trichophyton spp. genre. We present a 24-year-old man with an exuberant tumor in the occipital region with fistula, whose histopathological examination evidenced grains composed of hyaline hyphae and the culture for fungi isolated the agent Microsporum canis. Combined treatment of surgical excision followed by oral griseofulvin for two years was performed, with resolution of the condition.Pseudomycetoma is an extremely rare deep mycosis, caused by dermatophytic fungi that penetrate the tissue from infected follicles of tinea capitis. Both clinically and histopathology are similar to eumycetoma, being distinguished through the isolation of the fungus, which in the case of pseudomycetoma can be  Microsporum canis (Microsporum canis was confirmed. The patient was submitted to surgical excision of the tumor and associated oral griseofulvin, one gram per day for two years. In a one year follow-up after the end of griseofulvin, the patient showed no signs of relapse (A 24-year-old immunocompetent male, with tumoral lesion in the occipital area which started at two years of age with areas of alopecia that progressively evolved to a tumor. His first consultation was at age 14, presenting at the clinical examination a hardened tumor with granular fundus ulcerations. He abandoned follow-up, returning ten years later with a considerable increase of the lesion . Materiaum canis . Histopaum canis . He deni relapse .Figure 1Microsporum canis, Trichophyton tonsurans and T. mentagrophytes. According to these authors, mycelium aggregates formed by the dermatophytes would be pseudo-granules and the term pseudomycetoma should be applied to this deep dermatophytic infection.7Chronic inflammatory and invasive forms of dermatophytosis are the result of an intense hypersensitivity reaction to the fungal infection, more frequent in immunocompromised individuals.Clinical aspects of pseudomycetoma are identical to those of eumicetoma, yet in contrast to mycetomas, pseudomycetomas are more common in the scalp and do not have a history of trauma for its inoculation.M. canis is surgical excision of the fungal mass, since the systemic antifungal does not reach therapeutic concentrations,The treatment of pseudomycetoma by None declared.Ligia Rangel Barboza Ruiz: Approval of the final version of the manuscript; conception and planning of the study; elaboration and writing of the manuscript; obtaining, analysis, and interpretation of the data; effective participation in research orientation; intellectual participation in the propaedeutic and/or therapeutic conduct of the studied cases; critical review of the literature; critical review of the manuscript.Clarisse Zaitz: Approval of the final version of the manuscript; conception and planning of the study; obtaining, analysis, and interpretation of the data; effective participation in research orientation; intellectual participation in the propaedeutic and/or therapeutic conduct of the studied cases.Clarisse Zaitz: Intellectual participation in the propaedeutic and/or therapeutic conduct of the studied cases.John Verrinder Veasey: Approval of the final version of the manuscript; elaboration and writing of the manuscript; critical review of the literature; critical review of the manuscript.None declared.


Teaching Point: The typical imaging features of a retroperitoneal solitary fibrous tumor are relatively large-sized, have a well-defined margin, an avidly heterogeneous enhancement on arterial phase, a prolonged enhancement on delayed phase, and serpentine vessels along the periphery of the tumor. A 30-year-old female was admitted to our hospital after an elevated carbohydrate antigen  was discovered on a health check-up. Her past medical history was unremarkable. Contrast-enhanced computed tomography (CT) was performed to check for abdominal malignancy. Axial and coronal reformatted arterial phase CT images Figure , B, C shSFTs are rare spindle-shaped cell tumors that account for less than 2% of all soft-tissue tumors . They arLess than 100 cases of retroperitoneal SFTs have been reported in the English literature, with a mean size of approximately 9 cm [


Case Presentation. Total thyroidectomy, airway resection and bilateral neck dissection were performed in two cases diagnosed as advanced thyroid cancer with tracheal invasion (stage IV according to McCaffrey). The first case underwent partial tracheal resection and direct anastomosis by the V-shape technique, while the latter one required tracheal resection and permanent tracheotomy. After one-year follow-up, no evidence of tumor recurrence or any postoperative complications were found.  Tracheal invasion in thyroid cancer occurs in one-third of locally advanced cases and is the third most common site of infiltration following strap muscles and recurrent laryngeal nerves. Surgical resection plays an important role in the management strategy followed by either radioactive iodine or external beam radiotherapy. Nonetheless, there has been still controversy about the optimal extension of the surgery.  Surgical resection still remains the mainstay of management for advanced thyroid cancer in general and for tracheal invasion cases in particular. The decision of surgical resection and tracheal reconstruction methods mostly depends on the extent of tracheal invasion. Thyroid cancer is the original from of follicular derived cells or neuroendocrine C-cell-derived types . It is cThe standard treatment of DTC with tracheal invasion is still under controversy. In previous studies, some authors reported that total thyroidectomy and involved structure resection with negative margins could improve survival rates –6. HowevIn this paper, we reported two cases of DTC invading the trachea with different surgical approaches and favorable outcomes.A 54-year-old man with normal previous medical history was admitted to our department because of a mass in his left neck. The patient reported that the tumor had appeared before one year. However, he did not receive any treatment because this tumor did not affect his daily activities. As a result, for the last few months, the tumor had gradually increased in size, and the patient had experienced increasing hoarseness. On examination, there was a 3 × 4 cm, hard and fixed tumor in his left neck without any palpable neck lymph nodes.Ultrasound suggested a highly suspicious malignant lesion in the left lobe of the thyroid gland, categorized as TI-RADS 5 (according to Thyroid Imaging Reporting and Data System (TI-RADS) 2017). Neck CT scan as per  demonstrth edition, 2017) and stage IV (according to McCaffrey). Intraoperative evaluation reported a hard tumor in the size of 30 × 40 mm at the left thyroid lobe, invading the left recurrent laryngeal nerve and the anterior wall of trachea with 30 mm in length. There were multiple lymph nodes in the central compartment and the bilateral neck compartments. The patient underwent total thyroidectomy, central and bilateral cervical lymph nodes dissection, partial tracheal resection, and reconstruction by direct suturing with the V-shape technique , HBME1, Claudin1, and Ki67 (2%) and negative with CD 56 and p63.A 55-year-old man presented to our hospital with a hard mass in his neck. He noticed that the mass had been gradually increasing in size for six months. In the last month, he reported of having experience of hoarseness and dyspnea. In addition, his previous medical history was unremarkable. Clinical examination showed not only the hard mass with a size of 3 × 4 cm and limited movement but also bilateral cervical lymph nodes.Neck ultrasound and 128-slice computed tomography scan as per  revealedth edition, 2017) and stage IV . Because endotracheal intubation was not applicable due to constricted trachea, tracheostomy was performed for anesthesia. Intraoperative evaluation has shown that (i) the tumor invaded the trachea in the length of approximately 7 cm and (ii) reconstruction of the remaining parts of the trachea after resection was impractical. Eventually, total thyroidectomy, resection of the involving part of the trachea, and permanent tracheotomy . However, in patients with