T1 weighted images within the right sphenoidal sinus; a new CT examination revealed calcifications within the mass. Surgical histological examination showed fungus ball. Fungal ball should be included in the differential diagnosis of T1 hyperintense lesions in the sphenoid sinus.Isolated sphenoid sinus fungus ball is a very rare condition. CT is the most used imaging investigation for diagnosis. In some cases, MRI may provide further information to evaluate the extracompartmental invasion. We report the case of an elderly female patient who presented with headache and a soft tissue mass filling the right sphenoid sinus on CT, misdiagnosed as simple sinusitis. After 1 year, with recrudescence of symptoms, brain MRI showed a hyperintense soft tissue mass on  A 62-year-old female patient was admitted in April 2016 in another hospital complaining of severe persistent deep throbbing headache in the right retro-orbital region, not responsive to common non-steroidalanti-inflammatory drugs. Neurological examination did not show any signs of focal deficit. Ophthalmic examination of fundus oculi revealed normally appearing retina with normal eye pressure. In that occasion, the patient underwent brain CT, which showed no significant alterations of brain tissue; instead, obliteration of right sphenoidal sinus was observed and it was referred to sinusitis. After 1 month of oral antibiotic and corticosteroid treatments, symptoms partially reversed, with only a latent headache persisting during the follow-up period; however, throbbing headache recrudescence and posterior nasal drip occurred later in March 2017, when the patient came to our attention.T1, on transversal and sagittal planes, TR 450 ms, TE 8.90 ms, FA 90, thickness 5 mm, gap 6.50; BLADE T2, on transversal and coronal planes, TR 3800, TE 99, FA 141, thickness 5 mm, gap 6.5 mm; T2 FLAIR, TR 8500, TE 82, FA 150, thickness 5 mm, gap 6.5 mm; T2* GRE TR 830, TE 25, FA 20, thickness 5 mm, gap 6.5 mm, diffusion-weighted imaging, TR 7200, TE 81, FA 90, thickness 5 mm, gap 6.5 mm, b values 0 and 1000; post-contrast T1-MPRAGE TR 1900, TE 3.02, FA 15, thickness 1 mm, gap 0 mm. All the sequences were acquired using the same Matrix (320\320) and 1 NEX, except for diffusion-weighted imaging, in which 2 NEX was used. The MRI evaluation of the brain found only incidental chronic hypoxic lesions. The right sphenoid sinus showed irregular and hypertrophic T2 prolonged hyperintensity of the mucosa with central hypointensity (T1 weighted images (T2* blooming artefacts on MRI) and sclerotic bone wall without continuity into the cortical bone with the following MRI parameters applied: SE ntensity ; this contensity , and hypd images . After cd images . The lefical bone. The othT1 weighted hyperintensity has not been well described in the literature. Owing to this peculiar appearance, the differential diagnosis covered the following: mucocele, which with its protein content commonly demonstrates elevated signal intensity on T1 weighted images but also appears hyperintense on T2 weighted images and does not show enhancement; inverted papilloma, but it usually involves the nasal cavity (mostly the middle meatus) and secondarily affects sinuses; sinonasal mucosal melanoma, whose T1 hyperintensity is due to haemoglobin derivatives from previous bleeding, but it shows contrast enhancement and generally arises from the nasal cavities; mature nasal osteoma, for the presence of central fatty marrow that is hyperintense on T1 weighted images; FB.2 Our case was previously diagnosed as simple sphenoid sinusitis only on the basis of CT examination; on the contrary, our MRI examination revealed in the right sphenoid sinus a hyperintense hypertrophic mucosa on T2 weighted images, and a central hypointense signal alteration; for this reason, we excluded inverted papilloma and mucocele. The T1 weighted sequence documented a slight hyperintense signal intensity soft tissue mass within the sinus: this finding may be explained by the presence of tiny calcification in the tissue matrix.2 The mass did not show CE on fat suppressed T1 weighted images and, for this reason, we excluded melanoma; on the other hand, the sinus mucosa was enhancing. On T2* images blooming artefacts were noticed in the central part of the lesion and they were compatible with the gross calcifications detected on the CT scans; consequently, mature osteoma was not taken into account. No further significant pathological alterations were found in the other paranasal sinuses. The final diagnosis was that of isolated right sphenoid FB. Treatment is surgical, using two endoscopic approaches: transethmoidal and transnasal. The latter is considered easier and faster with less complications. A good preoperative assessment is mandatory because if a dehiscence of lateral sinus wall is noticed on radiological examinations, fungal hyphae may spread to the surrounding neurovascular structures during the surgical intervention, causing life-threatening complications. In order to minimize this possibility, medical treatment should be started about 4 weeks before surgery.4 Endoscopic surgical removal of the fungal mass with re-establishing sinus drainage and irrigation to clear fungal debris is the goal of management. Recurrence is rare; however, it can be seen as late as 2 years.4 After surgery, our patient experienced a complete remission of symptoms.As far as we know, FB with a central The patient underwent transnasal approach endoscopic surgery with mucosal curettage and establishing of a drainage opening. Histopathological examination confirmed the diagnosis of right sphenoidal sinus FB caused by Aspergillus fumigatus . Postope6 FRS is distinguished into two categories: invasive, which usually affects immunosuppressed patients and is characterized by the infiltration of the mucosa and spreading to the surrounding structures; non-invasive FRS, typically affecting immunocompetent patients, with no evidence of tissue invasion.7 Paranasal sinus FB was first described in the 18th century and defined as extramucosal accumulation of dense conglomeration of fungal hyphae in a solitary sinus cavity.8 FB usually affects elderly immunocompetent patients (mean age of 64 years), with a prevalence in the female sex.9,10 Several causes have been implied in its pathogenesis. The main pathological agents that sustain the infection are Aspergillus spp., but others pathogens have been also detected .5 Many misleading terms are found describing this condition such as mycetoma, aspergilloma and aspergillosis. Mycetoma is defined as chronic local invasive infection of the subcutaneous tissue that extends to adjacent structures, mostly seen in the hands and feet; with regard to aspergillosis and aspergilloma, Aspergillus species is responsible for many cases of invasive and allergic fungal sinusitis. In addition, in some cases of FB the culture is negative for Aspergillus and other fungal species, so these terms should be avoided and FB should be used instead.10 FB is found in about 3.7% of operated chronic inflammatory conditions affecting all paranasal sinuses. It is not a contagious disease and more commonly affects maxillary sinus— 83 to 88%—.11 The maxillary sinus colonization may be due to odontogenic origin, as a consequence of dental procedures, but it would not explain the sphenoid localization and the occurrence of FB in patient with no previous dental interventions;12,13 another theory, the so-called “aerogenic” theory, postulates that the paranasal sinuses may be infested by spores inhalation. It is possible that other predisposing factors are implied in FB development, such as host immunological state, concomitant pathologies (diabetes), oestrogens and anatomical variants.10,12 Isolated sphenoidal sinus FB is a relatively rare entity that is more frequent in elderly female patients and may present with vague symptoms.6 Until 1992, only 21 cases of isolated sphenoid sinus FB were reported and this might have been due to its vague presentation and lack of advanced imaging tools. Nowadays, the incidence is increasing.3 Owing to its clinical presentation, the diagnosis of FB is usually delayed in time; in fact, it has been noticed that only 29% of cases of paranasal FB are diagnosed within 1 year of onset of symptoms and even much later in cases of isolated sphenoid sinus FB;13 presence of refractory chronic sinusitis symptoms should then raise the suspicion for fungal infection.14 Blood tests are usually not contributory because the patients generally have no history of diabetes, immunosuppressive conditions or atopy.10 Clinically isolated sphenoid FB mostly manifests with headache, which radiates to the frontal, retro-orbital or occipital region in the case of sphenoid FB;11 other reported symptoms are those of chronic nasal inflammation  that does not improve with conventional antibiotic therapies.5,12 Radiologically, CT and MRI examinations are the most suitable imaging modalities for its diagnosis as well as for pre- and postoperative evaluation, while conventional X-rays give non-specific findings.15 On CT scan FB typically appears as a hyperdense mass  usually limited to a single sinus, with linear or punctuate central calcifications. Thickened and sclerotic bony sinus walls with bowing deformity are also common without any signs of invasiveness. The thickened inflamed mucosa may enhance after iodine contrast administration.8 On MRI, FB usually presents intermediate/low signal intensity on T1 weighted images and frank low signal intensity on T2 weighted images; blooming artefacts, due to the presence of calcifications, could be appreciated on T2* images. While the inflammatory mucosa appears hyperintense on T2 weighted images and may enhance as well, this latter aspect is best appreciated using T1 weighted images with fat suppression.13,15Fungal rhinosinusitis (FRS) is one of the most common pathologies that affects paranasal spaces, and radiological imaging plays an important role in its identification.6 Generally, functional endoscopic sinus surgery is the modality of choice for management with no further medical treatment required. In our opinion, on the basis of previous considerations, this pathology may be underdiagnosed on routine evaluation and sometimes wrongly interpreted as simple sinusitis, with possible life-threatening complications. In our case, the diagnosis was also validated by the singular sign of unusual T1 hyperintensity signal of the fungal mass.FB should be suspected in refractory sinus diseases or in long-standing vague symptoms in older individuals. CT scan is the modality of choice for diagnosis, and MRI should be done if bone involvement is suspected; however, there are no pathognomonic imaging features and this is why histopathological confirmation should be done postoperatively.FB is a very common disease usually correlated to Aspergillus thickness infections, which mostly involve the maxillary sinus; sphenoid sinus colonization is an uncommon event. Symptoms may be very vague and the diagnosis can be delayed, leading to complications such as venous thrombosis and visual disturbances.T1 weighted image, but the presence of tiny calcifications within the lesion may confer hyperintense signal.On CT, FB appears as a hyperdense mass filling a sinus cavity with thickening and sclerosis of sinus walls; on MRI, it usually appears as a non-enhancing hypointense mass on Surgical endoscopy is the treatment of choice, with expected complete remission of symptoms .Written informed consent for the case to be published  was obtained from the patient(s) for publication of this case report, including accompanying images.