advanced thyroid cancer, it is necessary to perform laryngoscopy, neck CT scan, or neck MRI and sometimes tracheobronchoscopy . LaryngoThe most common staging system applied to evaluate the extent of tracheal invasion in aggressive thyroid cancer is the classification described by Shin and McCaffrey . AccordiRegarding thyroid cancer, tracheal resection could be repaired by the following procedures: (1) shave procedures, (2) segmental/partial resection and direct closure, (3) partial resection together with reconstruction of musculocutaneous flap or cartilage graft or materials, and (4) total laryngectomy and permanent tracheotomy. For stages II and III according to McCaffrey, the resection of all gross tumors can be performed by “shave procedures” meaning the removal of a partial thickness of the airway tract wall. Several retrospective studies comparing radical resection and shave procedures combined with RAI showed no survival benefit in the patients undergoing radical resection –18. Simith to 7th postoperative day [In several previous studies, postoperative complication rate after tracheal resection with direct closure ranges from 15% to 39%, and postoperative mortality rate is roughly 1.2% , 21. Thetive day –25 or byen bloc resection of involved trachea is the standard treatment in advanced thyroid carcinoma. The surgical methods are based on the extent of airway wall invasion. The procedure of tracheal resection is safe with low rate of mortality.In conclusion,


It was first described by von Hansemann in 1901 and by Michaelis and Gutmann in 1902. The urinary system is the most commonly involved site. Female genital tract involvement is extremely rare. Treatment is prevalently based on antibiotics with surgical intervention sometimes necessary. Prognosis is usually good, but relapse may frequently occur.Malacoplakia is a rare chronic inflammatory disease. The name derives from the Greek “μαλακός” meaning “This report illustrates the first case of endometrial malacoplakia in a 40 years-old patient who received endometrial curettage due to the retention of placental rests following an abortion. After conspicuous vaginal sero-hematic secretions, the patient received a further curettage. The histological examination did not show any retention of chorionic rests, but an endometrial and myometrial infiltration of histiocytes with large granular cytoplasm within a chronic inflammatory background. Immunoreactivity for CK-pool was negative, while CD68 immunostaining was strongly positive.Malacoplakia of endometrium is an extremely rare condition, with few cases reported in the whole international literature. In this paper, we present the first case associated to an abortion followed by endometrial curettage procedures. This rare disease should always be attentively examined, considering, among differential diagnoses, uterine neoplasms or physiological conditions such as cumulus of foamy macrophages in the endometrium. Malacoplakia is a rare chronic inflammatory disease, with a mean age at diagnosis of 50 years old and a female to male ratio of 4:1. It occurs more frequently in patients with immunodeficiency conditions. The disease was first described by von Hansemann in 1901 and by Michaelis and Gutmann in 1902. The name malacoplakia derives from the Greek words “μαλακός”, which means soft, and “πλάξ”, meaning plaque, and is related to its macroscopic appearance: soft and friable yellow plaques or, more rarely, nodules or masses. Histologically, it is characterized by a mixed inflammatory infiltrate, composed of lymphocytes, plasma cells, neutrophils and aggregates of hystiocytes with abundant granular eosinophilic cytoplasm, known as von Hansemann cells. The genitourinary tract is the most common involved site, followed by the gastrointestinal tract, but any site of the human body may be affected. The female genital tract is a very rare localization, with only few cases reported in literature, involving vagina, uterus, fallopian tubes and ovary. The most common presentation of endometrial malacoplakia is post-menopausal bleeding. We report herein the first case of endometrial malacoplakia associated to an abortion followed by uterine curettage procedures.A 40-year-old female patient was admitted to our hospital for conspicuous vaginal sero-hematic secretions. Anamnestic information asserted that the patient previously received three endometrial curettage after a surgical abortion procedure. Histological examination after each endometrial curettage showed regression of chorionic villi and decidualization of the endometrium and the diagnosis of placental rests retention was made. After being admitted for conspicuous vaginal sero-hematic secretions, the patient received a further curettage in order to assess the possible retention of placental rests. The histological evaluation did not show any retention of chorionic rests, but demonstrated an endometrial and myometrial diffuse chronic inflammatory infiltration, with an extensive background of hyaline stroma. The inflammatory infiltrate showed a variegate composition, being made of lymphocytes, plasma cells and occasional polymorphonuclear leukocytes, but the majority of the cellular elements was represented by histiocytes with abundant granular cytoplasm and basophilic inclusions, morphologically relatable to the so called von Hansemann cells Fig. . PeriodiEndometrial malacoplakia is an extremely rare condition, as demonstrated by the paucity of cases reported in the international literature. The etiopathogenesis of the disease is far from being completely understood, but recent evidence agrees with the presence of defective macrophage phagolysosomal activity . Several


Retrocaval ureter (RCU) or circumcaval ureter is a rare cause of congenital hydronephrosis. The surgical correction of RCU should be performed in all patients with obstruction and hydronephrosis symptoms, lumbar pain, urinary tract infections, hematuria, or urolithiasis. Traditionally, an open surgical approach was used for the treatment of RCU. Nowadays, surgical correction of these anomalies is performed using minimally invasive techniques. We report on two cases treated with our standardized laparoscopic technique using only three 5-mm trocars. The proposed approach could be considered as the first-line treatment for RCU. Retrocaval ureter (RCU) or circumcaval ureter is a rare cause of congenital hydronephrosis. Although its exact incidence remains unknown, its estimated incidence is 1 in 1,100 live births per year,From January 2016 to January 2018, two female patients with symptomatic RCU were examined. Their ages at the time of surgery were 22 and 18 years. Body weights were respectively 72 and 56 kg. Patient 1 underwent augmentation enterocystoplasty with bilateral ureteral reimplantation for spinal dysraphism at the age of 5 years. The RCU was not recognized at that time, because the refluxing ureter was grossly dilated and tortuous. Recently, she presented recurrent episodes of right flank pain and two episodes of acute pyelonephritis, which were treated with antibiotics. No reflux was present in the cystogram. Patient 2 showed intermittent gross hematuria with several episodes of flank pain since the age of 12 years. Abdominal MRI with three-dimensional reconstruction revealed that both the patients had a typical S-shaped deformity of the right lumbar ureter, according with type 1 variant of RCURCU is a rare congenital anomaly generally associated with upper urinary tract obstruction and hydronephrosis. Its typical radiological presentation is an S- or sickle-shaped appearance, due to the passage of the ureter at the posterior side of the IVC.Our technique of RCU management utilizes only three 5-mm trocars, without any preoperative cystoscopy or indwelling ureteral catheter insertion. No critical difficulty was found in the intra-abdominal adhesions in patient 1. The simplicity and reproducibility of our procedure are its main advantages. We believe that this is a safe, feasible, effective, and minimally invasive approach, and therefore could be considered as the first-line treatment for RCU.


Teaching point: Barium granuloma is a rare but potentially risky complication of barium studies that should be prevented, especially in susceptible patients. An 81-year-old man presented with a two-month history of abdominal distention and pain with on-and-off fevers. There was a clinical suspicion of intestinal obstruction and a subsequent CT scan was performed. Abdominal CT Figure , arrow, Barium granuloma, also called barytoma or barium stone formation, is a rare complication of the barium contrast use in the gastrointestinal tract. It has been mostly described in the colon after barium enema studies but may occur in any part of the alimentary tract. It is often an incidental finding; however, it may rarely produce a polypoid, ulcerated or intramural lesion resembling a neoplasm. Barytoma may erode the mucosa and cause perforation, in which case it may remain visible for years or may result in acute chemical or chronic granulomatous peritonitis . Pre-exi


Nevus sebaceous (NS) is a lesion caused by congenital hyperplastic disorder of the sebaceous glands. It commonly noted in the scalp and face and rarely in the trunk, neck, or oral mucosa. We present a rare case of a lesion arising in the genital region.A 47-year-old woman complained of a gradual increase in the size of her bilateral labia minora over 2 years, which affected her sexual life and caused walking difficulty. She was admitted to the Department of Obstetrics and Gynecology. On physical examination, no ulcer, discharge, and vulval or vaginal bleeding were found. The bilateral inguinal lymph nodes were not palpable, bilateral labia minora were asymmetric, and the right side was evidently bigger than the left. The labia minora had serrated edges and numerous papillae with a maximum diameter of 0.5 cm. The vagina, cervix, and uterus with its attachments were normal. Blood samples tested negative for human immunodeficiency virus, human papilloma virus, hepatitis B virus, and hepatitis C virus.A diagnosis of NS of the bilateral labia minora was made following histopathological examination of the resected specimen.The bilateral labia minora lesions were resected general anesthesia on August 29, 2016. The operation was successful, and intraoperative blood loss was about 10 ml.After 40 months of postoperative follow-up, no recurrence or appearance of other tumors were noted.We recommend surgical removal of lesions in the genital area during adolescence or before adulthood. Adolescence may be the best period for surgical intervention owing to a greater risk of malignant change in adulthood. On the other hand, surgical risk should be avoided in children considering the low incidence of malignant transformation. It is also called a sebaceous gland hamartoma and was first reported by Jadassohn in 1895. The onset of NS typically occurs at birth, and occasionally, in adulthood. In childhood, NS presents as an elevated hairless patch, that is pale yellow with a smooth surface and waxy luster. In adolescent patients, because the sebaceous gland can be fully developed by then, the nevus presents as nodular, petaloid or verrucous lesions. In elderly patients, NS presents as a brown, hard verrucous lesion. It rarely occurs in other locations such as the eyelid and the oral mucosa, where it presents as plaques or nodules; multiple lesions are also a possibility.–5 NS occurring in the genital organs is extremely rare, with only 5 cases reported in the literature to-date, out of which only 3 have been reported in detail.,7 Since the specific morphological characteristics of genital organ NS are scarcely reported in the literature, clinical diagnosis is difficult, and cases can be easily misdiagnosed. This paper, with relevant literature review, elucidates the morphologic characteristics of labia minora lesions, that can aid plastic surgeons, dermatologists, and gynecologists to arrive at a suitable differential diagnosis and treatment strategy.NS most frequently occurs on the head and neck, and usually involves the scalp (87%–95% of cases) resulting in alopecia.22.1A 47-year-old woman of Han ethnicity with obstetric history G2P0L1A1, who complained of gradually enlarging labia minora over 2 years, was admitted to the hospital on August 26, 2016.2.2Two years prior to admission, she found the right labia minora to have gradually enlarged; it was soft to touch and deep in color. There were no symptoms of swelling, erosion, or pain in the right labia minora. Subsequently, she found that the left labia minora was gradually enlarging too. The bilateral labia minora were asymmetrical, with the right side being larger than the left. Three months before admission, the labia minora enlarged rapidly, accompanied by mild tenderness that affected sexual life and walking to a certain extent. There were, however, no complaints of ulcer, discharge, and vulval or vaginal bleeding.2.3The patient had a 2-year history of hypertension and regularly took telmisartan 40 mg/d to maintain a blood pressure of 120 to 130/80 mm Hg.2.4The patient maintained a normal diet, had no significant change in weight recently, and had no history of bowel or bladder dysfunction. Her menstrual cycles were normal; there was no history of radiation or poison exposure, previous surgeries, or relevant illnesses in the family.2.5Systemic examination was found to be normal. On local examination, the bilateral inguinal lymph nodes were not palpable. The bilateral labia minora were asymmetric, with the right side being evidently bigger than the left. The labia minora had serrated edges and numerous papillae with a maximum diameter of 0.5 cm Fig. A–C. The 2.6Blood test results showed that the blood type was O (+). Moreover, tests for human immunodeficiency virus, human papilloma virus, syphilis, hepatitis B virus, and hepatitis C virus were all negative; serum cancer antigen 125 level was 13.35 U/ml and carbohydrate antigen 19-9 level was 7.41 U/ml. The cervical pap smear showed normal results.2.7A transvaginal color Doppler ultrasound was suggestive of a uterine adenomyoma. The results of electrocardiogram, chest X ray, and abdominal ultrasonography for liver and spleen were normal.2.8Before surgery, the dermatological consultant suggested removing the lesions, but did not provide a clear diagnosis. The postoperative pathological diagnosis was NS.2.9The resection of bilateral labia minora lesions was performed under general anesthesia on August 29, 2016. The operation was successfully completed, and intraoperative blood loss was about 10 ml.2.10Pathological examination showed that the right-sided lesion was 2.5 × 1.0 cm in size, while the left was 2.5 × 0.8 cm. The surface of the bilateral labia minora lesions was partially covered with proliferative squamous epithelium and displayed papillary hyperplasia. A large number of proliferative sebaceous glands were found under the skin and deep dermis; they were well-differentiated but lacked hair follicles. Immunohistochemical analysis was positive for P63 in the basal layer, vessels positive for cluster of differentiation (CD) 34 and CD31, and negative for S-100 and Sox; 10% of Ki-67 was positive. Thus, the diagnosis was confirmed as NS  and unusual location” and 6 additional records were found, of which only 1 was ultimately relevant. After reading the above-mentioned articles, we found 2 other related articles,,10 though neither had a detailed description of NS. Herein, we list the information we retrieved and sorted , and Web of Science databases and retrieved only 2 related reports.