Breast pilonidal sinus is an extremely rare variant of the condition, the report aims to present a case of pilonidal sinus occurring in the breast.A 35-year-old female presented with a left breast discharging lesion for a 5-month duration. There was a local redness with multiple discharging sinuses on her left breast at 3–5 o’clock, 3–4 cm away from the nipple. The discharge was serosanguinous in appearance. The lesion was excised totally with primary closure under general anesthesia. Histopathological examination was consistent with pilonidal sinus.For atypical pilonidal sinus disease, surgery is the modality of choice but regarding sacrococcygeal pilonidal sinus, non-operative treatment begins to grow. The cardinal risk factors for recurrence and complications are male gender, family history, tobacco smoking, obesity, size of sinus, poor personal hygiene and surgical methods.Breast pilonidal sinus is an extremely rare variant of the condition. It should be suspected on clinical examination. Surgical excision is the definitive treatment strategy. It is an inflammatory condition resulting from skin penetration by a hair. The tract of the sinus is lined by granulation tissue which terminates in a pus-filled chamber . The typBreast PNS (bPNS) is an extremely rare variant of the condition with only three reported cases in the literature ,12,13. T1.1A 35-year-old housewife presented with a discharging lesion on her left breast for 5 months, not responding to medical treatment with a history of remission and relapse. She was nonsmoker, multiparous. Past-history was unremarkable.1.2There was a local redness with multiple discharging sinuses on her left breast at 3–5 o’clock, 3–4 cm away from the nipple . The dis1.3The lesion was excised totally with primary closure under general anesthesia. Histopathological examination showed invaginated epidermis forming a tract extending from the subcutaneous tissue to the skin. There was a hair shaft surrounded by granulation, consistent with a pilonidal sinus .Fig. 2Ha1.4Postoperatively she was followed up for six months, the wound healed completely without complications and there was no evidence for recurrence.2Before puberty and after the age of forty, PNS is uncommon. The condition demonstrates male dominance with a ratio of 3:1 .Young age, deep navel and cleft, hairiness, male sex, long term sitting and poor personal hygiene are among the risk factors . In the bPNS is a very rare variant of PNS ,12,13. KLahiri and associates presented a case of bPNS occurred in a 28-year-old female. She presented with a 4-week history of left breast collection, the condition was provisionally diagnosed as an abscess complicating periductal mastitis, surgical excision was done and microscopic examination of the specimen revealed features consistent with bPNS .Huges et al. reported their experience with a 38-year-old male patient who presented with a right breast mass for a 20-year duration with intermittent discharge from the lesion. After a thorough clinical examination and ultrasound study, they excised the lesion, histopathological examination demonstrated peri-areolar PNS disease .In composite, bPNS is an extremely rare variant of the condition. It should be suspected on clinical examination. Surgical excision is the definitive treatment strategy.No source to be stated.Approval has been taken from Kscien centre.Consent has been taken from the patient and the family of the patient.Abdulwahid M. Salih: Surgeon performed the operation and follow up.Shvan H. Mohammed, Fahmi H. Kakamad, and Rawezh Q. Salih: Writing the manuscript and follow up.Zuhair D. Hammood, Hiwa O. Baba, and Imad J. Habibullah: literature review, final approval of the manuscript.Not applicable.Fahmi Hussein kakamad.Not commissioned, externally peer-reviewed.There is no conflict to be declared.


The latest (4th) edition of the World Health Organization (WHO) Classification of Head and Neck Tumours, published in January 2017, has reclassified keratocystic odontogenic tumour as odontogenic keratocyst. Therefore, odontogenic keratocysts (OKCs) are now considered benign cysts of odontogenic origin that account for about 10% of all odontogenic cysts. OKCs arise from the dental lamina and are characterised by a cystic space containing desquamated keratin with a uniform lining of parakeratinised squamous epithelium. The reported age distribution of OKCs is considerably wide, with a peak of incidence in the third decade of life and a slight male predominance. OKCs originate in tooth-bearing regions and the mandible is more often affected than the maxilla. In the mandible, the most common location is the posterior sextant, the angle or the ramus. Conversely, the anterior sextant and the third molar region are the most common sites of origin in the maxilla. OKCs are characterised by an aggressive behaviour with a relatively high recurrence rate, particularly when OKCs are associated with syndromes. Multiple OKCs are typically associated with the nevoid basal cell carcinoma syndrome (NBCCS), an autosomal dominant multisystemic disease. Radiological imaging, mainly computed tomography (CT) and, in selected cases, magnetic resonance imaging (MRI), plays an important role in the diagnosis and management of OKCs. Therefore, the main purpose of this pictorial review is to present the imaging appearance of OKCs underlining the specific findings of different imaging modalities and to provide key radiologic features helping the differential diagnoses from other cystic and neoplastic lesions of odontogenic origin.Panoramic radiography is helpful in the preliminary assessment of OKCs.• CT is considered the tool of choice in the evaluation of OKCs.• MRI with DWI or DKI can help differentiate OKCs from other odontogenic lesions.• Ameloblastoma, dentigerous and radicular cysts should be considered in the differential diagnosis.• The presence of multiple OKCs is one of the major criteria for the diagnosis of NBCCS.•  Odontogenic keratocysts (OKCs), first described by Philipsen in 1956 . HistoloBecause of this histologic feature, the aggressive behaviour and the fact that a large proportion of lesions are associated with a mutation or inactivation of the tumour suppressor gene, also called the protein patched homolog (PTCH) gene, in the 3rd edition of the World Health Organization (WHO) Classification of Head and Neck Tumours, this pathological entity was included in the group of odontogenic neoplasms with the name of keratocystic odontogenic tumour (KCOT) .In the latest (4th) edition of the WHO Classification of Head and Neck Tumours published in January 2017 , the conPreoperative assessment is important for planning treatment and management, as OKCs require a more aggressive treatment than other low-attenuating lesions having similar radiological appearance.The aim of this pictorial review is to present the imaging appearance of OKCs underlining the specific findings of different imaging modalities and to provide key radiologic features helping the differential diagnoses from other cystic and neoplastic lesions of odontogenic origin.OKCs represent approximately 10% of odontogenic cysts and the reported age distribution is considerably wide (from 8 to 82 years), with a peak of incidence in the third decade of life , 8, 9. MThe presence of multiple OKCs, also occurring in different moments during the lifetime of the patients, is typically associated with the nevoid basal cell carcinoma syndrome (NBCCS), also known as Gorlin–Goltz syndrome, an autosomal dominant multisystemic disease. In these patients, the mean age of incidence decreases to about 25 years old –13.Similarly to other entities having an odontogenic origin, OKCs originate in tooth-bearing regions. They occur twice as often in the mandible as in the maxilla . When OKThe reported recurrent rate of OKCs after surgery is wide, up to 30%, with most recurrences occurring after conservative treatments of simple lesion’s enucleation , 19, 23.Higher recurrence rates are reported in patients affected by NBCCS and in multilocular lesions , 25. TheThe radiological imaging techniques most commonly used in the study of OKCs are conventional radiography (mainly panoramic radiography), computed tomography (CT) and magnetic resonance imaging (MRI). These imaging modalities differ significantly in their technical characteristics, acquisition modalities, indications and information provided.Panoramic radiography is a flat representation of the curved surfaces of the maxillary and mandibular dental arches and is helpful in the preliminary assessment of the location, size, shape, margins and extension of odontogenic lesions, such as OKCs. However, this radiographic technique has a limited role because it provides a two-dimensional view of maxillofacial structures with magnification, geometric distortion and overlapping. Therefore, to overcome the limitations of panoramic radiography, a three-dimensional imaging modality is often required for preoperative planning, particularly in larger lesions.Radiographically, OKCs appear as a well-defined unilocular or multilocular radiolucency bounded by corticated margins Fig. . UnilocuApproximately 30% of OKCs are associated with at least one unerupted tooth, most commonly the third molars Fig. a 9, 29], 299, 29The radiographic features of OKCs are not pathognomonic, particularly in smaller unilocular lesions . When a Large mandibular OKCs tend to grow predominantly along the length of the bone with minimal bucco-lingual expansion, especially within the body . On panoRadiographically, OKCs may show tooth displacement and root resorption; this latter finding is an uncommon radiographic feature of OKCs, with a reported incidence varying from 1.3 to 11% . The litIn clinical routine, there are two main CT techniques commonly used for the evaluation of maxillofacial diseases: cone beam CT (CBCT) and multidetector CT (MDCT). Both CT modalities are usually considered adequate for diagnosing OKCs and preoperative planning, owing to their ability to generate high-quality multiplanar reconstruction (MPR) images in different planes. In addition, using a dedicated reconstruction software for dental arches (DentaScan), the three-dimensional dataset produced by both modalities can be further processed in MPR images that are either parallel (panoramic) or perpendicular  to the curvature of the alveolar bones. These high-resolution MPR images allow three-dimensional views of the jaws and provide detailed information about the OKC and its relationship with surrounding structures .The main advantage that makes CBCT a particularly attractive technique in the evaluation of maxillary and mandibular lesions is its higher spatial resolution compared with MDCT. Conversely, the main disadvantage of CBCT is the poor contrast resolution, which is not suitable for soft tissue contrast discrimination. Hence, CBCT is not able to evaluate the extension into soft tissues and precludes the possibility of contrast medium injection . In the As with panoramic radiography, CT is able to display the main radiological features of an OKC, such as size, shape , margins (well-defined and corticated), internal appearance (uni- or multilocular) and effects on adjacent structures  . In addiTherefore, CT is considered superior to conventional radiography in differentiating OKCs from other unilocular or multilocular osteolytic lesions and in the preoperative assessment Fig. .In the mandible, the OKCs have a tendency to grow predominantly mesiodistally along the length of the bone, causing minimal expansion of the buccal and lingual cortical plates Fig.  33]. Ho. Ho33]. In contrast, large OKCs in the maxilla more frequently present a hydraulic expansion of the alveolar bone with remodelling, thinning, scalloping and perforation of the cortices Fig.  32]. In. In32]. The difference between the growth pattern of mandibular and maxillary OKCs may be partly due to the higher cortical thickness of the mandible compared to that of the maxilla . On CT iOKCs may be associate with an impact tooth Fig. ; this fiInternal high-density areas are frequently found and reflect the presence of keratinised material within the OKC Fig.  14]. Th. Th14]. In the evaluation of cystic lesions of the jaws, MRI is mainly performed as a complementary technique to CT (CBCT or MDCT), and it may be useful in selected cases to provide a better demonstration of the internal features and soft tissue involvement Figs.  and 15.FOKCs typically show various signal intensity on MRI images, which reflect the materials contained inside the lesions. They are represented by a large amount of keratin sometimes associated with hyaline bodies in the presence of inflammation .Various authors reported that most of the OKCs present intermediate or high signal intensity on T1-weighted sequences and heterogeneous signal intensity (from low to high) on T2-weighted sequences Fig.  20, 32,, 35. SomMRI with diffusion-weighted imaging (DWI) and calculation of apparent diffusion coefficient (ADC) is sensitive to physiological parameters such as tissue cellularity, nucleus-to-cytoplasm ratio and integrity of cell membranes, thus providing information about the microstructure of living tissues . DWI may− 3 mm2 s− 1 and the optimum cut-off for the differentiation with predominantly cystic ameloblastomas was 2.013 × 10− 3 mm2 s− 1 [In particular, as first demonstrated by Sumi et al. , the ADCmm2 s− 1 . These fmm2 s− 1 . On the mm2 s− 1 .Sakamoto et al., in a retrospective study including 35 odontogenic cystic lesions, showed that diffusion kurtosis imaging (DKI) could represent a quantitative evaluation tool for better differentiating OKCs from other cystic lesions . Indeed,Radiological imaging, mainly CT (CBCT or MDCT) and, in selected cases, MRI, plays an important role in the diagnosis of OKCs. However, OKCs, in particular smaller lesions, may exhibit imaging features almost indistinguishable to other osteolytic jaw lesions. Hence, in order to obtain a definitive diagnosis, a histopathological examination is required . From thIt is reported that, in some OKCs, the combination of clinical and radiological findings allows narrowing the differential diagnosis and, in some cases, making the correct diagnosis .Well-defined unilocular osteolytic lesion in the posterior region of jaws Fig. Large osteolytic mandibular lesion with few septa and minimal buccolingual expansion Fig. The imaging findings which are more effective for making a provisional diagnosis of OKC are:However, when an OKC is associated with an impacted tooth, it may simulate a dentigerous cyst. Similarly, when an OKC is multilocular and located in the posterior sextant or the ramus of the mandible, it may mimic an ameloblastoma. Finally, when an OKC has a periapical position or involves an edentulous area, it may be mistaken for a radicular cyst. As a result, dentigerous cyst, ameloblastoma and radicular cyst are considered the most common odontogenic lesions in the differential diagnosis of an OKC .Unilocular osteolytic lesion around the crown of impacted toothNo septa or loculation within the cystMore buccolingual expansion in mandibleMore homogeneous and high T2-weighted signal on MRIThe imaging features which are more effective for suggesting a diagnosis of dentigerous cyst rather than of OKC are , 32:UnilMultilocular osteolytic lesion with multiple internal septaMore buccolingual expansion in mandibleMore prominent tooth displacement and root resorptionPost-contrast enhancement of septa and mural nodule (more easily detectable on MRI rather than on MDCT)− 3 mm2 s− 1 on DWI [Mean ADC value higher than 2.013 × 101 on DWI The imaging features which are more effective for suggesting a diagnosis of ameloblastoma rather than of OKC are , 32:MultRound or pear-shaped unilocular osteolytic lesionEpicentre at the apex of a non-vital toothIron-like density within the cyst (indicator of endodontic overfilling)Finally, the imaging features which are more effective for suggesting a diagnosis of radicular cyst rather than of OKC are , 32, 33:The typical features of OKCs, dentigerous cysts, radicular cysts and ameloblastomas are summarised in Table The presence of multiple OKCs is considered one of the major criteria for the diagnosis of NBCCS, and their occurrence may be the first sign of the disease . NBCCS, Other features associated with NBCCS include craniofacial, neurological, sexual, ophthalmic and cardiac anomalies . The litMultiple OKCs are also observed in other syndromes, such as Noonan syndrome, Ehlers–Danlos syndrome and oral-facial-digital syndrome.In syndromic OKCs, the cysts occur at an early age (first or second decades of life), originate more often in the posterior sextants of the maxilla Fig. , have moIn rare case, multiple OKCs can be observed without any evidence of systemic disease .However, it should be noted that the occurrence of multiple OKCs should indicate, until proven otherwise, the presence of a syndrome, and a patient with multiple OKCs should be followed regularly to assess the possible appearance of any other systemic manifestations.The management of OKCs aims to reduce the risk of recurrence while minimising, at the same time, the morbidity for the patient. At the present moment, there is no consensus about the best treatment modality.Different factors take part in the choice of the more appropriate treatment, including size and location of the lesion, unilocularity or multilocularity, presence of cortical perforation or soft tissue involvement and the patient’s age.Various surgical options have been considered, including enucleation alone or associated with adjunctive measures , marsupialisation and decompression, marginal or segmental resection .In a systematic review of the literature, Johnson et al. showed that enucleation is associated with the highest recurrence rate of about 30%, followed by marsupialisation alone (approximately 18% recurrence rate). The association of lesion’s enucleation with adjunctive technique of chemical cauterisation with Carnoy’s solution, a mixture of chloroform, absolute ethanol, glacial acetic acid and ferric chloride, significantly reduced the recurrence rates to about 8% .Surgical resection, both marginal and segmental, is related to the lowest recurrence rate but, because of its morbidity, is not recommended as a primary treatment modality and should be reserved for retreatment of patients suffering from multiple recurring lesions .According to the literature, most recurrences of OKC occur within the first 5–7 years after treatment .In a paper by Apajalahti et al., the mean recurrence time was relatively shorter (about 2 years). This can be explained by the systematic use of CT in the follow-up of large OKCs Fig. , thus heFor this reason, periodic radiographic monitoring of patients with surgically treated OKCs is recommended annually for the first 5 years and at least every 2 or 3 years subsequently . PatientOdontogenic keratocysts (OKCs) are benign lesions of odontogenic origin accounting for about 10% of all odontogenic cysts and characterised by an aggressive behaviour.Radiological imaging, mainly computed tomography (CT) and, in selected cases, magnetic resonance imaging (MRI), plays an important role in the diagnosis and management of OKCs. Although radiological imaging does not always provide a specific diagnosis, the knowledge about typical and atypical radiological features of OKCs is essential for their diagnosis and treatment planning. In particular, the combination of clinical and radiological findings is useful in evaluating the extent of the lesions and the relationships with adjacent structures.The relatively high recurrence rate, especially after conservative surgery, make it necessary to perform a periodic radiographic monitoring of patients with surgically treated OKCs, at least for the first 5 years.