–10 retrieved by us were diagnosed as NS, and they were all located in the genital area. Kavak et al first reported a case of bilateral labia minora NS, for which the patient went to the Department of Dermatology and Plastic, Reconstructive and Esthetic Surgery. The patient sought help due to slight enlargement of the lesion with occasional pruritus. Lesion removal was planned for aesthetic reasons. After excluding the possibility of neurological or ophthalmologic disease and syphilis, the lesion was partially excised and primary repair was achieved. There was no recurrence or development of other tumors at follow-up (postoperative year 1). Ugras et al published the second paper on lesions of NS in the genital region, but the manuscript did not provide accurate details on tumor location. The vulvar biopsy was initially performed in another institution and reported as squamous hyperplasia, whereas a repeat biopsy performed in the author's institution was suggestive of NS. The surgery was performed consequently and the patient was followed up regularly for 10 months without recurrence. Feito et al recently reported a case of NS in 2018. The patient was a 13-year-old girl with hypertrophy and asymmetry of the left labia, but no pain or abnormal sensation. The lesion was surgically removed, and the patient was discharged 2 days later without further follow-up information.All the cases NS is a benign disease with a detection rate of 0.5% to 1% in all dermatologic patients, but it may lead to benign or malignant secondary tumors. Aslam et al noted in a meta-analysis of 4900 NS cases that secondary tumors developed in around 25% of cases. The most common benign tumor was trichoblastoma, while basal cell carcinoma (BCC) was the most common malignant tumor. Hsu et al published a research report on 450 cases, which pointed out that syringocystadenoma papilliferum was the most common benign tumor, accounting for 2.7% of all cases, trichoblastoma occurred in 1.6%, and BCC in about 0.9% of cases. What is worthy of our attention is that this article listed 8 large studies on NS, dated from 2000 to 2016. By analyzing the data reported by Hsu et al, we found that there were 3847 NS cases in total, and 36 cases of BCC accounting for about 0.94%, consistent with other studies. Kamyab-Hesari et al studied the histopathological features of 168 patients with NS in order to determine the proportion of different histological features and incidence of secondary tumors. The results pointed out 9 cases of neoplastic change, including 4 cases of trichoblastoma, 3 of tricholemmoma, and 2 of syringocystadenoma papilliferum. Neither BCC nor other malignant tumors was found. Hence, Kamyab-Hesari holds the view that unnecessary surgical intervention should be avoided, and recommends regular follow-up for children. A prophylactic excision, however, would need thorough evaluation and individualized therapy. Further, Santibanez-Gallerani et al assessed 658 NS pediatric patients under 16 years of age and found no malignancy, but deemed that the risk of NS-induced tumors increased over time. As a supplement, Cribier study of 596 NS subjects recorded 5 cases of BCC, but only in individuals over 22 years of age. Bernard Cribier et al noted that the incidence of BCC may be overestimated because of the similarity between BCC and trichoblastoma, and proposed that when clinical signs indicating malignant transformation were absent, children should not receive preventive surgery. Hsu research results strongly support that malignant transformation of NS mainly occurs in adulthood. When performing prophylactic excision in children, it is an elective procedure and the risks of general anesthesia and surgical complications should be considered. However, resection is strongly advocated during adolescence due to the significantly increased risks. In our opinion, NS is a congenital hyperplastic disease of sebaceous glands, and its treatment should be surgical removal during puberty. The definitive treatment for NS is full-thickness dermal and epidermal excision, and lesions should be excised with 2 to 3 mm margins since it extends at least as deep as the subcutaneous tissue with involvement of adnexal structures. In cases with incomplete clearance, close follow-up is necessary.As it is unclear whether the surgical resection was appropriate, we studied the relevant literature further. According to reports,HRAS and NRAS is responsible for the occurrence of sebaceous nevus, which can activate mitogen-activated protein kinase and phosphoinositide 3-kinase pathways and stimulate cellular proliferation causing so-called “RASopathy” in NS lesional keratinocytes.,12 Taking into account that benign and malignant secondary tumors of NS often occur in adulthood, Hsu et al and Idriss et al endorsed the idea that age may be a critical risk factor. Furthermore, Person et al were the first to propose the hypothesis that NS was related to hormonal hyperresponsiveness. They speculated that since sebaceous and apocrine glands are androgen-target organs, androgen receptors may increase in these lesions. To prove this hypothesis, they performed androgen receptor assays in 4 specimens of 3 NS cases, and unsurprisingly, the results showed a significant increase in androgen receptors. NS seems to be directly regulated by hormones, and the level of androgen receptors tends to be up-regulated at all ages, thus being particularly hypersensitive to the effects of hematological androgens.As for the pathogenesis of NS, Hsu viewpoint deserves our careful consideration. Mosaic mutation of HRAS and NRAS and is accompanied by an increased expression of androgen receptors. According to previous studies, genital NS lesions are usually located on either unilateral or bilateral labia minora, and can present as a slightly pigmented plaque with verrucous lesions or as a yellowish-tan-colored lobulated growth. All cases of NS in the genital area have been reported in female patients so far, with no other skin malignancies and the age of onset ranging from puberty to perimenopausal period concerning treatment strategies, full-thickness skin resection is an effective method, requiring at least 2 to 3 mm margin and close follow-up postoperatively. Whether for aesthetic reasons or for concerns of malignant transformation, with the consent of the patient, we recommend surgical removal of the genital lesion within the period of adolescence or before adulthood. Adolescence may be the best period for surgical intervention owing to a greater risk of malignant change in adulthood. On the other hand, the risk of surgery can be avoided in children, considering the low incidence of malignant transformation in the pediatric age-group. At present, the reported follow-up duration of patients is around 1 year, while we found no recurrence after 40 months of observation. Thus, we strongly believe that if patients with lesion-free margins have no recurrence after 1 year of follow-up, the possibility of recurrence in the future is lower. In addition, differential diagnosis is also very important, by virtue of the fact that patients may visit different departments under different circumstances. Although the diagnosis can be confirmed mainly by pathological examination, detailed history-taking and medical tests should be done to distinguish NS from neurological diseases, syphilis, and other potential diseases.Briefly, NS in genital or other parts is associated with mosaic mutations of In conclusion, we recommend full-thickness skin resection of NS lesions with at least 2 to 3 mm margin in the genital area during adolescence or before adulthood, and close follow-up postoperatively. Adolescence may be the best period for surgical intervention owing to a greater risk of malignant change in adulthood. On the other hand, surgical risks can be avoided in children considering the low incidence of malignant transformation in the pediatric age group. In addition, we strongly believe that if patients with lesion-free margins have no recurrence after 1 year of follow-up, the possibility of recurrence in the future is lower. Lastly, we hope that our report can provide some important references to other dermatologists, gynecologists, or plastic surgeons, and remind them to attach enough importance to the long-term follow-up of patients with NS.Conceptualization: Zhiwei Zhao, Jiang Wu, Yali Miao.Data curation: Qiao Wen, Yanlin Yang, Fengnian Zhao.Funding acquisition: Jiang Wu, Yali Miao.Investigation: Qiao Wen, Ling Wang, Juan Cheng.Methodology: Zhiwei Zhao, Jiang Wu, Yali Miao.Project administration: Zhiwei Zhao, Jiang Wu, Yali Miao.Writing – original draft: Qiao Wen, Zhiwei Zhao.Writing – review & editing: Jiang Wu, Yali Miao.