Complete surgical resection of chronic expanding intrapericardial hematoma was often difficult because of the severe adhesion. Preoperative coil embolization of the feeding vessels can prevent recurrent expanding of the residual hematoma and would achieve good results. He had a history of mitral valve plasty and left atrial maze procedure 8 years ago. Three years after the surgery, follow‐up transthoracic echocardiography (TTE) and chest computed tomography (CT) demonstrated a space‐occupying lesion close to posterolateral wall of the heart then left ventricular (LV) diastolic function decreased. Despite medication, LV diastolic function gradually worsened and resulted in pulmonary hypertension and functional tricuspid regurgitation (TR); therefore, a resection of the hematoma was performed via a left thoracotomy. Histopathological diagnosis was intrapericardial hematoma. A complete resection was not feasible, hence, 2 years after the procedure, follow‐up TTE and chest CT demonstrated relapsing intrapericardial hematoma in situ, and furthermore, the hematoma has gradually expanded Figure . The LV Postoperative TTE demonstrated recovery of LV cardiac function, and the LV wall compression was disappeared. Contrast‐enhanced chest CT and angiography showed reduction in space‐occupying lesion and no residual feeding vessels. Postoperative course was uneventful, and he discharged on day 26 after operation.3Chronic expanding hematoma was first reported by Reid et al,Recently, the utility of preoperative coil embolization for the feeding vessels to the chronic expanding hematoma has been reported.In our case, the capsule of the hematoma was formed by the epicardium and severely adhered to the myocardium; therefore, a complete resection of the pericardium was impracticable. Moreover, neovascularization and blood supply from several feeding vessels might induce the gradual expansion of the capsule; hence, we thought that the preoperative identification of the feeding vessels and severance of blood supply by coil embolization seems to be useful for the prevention of the recurrence.In conclusion, we reported the refractory case of chronic expanding intrapericardial hematoma treated with preoperative coil embolization of the feeding vessels. In cases where the resection of the capsule is difficult, we thought that this method would be very useful and may contribute to the prevention of the recurrence.None declared.MN: treated the patient and drafted the manuscript. HM: supervised the manuscript. MY: was responsible for the case. KT and YS: supervised the surgical strategy of the case.