Colorectal cancer (CRC) is ranked third worldwide and seventh in Tanzania. The liver and lungs are the most commonly involved sites. Disseminated carcinomatosis of the bone marrow (DCBM) from colorectal carcinoma is rare and typically indicates widespread disease and poor prognosis. We report a case of a 40-year-old African male, who presented to us with abdominal distension, weight loss, fever and change in bowel habit over the past month. He underwent colonoscopy which revealed a necrotic mass in the descending colon. Biopsies were taken, and histopathology confirmed the presence of poorly differentiated mucin-producing adenocarcinoma. The patient suffered a colonic perforation after the fifth cycle of chemotherapy, requiring surgical interventions. Patient's course was complicated by pancytopenia and bone marrow biopsy revealed infiltration by non-hematopoietic malignant cells and bone marrow necrosis. Disseminated carcinomatosis of the bone marrow is a rare and fatal condition; hence high level of clinical suspicion may help in detecting this rare manifestation of colorectal cancer. Colorectal cancer (CRC) is the third frequently diagnosed malignancy and fourth leading cause of cancer-related death worldwide, accounting for about 1.4 million new cases and 700,000 deaths in 2012. The incidence of CRC in Tanzania is 462/100,000 (3.1%) with a mortality rate of 323/100,000 (2.8%) [Death in CRC is attributed to the disease recurrence or distant metastases. Distant metastasis in colorectal cancers occurs in liver and lungs . PeritonWe hereby report a case of patient with colorectal cancer who developed colonic perforation after his fifth cycle of treatment with folinic acid, 5-fluorouracil and oxaliplatin (FOLFOX-4) together with bevacizumab (BV) , and subsequently diagnosed with DCBM by bone marrow biopsy after persistent pancytopenia.A 40-year-old African male presented to our center with the complaint of bloody diarrhea, accompanied with progressive abdominal distension for one month. The colonoscopy revealed luminal narrowing of the descending colon with a necrotic mass at the descending colon which was edematous and bled with minimal contact Figure .The histopathology revealed poorly differentiated mucin-producing adenocarcinoma Figure , 2b, 2c.Abdominal computed tomography (CT) scan revealed liver metastases with peritoneal seedlings. The patient was started on palliative chemotherapy FOLFOX-4 + BV and was followed up on outpatient basis.9. CT examination of the abdomen was performed, which revealed bowel perforation  revealed normocytic, normochromic erythrocytes, leucopenia with no blast cells.The patient's post-surgical course was complicated by pancytopenia, with white blood cells 0.7 x 10Bone marrow biopsy revealed bone marrow infiltration by non-hematopoietic malignant cells and bone marrow necrosis, i.e., disseminated carcinomatosis of bone marrow (DCBM) Figure .The patient’s condition got worse, he went into multi-organ system failure, and expired as an inpatient.This case report represents the first documented case of DCBM of a CRC patient in Tanzania and the first case in an African race.DCBM with DIC is an oncological emergency which unfortunately is very difficult to diagnose when a patient is alive and able to tolerate chemotherapy . Bone maAlthough most reported cases of DCBM secondary to CRC are associated with DIC, this case had no DIC until towards end . In our DCBM should be suspected in patients with CRC with abnormal complete blood count (CBC) results or DIC. Prompt bone marrow biopsy should be performed to establish a definitive diagnosis. Early diagnosis of DCBM is important. Clinicians need to watch out for DIC and counsel the patient and family on the poor prognosis associated with this disease entity.


Work-up included ultrasound and computed tomography, which identified a fat-containing splenic mass 5.6 cm in size. Due to recurrent symptoms, the patient sought medical care again. Subsequent images showed an increase in size to 7.6 cm, which was concerning for neoplasm. This was removed via open splenectomy, which was challenging due to intra-abdominal adhesions despite never having had any abdominal surgery. The patient's recovery was uncomplicated. Pathologic assessment indicated that the mass was a myelolipoma. Extra-adrenal myelolipomas are rare and typically found within the retroperitoneum but are extremely rare within the spleen. This case report adds the 6 The differential diagnosis for fat-containing splenic masses seen on CT scans range from benign neoplasms to malignancies of mesenchymal origin. Myelolipoma is a rare tumor, but most commonly found in the adrenal gland. It is a rare finding in extra-adrenal locations especially within the spleen . When thA 42-year-old male patient presented with irregular bowel movements and abdominal pain, requiring multiple hospital admissions. During these admissions, a splenic mass was identified radiographically. While this was deemed not to be the source of the irregular bowel movements, an ultrasound of his abdomen during one of these admissions 3 years prior to our evaluation revealed a left upper quadrant mass. This was subsequently assessed with computed tomography (CT) scan, which identified a fatty mass potentially arising from the spleen or adrenal gland. The mass was initially found to be 5.6 cm in size but was noted to be growing in size on repeat imaging 2 years later, with a subsequent measurement of 6 cm in size. He was advised to see a surgeon, but due to an upcoming move, he delayed further evaluation and work-up. Once settled, he established himself with a primary care physician and underwent CT scan 1 year later showing a 7.6 × 7.1 × 6.4 cm splenic mass , which wPercutaneous biopsy was not obtained due to concern for hemorrhage, and that as the mass was growing, surgery was recommended regardless of the biopsy findings. He was counselled on the risks and benefits of open splenectomy, as well as alternatives including observation. As the mass was growing in size and due to concern for sarcoma, the patient wished for operative intervention. An open approach was favored due to the overall size of the spleen along with concern for sarcoma. He gave consent to proceed with the operation. Presplenectomy vaccines were administered 2 weeks preoperatively.Splenectomy was performed through a left subcostal incision. There was significant adhesive disease  in the left upper quadrant, but the spleen was amenable to be removed intact and without violation of the mass. The patient had an unremarkable postoperative course and recovered well. Pathology identified the mass to be 8.5 × 8 × 6 cm, well-circumscribed, and with a yellow/tan heterogeneous cut surface . MicroscThe authors present a rare symptomatic case of extra-adrenal myelolipoma in the spleen. Myelolipomas are nonfunctional, benign neoplasms composed of dense adipose and hematopoietic tissue. They are the second most common adrenal incidentaloma. Rarely, myelolipomas can present in extra-adrenal locations such as spleen, kidney, bones, or thorax . The etith decade of life and followed with a surveillance protocol consisting of clinical monitoring for tumor progression [The majority of adrenal myelolipomas are found incidentally during the 6gression . If sympgression . CT is tAs splenic myelolipomas are rare, no consensus exists as to work-up or treatment. Similar to previous case reports –6, our pIn conclusion, this case underscores the importance of maintaining a wide differential diagnosis for splenic masses, including neoplastic and benign causes such as myelolipoma. In addition, while the prognosis of myelolipoma is excellent, surgery remains an appropriate treatment when concern for neoplasm is high.


We demonstrate a case, in which endoscopic ultrasonography‐guided fine‐needle biopsy (EUS‐FNB) was useful for determining the diagnosis of lesions of retroperitoneal fibrosis. In our case, accessing the retroperitoneal lesions by conventional percutaneous biopsy procedures was not feasible due to the difficulty of avoiding the inferior vena cava and ureter. We believe that our case demonstrates a unique approach for performing histological analysis in a challenging case. A 67‐year‐old man presented with back pain. Computed tomography (CT) revealed right hydronephrosis caused by a soft‐tissue mass surrounding the abdominal aorta and bilateral common iliac arteries Fig. . PositroRPF is a rare disease characterized by the presence of a fibroinflammatory tissue, which usually surrounds the abdominal aorta and the iliac arteries and extends into the retroperitoneum to envelop neighboring structures, such as the ureters.


Partial or complete failure of obliteration of the processus vaginalis in the female results in the formation of a potential space known as the canal of Nuck, into which various organs and/or collections can herniate. A 4-month-old female presented with a left labial mass related to herniation of the uterus, ovaries and fallopian tubes through the canal of Nuck. Early diagnosis is important as there is a high risk of ovarian torsion and incarceration. It is seen after 12 weeks of gestation and usually obliterates in early life. This obliteration occurs gradually from a superior to an inferior direction.2 The patency of the processus vaginalis in females leads to the presence of a potential space between the peritoneum, inguinal canal and labia majora, which is known as the canal of Nuck. Its distal part remains patent in males and forms the tunica vaginalis of the testis.The canal of Nuck is a potential space because of patency of the processus vaginalis in females. This was first described by the Dutch anatomist, Anton Nuck, in 1691.3 It also assists in maintaining the normal anteverted and anteflexed position of the uterus.2 Its adult homologues are the round ligament and the ovarian ligament. The gubernaculum plays a key role in testicular descent in males.The gubernaculum is another structure that is closely associated with the processus vaginalis and lies anterior to it in the inguinal canal. In females, it attaches to the midpoint of the uterus and prevents the descent of the ovary into the inguinal canal.3There may be herniation of various organs and/or collections through the canal of Nuck. The contents of the hernial sac may include omental fat, bowel loops, ovary, fluid, fallopian tube and urinary bladder. In extremely rare cases, the uterus can also herniate. It usually presents as a groin swelling or a labial mass, which may or may not be associated with pain.Here, we report a case study of a female infant, who presented with a left labial swelling and was found to have herniation of the uterus and bilateral adnexa into the canal of Nuck.A 4-month-old female infant presented with a left labial swelling that increased in size upon crying. The swelling was gradually increasing in size and was noticed by her parents whilst bathing her. They brought the child to the hospital 2 weeks after they first noticed the swelling. The infant was born by natural vaginal delivery at 34 weeks of gestation and had a birth weight of 1.6 kilograms (kg) with an uneventful post-natal course.On physical examination, a soft lump was seen in the left inguinal region with extension to the left labia majora . The lumShe was examined with a NextGen Logiq E portable ultrasound  with a 12.5 megahertz (MHz) frequency transducer. The sonographic examination revealed a 9.6 millimetre (mm) defect in the left inguinal fascia with herniation of the pelvic organs through it . Its conOn probe compression, a part of the uterus was seen to reduce into the inguinal canal. Doppler ultrasound demonstrated normal blood flow to all herniated organs . A finalThe patient was managed with an elective repair of the hernia. The ultrasound findings were confirmed intraoperatively. The hernia sac contained the uterus, bilateral ovaries and bilateral fallopian tubes. The broad ligament of the uterus was stretched. The herniated organs were mildly congested without any evidence of ischaemia. They were reduced, the hernial sac was ligated and the deep inguinal ring was closed. The contralateral side was explored and was found to be normal. The surgery and the post-operative course were free of complications.A palpable mass in the inguinal region of female infants has a relatively narrow range of differentials, including lymphadenopathy, canal of Nuck hernia, hydrocele of canal of Nuck, lipoma, Bartholin gland cyst, lymphatic malformations, haemangiomas, epidermal cysts and rhabdomyosarcoma.4 However, they are 6–10 times more common in male infants and are predominantly of the indirect variety.4 In females, the hernia through a patent inguinal canal is known as the canal of Nuck hernia. In one study of 22 cases of canal of Nuck hernias, the patient ages ranged from 1 to 137 months, with a mean age of 51 months.5 Prematurity is a well-known risk factor for this condition.6 Other risk factors include lung disease and mechanical ventilation, where herniation is related to increased intra-abdominal pressure.7The incidence of paediatric inguinal hernia ranges from 0.8% to 4.4%.8 Interestingly, all the cases with bilateral herniation have been reported to be on the left side, including our case study. The etiopathogenesis of herniation of the uterus is not clear. Okada et al. suggested multiple hypotheses, including traction induced by broad ligament, weakness of the pelvic suspensory ligaments and crying leading to high intra-abdominal pressures.9Herniation of the uterus through the canal of Nuck is extremely rare. There have been only nine reported cases of herniation of the uterus with only three reported cases of herniation of both adnexa.Although clinical examination can help in narrowing the differential diagnosis, ultrasound is the primary modality of choice for evaluation of these inguinal masses. Ultrasound is easy and non-invasive, without carrying the risks associated with ionising radiation. It can characterise the mass and provides information regarding its size, shape, location, internal contents and vascularity. It can also provide a dynamic evaluation of the mass in real-time. Magnetic Resonance Imaging (MRI) may be used in difficult cases. However, it has inherent challenges because of the need for sedation or anaesthesia in young patients and the long examination time.10 Various approaches have been described in the literature regarding the surgical management of these cases. These include a simple herniorrhaphy (high ligation of the sac followed by tissue repair),9 with or without the closure of the deep inguinal ring.11 Some authors have also recommended the anterior repair of the inguinal canal in these cases.12 The mesh repair is contraindicated in the paediatric hernia repair because of an increased risk of complications.13 There is a high risk of surgical complications in this entity due to the adhesions between the herniated organs and the wall of the hernia sac.11Herniation of the ovary through the canal of Nuck should be diagnosed early, as there is a high risk of ovarian torsion as well as incarceration in these cases, and therefore prompt surgical intervention is necessary.Hernia of the canal of Nuck is an uncommon entity that should be always be considered in a young female presenting with an inguinal or labial mass. Radiologists should be well aware of this condition and ultrasound is the modality of choice for initial investigation. The knowledge of the anatomy and embryology of the canal of Nuck is essential for interpretation and a correct diagnosis. The present case study illustrates a rare variant of this condition containing the uterus and bilateral adnexa. Early diagnosis of this condition is essential for prompt surgical management.