Recent advancements in multimodal therapy can provide oncologic benefits for patients with recurrent colorectal cancer. This report presents a case of locoregionally recurrent appendiceal cancer treated with neoadjuvant chemotherapy followed by surgical resection with vascular reconstruction. A 68-year-old Japanese woman was diagnosed with appendiceal cancer and underwent ileocecal resection. The pathological evaluation revealed KRAS-mutant adenocarcinoma with the final stage of T4bN1M0. She received oral fluorouracil-based adjuvant chemotherapy. One year later, she was found to have peritoneal dissemination in the pelvic cavity and vaginal metastasis. She received an oxaliplatin-based chemotherapy followed by surgical resection. One year after the second surgery, she developed a locoregional recurrence involving the right external iliac vessels and small intestine. She received an irinotecan-based regimen with bevacizumab as neoadjuvant chemotherapy, followed by surgical resection. At first, a femoro-femoral bypass was made to secure the blood supply to the right lower extremities. Subsequently, an en bloc resection including the recurrent tumor and the external iliac vessels was completed. Surgical resection for recurrent colorectal cancer is often technically challenging because of the tumor location and invasion to adjacent organs. In this case, a surgical approach with persistent chemotherapy achieved oncologic resection of locoregionally recurrent appendiceal cancer. The recurrence rate following curative resection with adjuvant chemotherapy for stage II or III colorectal cancer is approximately 30% . LocoregA 68-year-old Japanese woman with no significant medical history had suffered from pain in her right lower quadrant. She was examined at the regional cancer center and diagnosed with appendiceal cancer. She underwent ileocecal resection with lymph node dissection at the aforementioned hospital, and the postoperative course was uneventful. The pathological evaluation revealed KRAS-mutant (codon 12) moderately to poorly differentiated adenocarcinoma, and the final TNM stage was T4b  N1M0 (stage IIIc). Thereafter, she received adjuvant chemotherapy with oral tegafur/uracil and leucovorin for one year.One year after the surgery, a positron emission tomography/computed tomography (PET/CT) scan showed sporadic foci of intense tracer uptake in the pelvic cavity, consistent with peritoneal dissemination and vaginal metastasis. The recurrent tumor deposits were considered resectable. She received three courses of mFOLFOX6 regimen  as neoadjuvant chemotherapy, followed by resection of the peritoneal dissemination and partial resection of the vagina. The pathological diagnosis confirmed negative resection margins. Then, she resumed the mFOLFOX6 regimen as adjuvant chemotherapy; however, the regimen was discontinued after two courses for an allergic response to oxaliplatin.One year after the second surgery, a surveillance abdominal ultrasonography showed a 27 × 16 mm irregular and low-echoic tumor around the right external iliac artery . A contrWe assumed that the tumor was resectable; however, the external iliac vessels also required resection during the surgery, which required a simultaneous procedure for vascular reconstruction. We proposed neoadjuvant chemotherapy and subsequent surgery for the patient because it was deemed important to control the tumor progression and increase tumor resectability.We selected a FOLFIRI  plus bevacizumab regimen as neoadjuvant chemotherapy, considering the mutant KRAS status. We initially planned six courses of FOLFIRI plus bevacizumab therapy and assess the tumor progression and resectability every 3 months. We discussed with the patient, and she agreed with our proposed treatment strategy. Follow-up CT scans after three and six treatment courses showed no tumor progression. After six courses of the treatment, we recommended surgical resection for her as we planned, but she wanted to receive chemotherapy. FOLFIRI plus bevacizumab regimen was continued accordingly.After eight courses, she developed edema in the right lower extremities. A contrast-enhanced CT showed a narrowing of the right external iliac vein without any thromboemboli in both lower extremities; however, she was incidentally found to have suspicious emboli in her pulmonary arteries. The neoadjuvant chemotherapy was terminated, and she was administered an anticoagulant therapy (edoxaban). After two months, a contrast-enhanced CT was taken again and showed no thromboemboli and no significant tumor progression. Although she had been asymptomatic for the suspicious pulmonary embolism, her lower extremities remained edematous. We, thus, indicated surgical resection of the tumor. The tumor was considered to be resectable, and we asked cardiovascular surgeons for vascular reconstruction at the time of surgery.The surgery was performed in the following sequence. At first, vascular reconstruction was performed by cardiovascular surgeons. A femoro-femoral arterial bypass with synthetic vascular graft  was made to secure the blood supply to the right lower extremities. Subsequently, a laparotomy was performed by colorectal surgeons. A fixed and solid tumor was observed at the right inguinal fossa, involving the external iliac artery, external iliac vein, ileum, and right obturator lymph nodes. En bloc tumor resection was successfully performed including a partial resection of the external iliac artery, external iliac vein, and ileum . The posThe pathological evaluation revealed moderately and poorly differentiated adenocarcinoma consistent with rerecurrence of appendiceal cancer. Tumor invasion was extended to the external iliac vein with tumor embolus inside. The external iliac artery and ileum were free of tumor invasion but strongly attached to the tumor. The obturator lymph nodes and those in the resected mesentery of the ileum were positive for metastasis. Microscopically, cauterized cancer cells were present at the resection margin (R1 resection).She received a FOLFIRI regimen as adjuvant chemotherapy. After six courses, a follow-up CT scan showed liver metastasis, para-aortic lymph node metastasis, and peritoneal dissemination. These recurrent lesions were considered unresectable. The chemotherapy regimen was altered to trifluridine/tipiracil. After seven courses, a follow-up CT scan showed a significant progression of the liver metastasis. Regorafenib was then administered for two weeks; however, it was discontinued because she denied further aggressive treatment. She had received palliative care and died one and half years after the last surgery.Curative surgical resection is crucial for the improved survival of LRCC. A systematic review concerning outcomes of resection for LRCC demonstrated that the R0 resection rate was 51%, and a five-year survival after R0 resection of 52% compared to 11% and 0% for R1 and R2 resection, respectively. They concluded that resection of LRCC is performed safely with long-term survival but that patients who could undergo resection had good performance status; therefore, the results may have been influenced by patient selection .Several studies have investigated the prognostic factors after surgery for LRCC by univariate and multivariate analysis. These factors include margin status , 10, 11,LRCC often invades other organs such as the bladder, uterus, ovaries, and small intestine. Previous reports have shown that 26–100% of patients with LRCC undergo multivisceral resection , 11, 12.Currently, many patients receive systemic therapy including chemotherapy and radiotherapy before and/or after surgical resection for recurrent colorectal cancer. One advantage of preoperative chemotherapy is that we can evaluate the biological aggressiveness of metastatic colorectal cancer and choose appropriate patients who would receive long-term oncologic benefit from surgical resection . In thisSome authors have reported the effect of surgical resection combined with systemic therapy on LRCC. Harji et al. showed that preoperative chemoradiotherapy and/or chemotherapy for LRCC improved the radical resection rate . Ohira eHowever, the efficacy of systemic therapy on survival remains controversial. Kogler et al. reported that neoadjuvant or adjuvant therapy may influence median survival, although there was no statistical difference . In contIn this case, we initially expected that the treatment strategy with sequential chemotherapy followed by surgical resection for LRCC would have provided long-term oncologic benefit. She survived two years and nine months since the diagnosis of rerecurrence. Because the rerecurrent tumor was locoregional, she might have survived for similar duration without surgical resection. Before the last surgery, she had suffered from leg edema, and symptomatic relief was achieved by the surgical resection. Afterwards, she never suffered from leg edema. Therefore, we assume that the surgical resection may have contributed to improved quality of life and better tolerability for the subsequent chemotherapy, rather than oncologic benefit.The pathological evaluation revealed that cauterized cancer cells were present at the resection margin (R1 resection). Intraoperative radiotherapy with surgical resection has been shown to be beneficial for locoregionally advanced primary or recurrent colorectal cancer and other malignancies –27. In oRecent reports have shown the efficacy of en bloc resection of primary pelvic tumors involving main iliac vessels , 29. TheThere are concerns about the choice of vascular graft and the need for venous reconstruction during the surgery. In this case, we used a synthetic graft for arterio-arterial interposition. Autologous grafts are preferred in exenteration surgery where operative field may be contaminated by concomitant bowel resection . We perfSystemic therapy for LRCC appears to contribute to the curative resection rate and long-term survival in selected patients. However, there are no generally accepted protocols for systemic therapy for LRCC. Further investigations including randomized trials are needed to elucidate a standardized treatment strategy for LRCC.We reported a case of locoregionally recurrent appendiceal cancer in which our multimodal treatment provided relatively a long survival after the diagnosis of recurrence. Multiple regimens of neo- and adjuvant chemotherapy with surgical resection contributed to the oncologic outcome. Patient selection with careful assessment, availability of appropriate expertise, and meticulous discussions is necessary for the optimal treatment of LRCC.


Poorly differentiated neuroendocrine carcinomas (NECs) originating from the gastrointestinal (GI) tract are rare and very highly malignant disease with a poor prognosis. Poorly differentiated NECs most commonly arise in the esophagus and the large bowel; however, they may occur within virtually any portion of the GI tract. It is known, however, that they do not typically occur in the small intestine.A 21-year-old woman visited an emergency room with acute abdominal pain that commenced 2 days prior to her presentation. Thereafter, a computed tomography (CT) scan was notable for a small-intestine perforation, and huge masses were observed in the small intestine and the mesentery. The mass that was located at the ileum site is approximately 100 cm above the ileocecal (IC) valve, and while it is located on the anti-mesenteric border and it seems that luminal narrowing had occurred, an obstruction is absent. Also, a same-nature mass is on the mesentery. The pathologic reports confirmed a small-cell-type NEC with a mass size of 7.5 × 6.5 cm. The mitotic count is up to 24/10 high-power fields (HPFs), the results of the immunohistochemical stain are positive for CD56 and synaptophysin, and the Ki-67 level is 50%. %. After the operation, she was treated with Etoposide-Cisplatin (EP) chemotheraphy. Stable disease was seen during Etoposide-Cisplatin chemotheraphy. Liver metastasis was also confirmed after chemotheraphy. Additionally, Irinotecan and cisplatin were used for 3 cycles, but progression of disease, neutropenic fever, thrombocytopenia, general weakness persisted. Eventually, she died 1 year and 6 months after surgery.Ileum-located NECs are diagnosed very rarely. The most common locations for these tumors along the GI tract are the esophagus and the large intestine, but they can arise anywhere. The prognosis for NECs is poor due to the metastatic disease of most patients at the time of diagnosis. The role of adjuvant treatment requires further evaluation for the attainment of a better understanding of the overall treatment effect. Poorly differentiated neuroendocrine carcinomas (NECs) originating from the gastrointestinal (GI) tract are rare and exceptionally harmful illness with a poor prognosis. The knowledge of these malignancies regarding their tumor biology is scant . Poorly A 21-year-old woman visited an emergency room with acute abdominal pain that commenced 2 days prior to her presentation. A specific history of the woman was nonexistent, but she had anemia for 1 year for which iron supplements had been taken; it was suspected that the patient had panperitonitis. Thereafter, a computed tomography (CT) scan was notable for a small-intestine perforation, and huge masses were observed in the small intestine and the mesentery. Also, free air was detected around the small intestine and numerous seeding nodules were found in the abdominal cavity, but no other solid-organ metastasis was evident. Fig.  and 4 Afhigh-grade or G3 NECs, are greater than 20 × 10 HPFs, and the Ki-67 level is greater than 20%. The Ki-67 index is most likely the best accessible marker of tumor-cell proliferation. The angioinvasion of high-proliferation tumors with a Ki-67 level higher than 20% is extensive, and these tumors demonstrate an incredible potential to create metastatic disease. As indicated by research effectively detailed that the frequency for positive immunohistochemical reactivity for CK8, synaptophysin, NSE and CD56 in gastrointestinal small-cell NEC was > 90% and that these markers were helpful in diagnosis. Analysis by Shia et al. recognized the following three factors as having an adverse impact on 2-year disease-specific survival: the absence of an adenocarcinoma component (P = 0.04), the presence of synaptophysin staining (P = 0.05) and progressed disease stage (P < 0.0001). The present case has all three of these factors as known already. Subsequently, present case is likewise expected to poor prognosis [NECs of the GI tract are uncommon tumors and are classified as either the small-cell or the large-cell type. Thus, NEC of the small bowel is very uncommon. Pathologically, poorly differentiated NECs are similar to small-cell lung cancer (SCLC), and hence their present treatment techniques are similar . NECs arrognosis , 12. Therognosis . After trognosis . Albeit rognosis . In spitrognosis , 17. The


A 37-year-old (G4P3) woman was referred to our hospital at 32 weeks of gestation for the evaluation of a fetus with an intrathoracic cystic lesion. Ultrasonography and magnetic resonance imaging revealed that a fetal cystic lesion without a mucosal layer was located in the posterior mediastinum. These findings were consistent with a bronchogenic cyst. At 38 3/7 weeks of gestation, an elective cesarean section was performed because of her previous cesarean section. A female neonate without any external anomalies, weighing 2,442 g, with Apgar scores of 8 and 9, and requiring no resuscitation was born. Four weeks after delivery, the neonate was admitted because of respiratory distress due to mass effect. At right lateral thoracotomy, a 105 × 65 mm of solitary smooth-walled cyst containing serosanguineous fluid was found in the posterior mediastinum, which was excised completely. Histologic examination revealed the diagnosis of the mediastinal gastric duplication cyst. The neonate made an uneventful recovery. Accurate diagnosis is not necessary, but detection and continuous observation are logical. Although gastric duplication, particularly intrathoracic, is a rare pathology, it should be considered in the differential diagnosis of any intrathoracic cyst. Foregut duplication cysts are rare congenital anomalies of enteric origin; they constitute 10%–18% of all mediastinal lesions. They are further subdivided into bronchogenic, esophageal, gastric, enteric, and pancreatic cysts . AlthougA 37-year-old (G4P3) woman was referred to the University of the Ryukyus Hospital at 32 weeks of gestation for the evaluation of a fetus with an intrathoracic cystic lesion. An US examination revealed a 39 × 30 × 44-mm sized monocystic lesion in the mediastinum, in which the aortic arch was displaced upward . MagnetiThe diagnosis of intrathoracic cysts is challenging because of several possible pathologic findings, including bronchogenic cysts, neurenteric cysts, and other foregut duplication cysts. Most intrathoracic alimentary tract duplications present before the age of 2 years , 4. TherDuplication cysts can present various symptoms, such as dyspnea, stridor, or persistent cough, according to the location and type of the cysts. Asymptomatic patients later become symptomatic because of cyst enlargement, which was seen in our patient . HoweverThe etiology of enteric duplication remains speculative. The most accepted theory “split notochord syndrome” postulated the abnormal separation of the notochord from the endoderm, leading to enteric duplications . Althoug


Gastric duplication is a relatively rare congenital malformation, accounting for approximately 2.9–3.8% of gastrointestinal duplications. Gastric duplication cyst is a congenital anomaly that is rarely observed in adults. Accurate diagnosis of these cysts before resection is difficult. In this report, we describe a patient with gastric duplication cysts that were treated by laparoscopic resection.A 46-year-old Japanese woman was referred to our institution because a cystic lesion in the pancreatic tail was detected by ultrasonography during a health examination. The lesion had a clearly defined boundary of approximately 40 mm. A thick cystic lesion of the septum was observed in the pancreatic tail, but invasion into the stomach wall was not recognized on a computed tomographic scan. Endoscopic ultrasonography revealed that the tumor appeared smooth with a marginal edge, which was characterized by echo with high homogeneity, and the presence of viscous mucus was suspected. The preoperative diagnosis of mucinous cystic neoplasm was the reason for laparoscopic tumor resection. The resected specimen was a smooth surface tumor, and it was full of mucus. Histopathological study revealed that the mucosa was covered with crypt epithelium, muscularis mucosae, intrinsic muscularis, and serosa, and the wall of the tumor had a structure very similar to that of the stomach wall. The mucosa was partially drained by intrinsic gastric glands, but most of them were denucleated. No pancreatic tissue was present, and the tumor had no continuity with the spleen. These findings indicated a diagnosis of gastric duplication cyst with no continuity with the stomach wall.In our experience, it is difficult to differentiate gastric duplication cyst from mucinous cystic neoplasm before laparoscopic resection. Events such as infection, bleeding, perforation, ulceration, fistula formation, obstruction, and compression have been linked to gastric duplication cysts, and malignant transformation of these cysts has been reported. Therefore, we suggest that resection should be the first treatment option for gastric duplication cysts. Gastric duplication cyst (GDC) is a congenital anomaly that is rarely observed in adults . In apprAccurate diagnosis of these cysts before resection is difficult. Differential diagnoses are varied, including gastrointestinal stromal tumors, neuroendocrine tumors, pancreatic heterotopia, pancreatic pseudocysts, and neurogenic tumors .GDCs are extremely difficult to diagnose preoperatively. We describe a case of a patient with laparoscopically resected GDCs, focusing on diagnostic and therapeutic methods. We initially misdiagnosed our patient as having mucinous cystic neoplasms (MCNs) and performed laparoscopic resection.Our patient was an otherwise healthy 46-year-old Japanese woman who was referred to our institution because a cystic lesion in the pancreatic tail was detected by ultrasonography during a health examination. Her past medical history and family medical history were unremarkable. She was not taking any medication. She did not have a smoking habit; however, she occasionally drank alcohol. Blood tests revealed no abnormality. Levels of tumor markers were not elevated Table . She hadAbdominal ultrasonography Fig.  revealedDuring laparoscopic surgery, a soft tumor whose surface was smooth, like the serosa of the stomach wall, was found in the pancreatic tail Fig. . There wHistopathological study Fig.  revealedThe postoperative course was uneventful, and the patient was discharged 6 days after surgery.The patient’s subsequent clinical course was unremarkable, and she visits our institution on an outpatient basis every 6 months.GDCs may occur in any anatomical region of the gastrointestinal tract, from mouth to anus, and are present in 1 per 4500–10,000 live births [The essential criteria for diagnosis of GDC are as follows: (1) the whole cyst is contiguous with the stomach wall; (2) the cyst is surrounded by smooth muscle, which is continuous with the muscles of the stomach; and (3) the cyst wall is lined by epithelium of gastric or any other type of gut mucosa –8. More Because most cases occur along the greater curvature of the stomach, the cysts can potentially compress adjacent organs, such as the pancreas, spleen, and adrenal glands. Therefore, the differential diagnosis includes lesions arising from these organs . It is eThe cysts may also be manifested by complications such as infection, bleeding, perforation, ulceration, fistula formation, obstruction, and compression . In adulGDCs are difficult to differentiate from MCNs until they are laparoscopically resected. Events such as infection, bleeding, perforation, ulceration, fistula formation, obstruction, and compression have been linked to GDCs, and malignant transformation of these cysts has been reported. Therefore, we suggest that resection should be the first treatment option for GDCs.


Papillary thyroid carcinoma (PTC) arising in a coexistent struma ovarii (SO) is a rare malignancy. It manifests with abdominal symptoms such as palpable mass, pain, distension, and possibly ascites. It is usually diagnosed postoperatively, and its histopathological diagnostic criteria remain identical to that of papillary carcinoma of the thyroid gland. Due to the relative rarity of the disease, definitive guidelines for its overall management are still undefined. We present a case of a 51-year old female with suspicion of a left ovarian tumor due to her presenting symptoms: raised serum CA-125 levels and abnormal abdominopelvic CT scan findings. She underwent complete surgical debulking of the mass , bilateral salpingo-oophorectomy (BSO), omentectomy, appendectomy, and pelvic lymphadenectomy). The mass was postoperatively diagnosed by histopathology as PTC in SO (stage IA). Furthermore, our patient did not receive any adjuvant treatment. The patient has been disease-free for 24 months post-surgery and is scheduled for regular biannual follow-ups. Struma ovarii (SO) is defined as the presence of a single predominant type of mature thyroid tissue in a coexisting ovarian tumor. To date, only about 200 cases have been reported in the literature. It presents more frequently between the ages of 40 and 60 . The majA 51-year-old female  presented to our walk-in clinic with a four-month history of progressive lower abdominal pain and distention in the lower left quadrant. She complained of indigestion and had had significant weight loss in the last two months. Her past medical history revealed she had been operated for cholelithiasis 14 years ago. The patient's family history was insignificant for other comorbidities and cancer. Physical examination was consistent with a palpable abdominopelvic mass (8-10 weeks in size) on the lower left side.A CT scan of the abdomen and pelvis yielded a large left adnexal mass measuring approximately 8.1 x 6.7 cm Figure . The uteHistopathological studies of the left ovarian mass revealed a focus of classical PTC measuring 2 cm in the background of a SO Figures -5. The fOur patient was scheduled for biannual follow-ups and is now disease-free two years post-resection. Postoperative serial measurements of thyroglobulin, anti-thyroglobulin antibody, serum CA-125 levels and contrast CT imaging of chest, abdomen, and pelvis have been conducted. CT has not shown any residual or recurrent disease so far. The patient is euthyroid with normal postoperative thyroglobulin and anti-thyroglobulin antibody levels. Serum CA-125 levels have shown an initial decreasing trend post-surgery. However, during the second follow-up visit, a raised serum CA-125 level was noted, which regressed to baseline during the subsequent follow-ups. The preoperative and postoperative progression patterns of serum CA-125 levels are noted below in Table Germ cell tumors of the ovary are known to arise from any form of pathogenic insult to the normal ovarian germ cell lineage. Such disruption in physiological growth can occur during any stage of ovarian germ cell maturation ultimately leading to neoplastic transformation . Table 2Due to the rarity of MSO and its available literature data, no formal standard criteria have been established regarding its diagnosis, management, and follow-up ,2. ThereConfirmation of PTC in a SO is usually postoperative because it requires a histopathological analysis for its exact identification . AccordiPatients can also have high levels of CA-125, a finding also consistent with our patient (413 U/ml preoperatively) . FactorsNo definitive management guidelines regarding PTC in SO have been proposed so far. The decision about the surgical treatment of MSO depends mainly on the need to preserve the fertility of the patient and the extent of the tumor. A unilateral salpingo-oophorectomy is recommended if the patient wants to preserve fertility, the tumor is confined to the ovary, and if there is no evidence of thyroid nodules ,4. When Studies have shown a favorable postoperative prognosis for non-metastasized MSO. A study conducted by Goffredo P et al., which delved into 68 patients, indicated survival rates of 96.7%, 94.3%, and 84.9% at 5, 10, and 20 years respectively . The comWe strongly recommend presenting patients with a plan to adhere to a regular biannual or triannual follow-up with abdominopelvic CT imaging with contrast and serum CA-125 monitoring. Postoperative thyroid carcinoma surveillance is further essential to exclude ovarian metastasis as a possibility, and this can be achieved with serial measurements of thyroglobulin and antithyroglobulin antibody levels. Follow-up surveillance for possible metastasis should also include contrast CT imaging of the chest.We reported a rare case of a 51-year-old female, who presented with a four-month history of progressive lower abdominal pain and a mass (8-10 weeks in size) in the left lower quadrant. With the aid of CT imaging and histopathological studies, the diagnosis of MSO was confirmed. Our case report discusses the etiology, clinical presentation, and management strategies of MSO. Due to the chances of metastasis and recurrence, we also lay stress on the importance of implementing postoperative follow-up surveillance. Furthermore, we also reiterate that, given the rarity of MSO, no formal standard criteria have been established. This makes the management of such cases a real clinical challenge.