Acute appendicitis is a rare but important complication in children with leukemia. It can be difficult to diagnose, and it has a complicated disease course, especially in patients receiving chemotherapy. Awareness of these complications is critical, particularly in cases where surgical intervention is required. We report a child with T-cell acute lymphoblastic leukemia and chemotherapy-induced neutropenia who presented with acute appendicitis. He was successfully treated with broad-spectrum empiric antibiotics and a delayed laparoscopic appendectomy.  Children with leukemia, especially those treated with chemotherapy, are vulnerable to a variety of infections that result from bone marrow suppression and neutropenia. Intra-abdominal and right lower quadrant (RLQ) infectious complications, in particular, are often sources of severe sepsis and result in an increased mortality rate . Acute aA 10-year-old boy with T-cell acute lymphoblastic leukemia (T-ALL) and chemotherapy-induced neutropenia presented with a two-day history of abdominal pain, vomiting, and fever. The pain, which commenced gradually, was intermittent and dull, and the patient sought relief by lying still. Though initially localized to the periumbilical area, the pain migrated to the RLQ the day after onset, and it was associated with several episodes of vomiting containing no bile or blood. The patient reported feeling feverish with chills and a decreased appetite. He denied a history of sore throat, cough, shortness of breath, diarrhea, or dysuria.The patient was diagnosed two months earlier with T-ALL after presenting with an anterior mediastinal mass. He was treated with chemotherapy according to the Berlin‐Frankfurt‐Munster protocols . The conAfter admission, the patient had a fever, with a maximum recorded temperature of 38.5°C. An examination revealed that the patient was mildly dehydrated and had a blood pressure of 100/70 mmHg and a heart rate of 85 beats/min. Deep palpation during a serial abdominal examination revealed tenderness at the right iliac fossa but no peritoneal signs. The abdomen was not distended, and guarding, rigidity, and rebound tenderness were not evident. Bowel sounds were present. The remainder of the examination was unremarkable.A complete blood count at admission revealed pancytopenia Table , and sevUltrasound revealed a blind-ended non-compressible dilated appendix measuring 7 mm in diameter accompanied by peri-appendicular edema and probe tenderness. These findings were confirmed by CT Figure , establiThe patient was managed conservatively with intravenous antibiotics, proper hydration, and analgesics. Employing empirical coverage for febrile neutropenia, a seven-day course of antibiotics was started that included piperacillin-tazobactam , gentamicin (80 mg three times daily), and metronidazole (330 mg four times daily). Appendectomy was deferred until the bone marrow recovered from chemical suppression. Both filgrastim (300 mcg daily by subcutaneous injection) and platelet transfusion were required to correct pancytopenia. After six days of hospitalization, neutrophil count normalized with an increase in hemoglobin and platelet levels Table .Although the patient did not deteriorate during hospitalization, RLQ abdominal pain with tenderness on physical examination recurred. He underwent a laparoscopic appendectomy the day after neutropenia resolved. An acutely inflamed appendix was removed with no immediate intraoperative complications. The tip of the appendix was adherent to the cecum and lateral abdominal wall. The appendix was not gangrenous nor perforated, and no purulent material was noted. The histopathological examination of the appendix revealed focal mucosal ulcerations and transmural neutrophilic infiltration, but no leukemic cells were observed Figure .The patient was vitally and clinically stable after surgery. On the second postoperative day, he was ambulatory and tolerating food. He did not report fever, vomiting, or abdominal pain. He was discharged on the fourth postoperative day with close follow-up at the pediatric surgery and hematology outpatient clinics.Appendicitis is the most common reason for surgery in the pediatric population, affecting 1% of all children below 15 years of age . HoweverWith hematological malignancies, acute appendicitis can present with atypical signs and vague discrete symptoms such as non-localized abdominal pain, abdominal distention, fever, and diarrhea. A physical examination can include diffuse and RLQ abdominal tenderness without peritoneal signs ,5,7. BasMany times, a diagnosis of acute appendicitis requires imaging studies in neutropenic patients. Abdominal ultrasounds and CT scans are reportedly appropriate in these patients ,5,12. A The management of acute appendicitis in children with leukemia and chemotherapy-induced neutropenia is debatable, and the timing of surgical intervention remains controversial . Some auSeveral authors noted lower rates of complications and mortality with early surgical treatment of acute appendicitis in children with leukemia. Angel et al. reported a series of 14 pediatric patients with leukemia, 13 of whom received appendectomies, and one received conservative treatment. The latter died. Of the surgical patients, nine had uneventful recoveries, three had complications (including infection and intraoperative bleeding), and one died ,3,5. MorHigher rates of infectious perioperative complications are expected in neutropenic patients, and postoperative mortality rates have been reported as high as 41% . Thus, aAcute appendicitis in children with leukemia and chemotherapy-induced neutropenia often presents atypically. Correct and early diagnosis is essential and has a direct impact on treatment. Abdominal ultrasounds and CT scans provide valuable diagnostic support; nevertheless, a histopathological assessment of the appendix can be required.Our observations support the effectiveness of a combined approach in the treatment of appendicitis in these cases, employing broad-spectrum antibiotic therapy and a delayed appendectomy. However, continuous monitoring for signs of deterioration that might indicate the need for an emergency surgical intervention is required. In these patients, the laparoscopic surgical approach seems to limit the risk of postoperative complications and shortens the hospital stay.