The clinical findings of early anal gland carcinoma (AGC) have not been well delineated because AGC is a rare malignancy usually diagnosed at an advanced stage. Knowledge of the characteristic findings will be helpful for both diagnosis and determination of the treatment options for early AGC.A 62-year-old man was referred to our hospital for treatment of a rectal submucosal tumor (SMT) detected during a medical checkup at another hospital. Trans-sacral resection of the tumor was performed under the diagnosis of a rectal benign cyst. Pathological examination of the resected tumor showed a mucin-producing adenoma. About 14 months later, a new cystic lesion was found by follow-up examination, and trans-sacral resection of the tumor was performed again. The second pathological diagnosis was a mucinous adenocarcinoma with a possible remnant tumor at the local site. After providing sufficient informed consent, the patient underwent intersphincteric resection (ISR) of the rectum to preserve anal function. The final diagnosis was mucinous adenocarcinoma of the anal gland, T1N0M0. The patient remained alive without recurrence or complications for 6 years 7 months postoperatively.We have herein reported a case of early AGC with a characteristic SMT-like appearance. Because the anal gland is located within both the submucosal layer and the internal sphincter muscle, ISR may be selected when the tumor is limited to inside the gland. Adenocarcinomas of the anal gland are rare malignancies that are found in an advanced stage in most patients . MucinouA 62-year-old man was referred to our hospital for treatment of a rectal SMT detected during a medical checkup at another hospital. Digital examination of the anus and rectum revealed a 20-mm elastic hard tumor palpable on the right and ventral sides of the anal canal and located 2 to 3 cm proximal to the anal verge. Laboratory examination showed no elevation of either carcinoembryonic antigen or cancer antigen 19-9. Colonoscopy showed a 20-mm SMT in the anal canal Fig. . MagnetiCarcinomas of the anal canal are rare malignancies, comprising approximately 1 to 2% of all gastrointestinal carcinomas . Most anAlthough local excision can be performed in some cases when the tumor is limited in its location , 12, theIn conclusion, we have described a case of early stage mucinous adenocarcinoma arising from the anal gland with a characteristic SMT-like cystic appearance. When the tumor spread is limited to within the anal gland, an anal sphincter-preserving operation may be considered in addition to abdominoperineal resection for total removal of the anal gland even after local excision has failed.


Vascular leiomyomas or angioleiomyomas are rare benign solitary smooth muscle tumors that origin usually in the extremities. Most of these tumors are composed of venous vessels. Here in, we report a rare case of subcutaneous vascular leiomyoma of the right knee of a 38 year old woman who was presented with recurrent anterior right knee pain and soft tissue swelling. Clinical findings, magnetic resonance imaging and histopathologic findings of the tumor is discussed. Leiomyomas are not mostly considered in the differential diagnosis by radiologist due to its rarity. Typical imaging and clinical findings of a tumor is an important clue for an accurate and early diagnosis. Angioleiomyomas or vascular leiomyomas are rare subcutaneus benign tumours arising from smooth muscle cells of arterial or venous walls . They ar2A 38-year-old woman presented with painful, mobile swelling in the right knee. There was no trauma history, and the onset of pain was sporadic and had reduced her mobility. On physical examination, there was a mobile painful swelling on the lateral aspect of her right knee. The laboratory findings were normal. Plain orthogonal radiographs including lateral and skyline views of the knee demostrated no bone abnormalities Figure . But theThe patient underwent surgery and complete excision of the tumor was performed. Gross examination revealed a 10×10 mm firm, well-circumscribed, bean-shaped mass with a white-beige cut surface. Histological sections demonstrated a solid tumor composed of intersecting fascicles of mature smooth muscle cells surrounding vascular structures Figure . The smoVascular leiomyomas or angioleiomyomas are benign subcutaneus tumours that originate from the smooth muscles of blood vessels. They commonly influence the lower extremities, but they seldom affect the knee 2. Female2Morimoto defined three subtypes: solid or capillary, cavernous, and venous 4. Solid 4Ultrasound remains the first-line imaging tool to confirm the presence of a mass lesion and its solid nature. MRI is the best choice for imaging because it can better delineate the lesion and define its relationship to the adjacent structures. Hwang et al. defined that hyperintens areas on T2-weighted MR images show the smooth muscle and numerous vessels in vascular leiomyomas . The fib456In conclusion, when a painful subcutaneus swelling in lower extremities is seen, vascular leiomyoma must be kept in mind for the differential diagnosis. MRI is the best choice for imaging that can delineate the lesion and define its relationship to the adjacent structures, allowing better pre-excisional planning.Leiomyomas are not often considered in the differential diagnosis by radiologists due to their rarity. They are usually well-circumscribed, sharply outlined, homogenous, and show prominent enhancement. Typical imaging and clinical findings of a tumor are important clues for an accurate and early diagnosis.The authors declare that they have no competing interests.


Post-traumatic localized hand lymphedema is a rare situation and its diagnosis may be difficult, causing lack of care leading to failure of care. Our case study is of two young women with massive post-traumatic hand lymphedema who were treated for algodystrophy for 2 years, and whose bandages and physiotherapy were unsuccessful. Major social and psychological consequences due to difficulty with diagnosis and management resulting in inappropriate tests and therapeutic treatment were prescribed due to these issues. Noncontrast magnetic resonance lymphography revealed complete lymphatic vessel blockage in the hand and wrist. A vascularized lymph node flap harvested at the groin level was transferred to the elbow level 1 month after local dermolipectomy. These procedures resulted in the restoration of lymphatic flow. Both patients were definitely cured, and they returned to normal life within 6 months after surgery. Lymph node flap transfer associated with dermolipectomy may cure massive localized lymphedema in selected cases. Lymphedema of the arm as a complication of surgery for breast cancer is the most common situation and its etiologies are well known. However, many physiological questions persist concerning spontaneous, congenital, or post-traumatic forms of this disease. To our knowledge, no series occur in literature concerning post-traumatic and post-burn lymphedema. Only rare cases describe lymphedema of the hand following a fracture of the distal radius, and the role that the traumatism played was controversial . Older dA 21-year-old woman without former medical history had a bicycle accident resulting in her right-hand traumatism. No fractures were detected on X-rays. Wrist sprain was suspected because of persistent swelling. A plaster cast was applied for 15 days. Physiotherapy, including manual drainage, was performed. Two months later, the hand was still inflated and painful. Algodystrophy was suspected following a bone scan. Venous Doppler ultrasound of the upper limb excluded the diagnosis of phlebitis. The hand was so swollen a and paiA 26-year-old woman without former medical history had had a right-hand lymphedema for 4 years, which evolved quickly within a few weeks. The origin was apparently a burn of the hand. Six months after analgesic treatment, the patient was referred to a lymphology center. She was told that it was a chronic disease without any possibility of recovery and could be treated by massages, lymphatic drainage, and bandages. Unable to work because of hand impairment, she lost her job and could not find a new job because of the monstrous aspect of her hand . Her refPost-traumatic hand lymphedema is a rare lymphatic disease and is most certainly due to a local dysplasia of a weak lymphatic system, deteriorated by infections. In our two case studies, the diagnosis was so delayed that the prognosis worsened considerably. Despite new recently published treatments , adaptedHand lymphedema mechanisms are poorly defined. An external factor  may decompensate a congenital malformation by sudden or progressive blockage of the LF in malformed LVs. At wrist level, the diffusion spaces are restricted and the phenomena can quickly lead to important complications as can be observed in carpal tunnel syndrome post-lymphedema. High tissue pressure might also block LVs regeneration usually occurring after LV injury. Misdiagnosing the disease as an incurable chronic illness might have had irreversible consequences . For our two patients, NCMRL and surgery fortunately helped avoid these fatal outcomes.Concerning lymphatic imaging, bipedal-lymphography is not suitable and lymphoscintigraphy is not always appropriately suitable. In fact, this exam is of no interest in the case of obliterated LVs, because it requires functional LF. Nowadays, NCMRL enables the exploration of complex lymphatic abnormalities  and provThe surgical technique of VLNFT is based on two essential notions: LVs anatomy and knowledge of LVs growth patterns − lymphangiogenesis. Indeed, VLNFT brings vascular endothelial growth factor-C-producing tissue, favoring local lymphangiogenesis and bridging the distal system with the proximal lymphatic system. Harvesting lymph nodes (LNs) with their feeding blood vessels and transferring them to another site without disrupting the donor-site LF  is safe Secondary lymphedema due to donor-site LV injury is rare and its severity is never comparable to that of the treated lymphedema. The analysis of LVs anatomy by NCMRL allows verification of LNs transfer feasibility and prevention of such complications  − in theTo conclude, we demonstrated that lymph node flap transfer associated with dermolipectomy may cure massive localized lymphedema in selected cases. However, to date, this technique has been performed only by a few expert surgeons who remain the sole references for this atypical pathology.The authors declare that they have no conflicts of interest in relation to this article.


We report the case of a 51-year-old patient, known as hypertensive and type 2 diabetic, admitted to emergency for acute pyelonephritis. She suffered from low back pain in a feverish context. A Uro-scanner showed the presence of a 12mm pyelic renal calculus and an calycal calculus of 6mm diameter responsible for a moderate dilation of left pyelocalictic cavities. First, we performed drainage with a double J probe. Then, in a second step, the left ureteroscopy allowed partial laser fragmentation. The presence of a suspect soft magma prompted us to stop the procedure. After performing a hydatid serology that has returned negative, a laparoscopic left pyelotomy allowed the progressive externalization of the suspect magma. It presented with a greenish, fibrinous, semi-solid shell completely molding the pyelon and the pyelocalicielles cavities. The introduction of the flexible cystoscope through a trocar at the end of the procedure allowed us to check the complete cleaning of the excretory cavities. The anatomopathological study is back in favor of a weakly eosinophilic acellular material with some polyhedral crystals and some inflammatory elements. There were no signs of malignancy. The patient did not present any postoperative complication and did not present a recurrence.