Adamantinoma of long bones is a slow-growing, low-grade primary malignant bone tumor. It is a rare entity and accounts for less than 1% of all primary bone tumors, and in most cases, it occurs in the mid-axis of the tibia of adolescents and young adults. In this report, we describe the case of a 53-year-old woman with a diagnosis of adamantinoma of the left tibia who was initially treated surgically in 2004. Two years later, she presented with local relapse, for which she underwent new surgical management. The patient was referred to our practice 16 years after the initial diagnosis, and she presented with recurrence characterized by ipsilateral inguinal lymph node metastasis. The histological findings and immunohistochemistry were compatible with metastatic adamantinoma with high-grade differentiation, which ultimately required surgical salvage management. Adamantinoma is a rare bone neoplasm characterized by benign behavior. Usually, it tends to be cured with the first surgical procedure itself. However, there has been anecdotal evidence of its recurrence, which occurs systematically and, in rare instances, locally -4. RegarWe present the case of a 53-year-old female patient with a history of adamantinoma of the left tibia, which had been managed initially by orthopedic surgery with curative intent in 2004. The patient had presented two years later with local recurrence, and she had undergone wide resection plus allograft implantation at that time. Sixteen years later, the patient was referred to our office with evidence of a new recurrence, documented by the appearance of a new mass in the proximal third of the thigh that extended to the inguinal region, associated with neuropathic pain and Trendelenburg gait due to mass.Physical exam revealed an indurated mass in the left thigh, which was not painful, had been fixed, and with a diameter of 6 x 6 centimeters. There were no other remarkable findings in the physical exam. The MRI revealed superficial adenopathy in the inguinofemoral region in contact with the muscular fascia and greater saphenous vein, suggestive of secondary compromise as per the first known image. A node biopsy was performed, which revealed an immunoprofile that favored the presence of metastatic adamantinoma. The immunochemistry findings were as follows: Enolase +, CD99 +, BCL2 +, P63 +, CKAE1/AE3 focal +, FLI1 +, Synaptophysin -, S100 -, Desmine (-), EMA (-). Abdominal and thorax CT were performed, which revealed the absence of any secondary lesions. There was evidence of local compromise, and the patient was scheduled for a salvage procedure, with inguinofemoral lymph node dissection. The chirurgical procedure revealed the presence of spindle cell sarcoma of grade 2-3, with extension to soft tissue and extranodal, coupled with perineural invasion. Mitosis count was as follows: 12 mitosis per camp, no necrosis. Three additional nodes were free of tumor, the skin was free of tumor, and the section edge was also free of tumor. Immunohistochemistry revealed P63 +, p40 +, vimentin +, CK5/6 +, D240 +, EMA focal +, CKAE1AE3 focal +, CAM5.2 focal +.Due to limited evidence regarding the benefit of chemotherapy or radiation therapy in the adjuvant or “pseudoadjuvant” scenario, and with these procedures thought to be beneficial only in patients with systemical compromise, it was decided to continue with a follow-up strategy for our patient. The patient has remained disease-free for the past six months and has not shown any clinical signs of relapse.Adamantinoma is a rare primary malignant tumor of the bone that predominantly affects the tibia of young adults, with an incidence rate of less than 1% ,6. It maThe etiology of these tumors is still a matter of debate, but the most widely accepted hypothesis postulates that the displacement of the basal epithelium during the embryologic development, when the bone forms the endochondral surface, generates the precursor cells that give rise to this class of tumor . It has Surgical management is the preferred treatment modality with en bloc resection with wide margins and limb reconstruction, and it may occasionally lead to amputation based on the extent of bone involvement and the response to initial management. The tumor is highly resistant to radiation, and chemotherapy has not been shown to be effective either .Some studies in the literature have reported that local relapse occurs in 30-35% of cases, with a rate of mortality of 6-18%, and pulmonary metastases or involvement of the lymph nodes in 12-29% of cases [It has been reported that isolated local recurrence and recurrence in pulmonary parenchyma can occur 13-25 years after the initial diagnosis ,10. In tThere have been scarce reports of local relapse with posterior lymph node compromise, and it is this paucity of such cases that prompted us to discuss this particular case. The common risk factors for local relapses and metastases include the male gender, initial management that is limited to biopsy, positive margins, and narrow margins, with the last one being the main possible cause of the local relapse in the present study ,6,11-13.In the following table, we present a comparison of the case reports about metastatic adamantinoma; it includes the type of evolution, patient age, histopathological and immunohistochemical classification, as well as the details of the dedifferentiation characteristics noted in our current case.Adamantinoma is associated with a good prognosis and high overall survival rates; local and distant metastases are rarely reported, which are probably caused by initial surgical management. Surgery is the preferred modality for managing this malignant tumor. A few studies have reported frequent relapses in some cases, and hence it is important to conduct long-term follow-ups for adamantinoma patients.


Intradural spinal epidermoid cysts are rare, benign lesions either acquired from trauma, surgery, lumbar puncture or arise as congenital lesions, particularly associated with spinal dysraphism. Epidermoid cyst arising from the spine with expansile destruction of vertebrae has not been reported yet in the literature. We report a case of 36-years male presented with history of fall 8 years back with progressive symptoms of lower back pain, weakness of left lower limb and bladder/bowel incontinence. Computed tomography revealed large lytic expansile, midline sacral vertebral lesion with soft tissue component and multiple calcific foci. Magnetic resonance imaging demonstrated large non-enhancing heterogeneous mass showing restricted diffusion on diffusion weighted images. The patient underwent biopsy confirming the diagnosis of an epidermoid cyst. The possibility of an epidermoid tumor should be kept in the differential diagnosis in patients presenting with posttraumatic sacral mass. In the spine, the common locations of epidermoid tumors are intradural spinal canal and prevertebral. Intradural spinal epidermoid tumors are rare, comprising less than 1% of tumors involving the spine.A 36-years male, presented with low back pain and leftsided sciatica. Patient had experience occasional mild low back pain for several years which was markedly worsened in the last 4 months. He also had progressive urinary urgency and bowel incontinence for same duration.On clinical examination, the patient had 2/5 power on left foot dorsiflexion and plantar flexion, 1/5 power in left hip flexion and knee extension, while no weakness was seen in the right lower limb. The sensation was grossly intact and his reflexes were normal without hyperreflexia.CT of the lumbosacral region showed lytic expansile midline lesion causing destruction of sacrumwithlarge presacral component, bilateral neural foraminal and sacral canal components . Based oSubsequent magnetic resonance imaging (MRI) demonstrated heterogeneous non-enhancing mass involving presacral region, sacral vertebra and adjacent spinal canal . DiffusiIntradural spinal epidermoid cysts are rare, benign lesions that are either acquired from trauma, surgery, lumbar puncture or arise as congenital lesions, particularly associated with spinal dysraphism. Since the clinical symptoms of spinal epidermoid tumors are variable and non-specific with slow progression, the diagnosis may be delayed. Spinal epidermoid tumors are difficult to diagnose on clinical symptoms alone. Plain radiographs usually show normal or non-specific findings, especially in acquired spinal epidermoid tumors.4 The etiologies of spinal epidermoid tumors are thought to be both congenital and acquired. Acquired epidermoid were mostly reported in the region of the cauda equina because lumbar punctures are usually performed around the level of the iliac crests.6 To the authors' best knowledge, there has never been any report describing acquired spinal epidermoid tumors involving the vertebral body after trauma and that too with a large presacral component. In our case, skin epithelium might have got implanted in the sacrum and pre-sacral region, secondary to trauma. Surgical treatment is required when the patients develop neurological symptoms. Complete excision of epidermoid is the surgical treatment of choice. However, complete resection of the encapsulated tumor is difficult in all cases, because the epidermoid capsules are very thin and the tumor often adheres to the arachnoid membrane, spinal cord, or nerve roots. Thereby, subtotal resection is commonly performed.10A specific histologic feature of an epidermoid cyst is a layer of stratified squamous keratinizing epithelium surrounded by an outer layer of collagenous tissue, with the absence of skin adnexa. The desquamation of keratin from the epithelial lining creates numerous cholesterol crystals.Epidermoid cysts have specific imaging findings, especially in MRI that can help in the diagnosis. The lesions follow fluid-like signals in T1 and T2 weighted images. Lesions show diffusion restriction in diffusionweighted images, as in our case, which is commonly due to the orientation of the keratin layers which restrict the free movement of water. They show minimal to no enhancement, especially the diffusion restricting regions, contrasting with malignant tumors.JNMA Case Report Consent Form was signed by the patient and the original article is attached with the patient's chart.None.