Müllerian adenosarcoma is a rare malignancy. These tumors occur mainly in the uterus, but also in extrauterine locations, usually related to endometriosis. Because of their rarity, there is limited data on optimal management strategies.We present a 44-year-old woman with a history of endometriosis who consults for chronic pelvic pain. In the imaging tests, a heterogeneous mass is observed that impresses endometriosis, encompassing the uterus and left appendage. Surgery is performed by finding an extrauterine adenosarcoma that affected the uterus, ovary and bladder wall.This is a rare case but should be considered in a patient with atypical clinical characteristics or preoperative pathology, so we show the diagnostic and therapeutic strategies carried out for the resolution of the case. Endometriosis is a common condition among women of reproductive age. Although considered a benign disease, malignant transformation is possible. The incidence is higher in ovarian lesions (6–8%) and rare in extraovarian endometriosis 1%) [% [1]. ThIn this clinical case, we present a low-grade adenocarcinoma that arising in deep endometriosis. The patient was informed and gave her written consent to the elaboration and publication of this review. Similarly, the ethics committee of our centre gave its approval.44 year old patient, nulligest, with a history of deep endometriosis. Intervention of appendectomy, tonsillectomy and Toilette via laparoscopic, remitted for severe abdominal pain.Followed for 3 years, in another center. Current treatment with gestagen, dienogest, comes to our center for severe abdominal pain requiring admission. Asked about menstrual symptoms, the patient reports spotting and irregular bleeding.Provides additional evidence; transvaginal ultrasound: 3 cm intramural myoma, left ovary (39 × 28 mm) 18 mm cyst of endometriosis appearance; adjacent to ovary tubular heterogeneous formation of 54 × 23 mm . Blood test on admission: hemogram, coagulation and biochemistry were normal, CRP 5.2 and BHCG negative. Elevated tumor markers CA 125253.7.She is discharged for clinical improvement and a full outpatient study is completed.Rectosigmoidoscopy: without pathological findings, CT and MRI scan present in contact with the lateral wall of the uterus and without separation, an imprecise edge lesion of approximately 10 cm that extends throughout the left hemipelvis, inside which there are cystic areas, solids and several hemorrhagic foci, this lesion includes the left annex. To rule out Carcinoma of ovary as first diagnosis.  due to the unusual nature of the lesions and their infiltration, all reported as endometriosis. Double J placement is performed and then total hysterectomy with double adnexectomy + partial bladder resection at the posterior face level + resection of endometriosis implant in left iliac fossa is performed.Postoperative evolution favorable, the patient receiving discharge postoperatively on the 4th postoperative day.The result of the surgical piece was: a uterus with an inactive endometrium, extensive adenomyosis, a 3 cm intramural myoma and low-grade adenosarcoma. Left ovary with a fibroid and low-grade adenosarcoma. Fragment of bladder wall with low-grade adenosarcoma infiltration. Adenosarcoma samples with estrogen receptor positive 100%, progesterone receptor positive100% and CD10 positive .Differential diagnosis with polypoid endometriosis was proposed, despite the stromal cellularity with a pattern of low-grade stromal sarcoma with mild and focally moderate cytological atypia as well as the histological pattern (areas with polypoid projections in glandular lights) and the behavior indicate without a doubt that it is a low-grade adenosarcoma.The oncology committee decides on treatment with hormone therapy, with Letrozole being the standard. Control at 3 with imaging test (CT) without observing alterations or new lesions. Control at 6 months, the patient being asymptomatic and with good quality of life.A mullerian adenosarcoma is an exceptional tumor characterized by a generally low malignant stromal component and a benign glandular epithelial component , 6. ThesThe risk of developing adenosarcoma increases in post-menopause age, with the average age at diagnosis being between 50 and 60 years. However, in the case of extra-uterine müllerian adenosarcomas, presentation in younger women is common, with an average age at diagnosis of 44 years. These tumours present a more aggressive behaviour, probably due to their location, which allows them to spread through the peritoneal cavity .With respect to treatment, surgery is usually sufficient and curative in mullerian uterine adenosarcomas, with a recurrence rate of approximately 25% of cases. On the other hand, in the case of extra-uterine adenosarcomas the recurrence rate increases above 50% of patients and mortality rates are approximately of 35% .The pathogenesis of the tumor is unclear. However, the association between exogenous hormone therapy and the development of malignant tumours in endometriosis is well known, and several studies have suggested the possibility of a neoplastic transformation of endometriosis sites .A large Stern review of endometriosis-associated neoplasms showed that the most common malignancies were endometriode adenocarcinoma and clear cell carcinoma, with adenosarcoma mülleriano being much less common , 8.This case meets the Sampson  criteriaExtrauterine tumors are clearly less common and are located mainly in the ovary, vagina, fallopian tube, broad and round ligaments, bottom of the Douglas sac, intestinal serosa and liver , 3.The low incidence of Mullerian adenosarcomas complicates the development of well-defined and consensual action protocols. The main therapeutic weapon continues to be radical surgery and a similar approach to that used in endometrial carcinoma is usually recommended. The extent of muscle invasion and sarcomatous growth have a great influence when considering adjuvant chemotherapy. Adjuvancy is also used in the extra-uterine müllerian adenosarcoma although its usefulness is still debatable .The most important negative prognostic factors are the location of the lesion, the degree of myometrial invasion and sarcomatous overgrowth . In contOur case shows an association with endometriosis, which supports the hypothesis that an extra-uterine müllerian adenosarcoma can be transformed from this disease. The action of exogenous and endogenous oestrogens is currently being studied due to their possible relationship with the transformation process. It is also thought that the titles of the CA 125 tumour marker, that are well related to the clinical course of endometriosis, may become a predictor of sarcomatous overgrowth .In conclusion, an extra-uterine müllerian adenosarcoma is rare, but should be considered in a patient with atypical clinical features or preoperative pathology, especially in a patient with a history of endometriosis. More research is needed to establish the pathogenesis of extra-uterine adenosarcoma and the therapeutic and diagnostic guidelines.


The plantar fascia (PF) is an important anatomical structure that stabilizes the longitudinal arch of the foot. While plantar fasciitis is a common pathology affecting the foot, tears of the PF are uncommon injuries characterized by acute pain in the plantar aspect of the foot. The main purpose of this paper was to describe, in detail, how the ultrasonographic pattern of PF rupture can be combined with the clinical features to define the prognosis and promptly plan the therapeutic approach.In the first case, a 39-year-old male patient was seen due to acute pain in the mid plantar foot which appeared 3 days after a tennis match. The pain was accompanied by a “snap” noise and intense pain. In the second case, a 44-year-old male patient was seen due to pain in the heel region which appeared 2 days after a running session.One case of noninsertional complete tear of the central bundle of the PF with retraction of the 2 stumps and 1 case of partial tear of the central bundle of the PF at the level of the insertional region.Both patients were treated with conservative therapies including load management, oral nonsteroidal anti-inflammatory drugs, foot orthosis, and restriction of sport activities.At follow-up, the patient with spontaneous complete tear of the PF  showed a small fibrous bridge between the 2 stumps, with partial alignment of the proximal and distal portions, the ability to walk for a medium to long-distance, and difficulty going up and downstairs. The patient with the spontaneous partial tear  showed complete fibrous scar tissue with restoration of the fascial continuity, and the ability to walk for a long-distance and go up and downstairs without pain.Based on the clinical and ultrasonographic findings, we suggest that partial tear of the PF in the insertional region presents a favorable prognosis with complete recovery, both clinically and anatomically, while a complete tear in the noninsertional region is associated with partial functional and histological recovery when managed with a conservative approach. Therefore, coupling the clinical findings with the sonohistologic pattern is a valuable approach to plan the most suitable treatment for patients with spontaneous PF tear. It consists of 3 main bundles: medial, central, and lateral. The central bundle is the thickest one, starting from the medial tuberculum of the calcaneus and extending forward to cover the plantar surface of the flexor digitorum brevis muscle. The lateral bundle beneath the plantar surface of the abductor digiti quinti muscle continues laterally with the dorsal fascia and distally with the fifth metatarsophalangeal joint capsule. The medial bundle of the PF is the thinnest one, located under the plantar surface of the abductor hallucix muscle, extending proximally to the flexor retinaculum of the foot and distally to the first metatarsophalangeal joint capsule.[Plantar fasciitis is commonplace in daily clinical practice, and is estimated to be responsible for more than 1 million patients seeking treatment annually.Traumatic tears are often related to forcible plantar flexion of the foot , and typically occur distal to the calcaneal insertion of the PF. Thus, chronic overuse is usually considered an aetiological factor in these patients.[2Here, we report 2 cases of spontaneous tear of the PF in patients with no preceding symptoms or steroid injections. The clinical findings were confirmed by diagnostic ultrasound (US) examination, which revealed severe fasciopathy with structural lesions.2.1A 39-year-old male patient was seen due to acute pain in the mid plantar foot which appeared 3 days after a tennis match. The pain was accompanied by a “snap” noise and intense pain. Physical examination revealed local swelling, ecchymosis, and tenderness at the level of the heel and plantar foot, in addition to painful dorsiflexion of the first metatarsophalangeal joint. US examination was performed in accordance with the standard procedure for scanning the plantar region of the foot,2.2A 44-year-old male patient was seen due to pain in the heel region which appeared 2 days after a running session. Physical examination revealed local swelling at the level of the calcaneal region and plantar foot and moderate pain during dorsiflexion of the first metatarsophalangeal joint. US examination was performed in accordance with the standard procedure for scanning the plantar region of the foot,3The diagnosis of PF tear mainly relies on the patient's medical history and on physical examination. A history of acute pain in the sole of the foot during activity, together with clinical findings of a tender lump, local swelling from subcutaneous bleeding and often accompanied by a “snap” noise, suggest the diagnosis.US examination is the first-line modality to localize the tissue lesion and measure its extension. Furthermore, dynamic maneuvres facilitate confirmation of a complete tear by verifying the presence of a gap between the 2 torn parts of the PF.Based on the clinical and ultrasonographic findings, we speculate that a partial tear of the PF in the insertional region could be considered a lesion with a favorable prognosis which is expected to recover completely, both clinically and anatomically. Complete restoration of the fascial continuity in the insertional area is probably related to the continuous microtraumas that occur during walking, and; therefore, greater stimulation by local growth factors. On the other hand, complete tear of the PF in the noninsertional region could be considered a lesion with partial functional and histological recovery when managed with a conservative approach. Acceptable alignment of the 2 stumps and an incomplete connection of the proximal and distal segment of the PF with a fibrous bridge could be sufficient for daily activities but not for sport activities. The gap between the 2 stumps and the distance from the loading zones of the foot  are probably correlated with poor clinical and ultrasonographic outcomes.Conceptualization: Giulio Cocco.Data curation: Giulio Cocco.Investigation: Giulio Cocco, Vincenzo Ricci, Maria Teresa Guagnano.Methodology: Andrea Boccatonda.Project administration: Vincenzo Ricci.Supervision: Michele Abate, Cosima Schiavone.Validation: Andrea Boccatonda, Cosima Schiavone.Writing – original draft: Giulio Cocco.Writing – review and editing: Vincenzo Ricci, Andrea Boccatonda, Cosima Schiavone.


Undifferentiated pleomorphic sarcoma (UPS) is a rare tumor of mesenchymal origin affecting the hand in less than 3% of cases. A 92-year-old male reported an enlarging, painless mass on his left hand of three months duration. Examination of the hand revealed a soft, non-tender, immobile mass over the wrist joint, just proximal to his thumb. Immunohistochemistry findings on ultrasound-guided biopsy were consistent with UPS. Further intractable pain, ulceration, and bleeding necessitated urgent radiotherapy, which provided no relief. This was followed by amputation above the elbow. Seven months later, the patient presented with confusion. A chest X-ray revealed extensive bilateral pulmonary metastases. In light of this result, the patient was referred to the palliative care team. UPS carries a poor prognosis, with a high risk of metastases. Early diagnosis and treatment is required for optimal clinical outcomes. Through this case, we highlight the need for a more effective treatment strategy to improve clinical outcomes. Sarcomas are a heterogeneous group of rare malignant mesenchymal tumors, which can be categorized into either bone or soft tissue sarcomas. They have an annual incidence of two to three cases per 100,000 individuals . We presA 92-year-old, right-handed male presented to the oncology department with a three-month history of a rapidly enlarging, painless mass on the dorsum of his left hand. He had a past medical history of type 2 diabetes mellitus, hypertension, atrial fibrillation, and cerebral vascular disease. On examination of the hand, there was a 7 cm x 6 cm soft, non-tender, and immobile mass on the lateral aspect of the left hand, with no tethering to the overlying skin. There was a full range of movement of his wrist joint and fingers, and there were no neurological deficits. Repeat examination a few months later revealed that the mass had increased in size, ulcerated, and began to bleed Figure .An initial ultrasound scan demonstrated a large mass arising from the radial wrist, measuring approximately 6.4 cm x 5.3 cm x 2.7 cm. Before a biopsy was attempted, a computed tomography (CT) angiogram was performed to rule out any aneurysm within the mass. This demonstrated that the mass had increased in size (diameter of 8.5 cm) after two months. An ultrasound-guided biopsy of the hand mass was performed: this displayed moderately pleomorphic epithelioid spindle cells within a myxoid stroma containing thin-walled and compressed vessels. These features were suggestive of a spindle cell sarcoma.A CT scan performed two months later demonstrated a mass lesion measuring 12.5 cm x 8 cm x 6 cm, centered around the level of the radiocarpal joint and enveloped the radial artery. A staging CT scan of the chest, abdomen, and pelvis revealed a single 4-mm nodule in the upper lobe of the right lung. This was thought to be a simple nodule, and, therefore, a surveillance CT scan of the chest was booked to take place in four months.Because of extreme pain and bleeding from the tumor, neoadjuvant radiotherapy was given to reduce tumor size and relieve pain. However, despite the radiotherapy, there was no appreciable improvement in size or symptoms. After extensive multidisciplinary team discussions, a left above-the-elbow amputation with wide safety margins was performed to provide the patient with a better quality of life. Histopathology examination of the post-surgical specimen illustrated ovoid-spindle cells and pleomorphic tumor cells with no definite line of differentiation (mitotic count more than 20 per 10 high-power fields in some areas). The immunohistochemical examination demonstrated negative staining for S-100 and CD34, scanty staining for cytokeratin AE1/AE3 and patchy positive staining for desmin. These features confirmed a high-grade (grade 3) UPS  is an aggressive malignant tumor affecting the soft tissues. UPS has no specific cell of origin or histiocytic line of differentiation and is usually a diagnosis of exclusion . It commHistology is essential in determining the diagnosis; characteristic features include a combination of storiform and pleomorphic areas containing pleomorphic spindle cells with marked atypia, numerous mitotic figures, and areas of necrosis with lymphohistiocytic infiltrates . When UPTreatment involves total surgical excision of the tumor with wide safety margins preceded by adjuvant radiotherapy in patients with non-metastatic UPS; however, the high-grade nature of the tumor limits any benefit from localized treatments.UPS has a significant recurrence rate with 44% of patients experiencing local invasion and 42% metastasizing despite surgical excision of the primary tumor .Although intensive chemotherapy and the establishment of surgical procedures have improved the outcomes of patients with sarcoma, the curative rate of recurrent and metastatic sarcomas is still not satisfactory. A previous study compared standard neoadjuvant chemotherapy (epirubicin and ifosfamide) with histotype-tailored neoadjuvant chemotherapy (gemcitabine and docetaxel) for the treatment of UPS. This study did not demonstrate any superiority between the two regimens, although the common side effects of thrombocytopenia, anemia, and neutropenia occurred less frequently in the histotype-tailored chemotherapy group . There aThis is a unique case in that the age and location of presentation were highly unusual when compared to available case studies. Additionally, the rate of metastases was far faster than the usual 12-24-month time window. It is highly probable that the suspected pulmonary nodule found on the initial CT scan of the chest was the first sign of early metastases.Early and accurate histological typing, grading, and staging of UPS is essential in determining the prognosis and subsequent management of patients. There should be a low threshold for radical surgery due to the high local recurrence and metastatic rates. Through this case, we highlight the need for a more effective treatment strategy to improve clinical outcomes.


A cystic mass that rapidly increases in volume in a dangerous anatomical region, notably the supraclavicular region, which contains important neurovascular elements, must be considered at high risk. A vascular study is mandatory before biopsy. Neoadjuvant chemotherapy can be a treatment option in high‐risk synovial sarcoma. A cystic mass that rapidly increases in volume in a dangerous anatomical region, notably the supraclavicular region, which contains important neurovascular elements, must be considered at high risk. A vascular study is mandatory before biopsy. Neoadjuvant chemotherapy can be a treatment option in high‐risk synovial sarcoma. There were no symptoms of fever, weight loss, dysphagia, or dyspnea.Physical examination revealed a large (9 × 7 cm), elastic, nontender, and nonpulsatile mass located in the right supraclavicular region of the neck Figure . On laryCT scan showed a voluminous solido‐cystic tumor of the right supraclavicular region with predominantly fluid component density 23 HU) and spontaneously hyperdense zones (54 HU). This mass enhances slightly after injection of contrast medium in a heterogeneous manner. It measures 9.4 × 7.7 cm and extends over 9 cm. The tumor arrives down to the 5th right intercostal space. At the top, it comes into contact with the lateral muscles of the neck. We note a laminar appearance of the axillary vascular pedicle and infiltration of adjacent fat. In addition to bilateral cervical and right axillary adenopathies, some of which were necrotic. The right axillary adenopathy is the largest measuring 14.8 × 8.5 mm .After five courses of chemotherapy, an angio‐MRI was performed. It showed 42% reduction in tumor size. The tumor came into contact with the subclavicular vessels without invading them and passed above the axilla. No feeder vessel was identified; therefore, embolization was not performed.The patient was staged II A  Figures  and 7. TAdjuvant radiation therapy to the neck was administered. The patient was disease free until her six‐month follow‐up with no evidence of local recurrence or distant metastasis.3Synovial sarcoma (SS) is a mesenchymal malignancy that is termed SS since histological appearance is similar to that of the synovium. However, SS rarely exhibits a synovial structure and is considered to originate from pluripotent mesenchymal cells.The diagnosis of SS is made on the basis of histopathological appearance, immunochemistry, and cytogenetic analysis which proved valuable in confirming morphological diagnosis.Interestingly, SS shows a considerable morphologic heterogeneity in which distinct histological subtypes can be distinguished: monophasic, containing spindle‐shaped mesenchymal cells; biphasic, containing both mesenchymal and well‐developed glandular epithelial cells; and the poorly differentiated subtype.SS harbors a highly specific, usually balanced and reciprocal t translocation, in which the SS18 (formerly SYT) gene (at 18q11) fuses with SSX genes: SSX1, SSX2, or rarely SSX4 , leading to the generation of SS18‐SSX fusion oncogenes.Our senior pathologist did not use PCR because the histological and immunochemistry findings were in favor of the diagnosis.Optimal treatment of head and neck synovial sarcoma (HNSS) is controversial and is likely to vary according to the size of the tumor on presentation. The most common treatment of these tumors is surgery, followed by radiotherapy.P = 0.003).In a study of 167 HNSS, Mallen‐St et al reported that 89.8% of patients had surgery and 64.77% had radiotherapy, as radiotherapy improved disease‐specific survival  rates ranging from 40% to 70% with a recurrence rate of 40%.P = 0.008).In a review of 44 patients with HNSS, primary tumor size was a predictor of progression (Histologic subtype has not been known to influence survival in HNSS patient with no difference seen with PFS or OS based on subtype. Moreover, age has never been explicitly identified as a prognostic indicator for SS.The hyper vascularized cystic variant of the tumor and its localization in a dangerous anatomical region in contact with the subclavian vessels and the axillary plexus were the main limitations to surgery.4Despite its rarity, SS should be considered in the differential diagnosis of cystic lesions in the head and neck. The optimal treatment needs to be more clearly elucidated. We cannot draw conclusions on the efficacy of treatment modalities we adopted on the long term as the follow‐up was short. The literature lacks randomized studies that evaluate the efficacy of neoadjuvant chemotherapy in head and neck synovial sarcoma. However, other reports showed effectiveness of neoadjuvant chemotherapy in locally advanced high‐risk synovial sarcoma of the trunk and extremities.The authors have nothing to disclose.SH: involved in data acquisition and manuscript writing; AB and HA: performed manuscript preparation and review; SR: is the main surgeon; RA, MR, and MM: analyzed and interpreted the data; HK, SB, and FM: involved in pathology diagnosis.