Retroperitoneal ganglioneuroma is a rare neuroectodermal tumor with a benign nature. We performed a literature review among 338 studies. We included 9 studies, whose patients underwent CT and/or MRI to characterize a retroperitoneal mass, which was confirmed to be a ganglioneuroma by histologic exam. The most common features of ganglioneuroma are considered to be a solid nature, oval/lobulated shape, and regular margins. The ganglioneuroma shows a progressive late enhancement on CT. On MRI it appears as a hypointense mass in T1W images and with a heterogeneous high-intensity in T2W. The MRI-“whorled sign” is described in the reviewed studies in about 80% of patients. The MRI characterization of a primitive retroperitoneal cystic mass should not exclude a cystic evolution from solid masses, and in the case of paravertebral location, the differential diagnosis algorithm should include the hypothesis of ganglioneuroma. In our case, the MRI features could have oriented towards a neurogenic nature, however, the predominantly cystic-fluid aspect and the considerable longitudinal non-invasive extension between retroperitoneal structures, misled us to a lymphatic malformation. In the literature, it is reported that the cystic presentation can be due to a degeneration of a well-known solid form while maintaining a benign character: the distinguishing malignity character is the revelation of immature cells on histological examination. Ganglioneuroma (GN) represents 0.7–1.6% of primary retroperitoneal solid masses. It is a benign neuroectodermal neoplasm formed by ganglia and Schwann cells into a variable amount of myxoid stroma, ganglion cells, and nerve fibers; immature neuroblastic elements are not included. . GanglioDue to the existence of different histologic forms and the possibility of developing ganglioneuroma in different anatomic sites, the imaging pattern can be variable and many atypical forms exist ,14,15.Cystic degeneration is more frequent in schwannomas, even if it cannot be considered a characteristic feature . This unAccording to the International Neuroblastoma Pathology Classification, Ganglioneuroma-Maturing is composed predominantly of a Schwannian stroma and mature ganglion cells .Furthermore, few studies have specifically focused on the differential diagnosis of retroperitoneal masses, which is necessary to plan the most appropriate diagnostic and treatment strategy. To date, CT and MRI, also due to innovation in 3D reconstructions, can give a high suspicion of malignant behavior and can identify ganglioneuromas through their typical features . CT and We report a case of an 18-year-old female patient, presenting only the symptom of significant recent back pain, who underwent a lumbosacral spine MRI. The survey and the axial T2-weighted MRI sequences show a multiloculated cystic mass, with a maximum axial diameter of about 7.5 cm and longitudinal extension of about 10 cm, in the left retroperitoneal site, with predominantly sub-renal development and a wide contact surface with the ipsilateral psoas muscle, hyperintense in the T2-weighted sequences then predominantly fluid. We also report a pelvic effusion of about 3.5 cm thickness .The exam is inappropriate for a relevant characterization of the lesion; therefore, a dedicated complete abdominal MRI was performed using TSE T2, DUAL, STIR, DWI, and THRIVE sequences, with multiplanar imaging acquisition, before and after administration of paramagnetic contrast agent.The exam confirms the presence of a retroperitoneal fusiform expansive formation in the left anterior pararenal space, approximately 11 × 8 × 7.4 cm; the major longitudinal axis extended from an axial plane passing through the renal hilum and the pancreatic tail. It confirms the wide contact surface with the ipsilateral psoas muscle. The lesion displaces the kidney posteriorly, but it spares the adjacent parenchyma and vascular structures. The MRI also confirmed the multilocular cystic appearance, hypointense in T1-weighted sequences and hyperintense in the T2-weighted ones, and added a more heterogeneous pattern in the medial component .The dynamic contrast study shows late contrast enhancement of the intra-cistic septa and of the wall. In the STIR sequences, there is no suppression of the central component signal, and in the DWI/ADC weighted sequences, there are no evident signs of restricted proton diffusivity .The MRI characteristics point toward the suspicion of a disontogenic cyst or cystic lymphangioma .After laparoscopic surgical exploration, the para-aortic paravertebral retroperitoneal mass has the appearance of a white neoformation with defined margins, and a good cleavage plane that allows complete excision.The histological characterization establishes the diagnosis of ganglioneuroma with maturative aspects, a lesion microscopically constituted by fascicles of spindle cells of Schwannian aspect (S100+ and SOX10+), mixed with scattered ganglion elements (synaptophysin+), also arranged in small clusters, of different degrees of maturation but without significant proliferative and mitotic activity. There are aspects of myxoid degeneration and occasional calcifications and chronic inflammation.The first imaging follow-up was carried out 3 months later. An abdominal MRI exam was performed with the same pre-operative study technique. In the surgical site, no MRI signs of residual disease or abdominal–pelvic lymphadenopathy are documented; only a residual pelvic effusion of 2 cm thickness is reported.We searched the PubMed database for the following keywords: retroperitoneal mass; ganglioneuroma; MRI; CT; diagnosis. We considered the papers published until April 2023. Articles were first filtered and chosen by title and abstract, then a full-text evaluation was performed. Case series with a minimum of four patients were included. Reviews, letters, and case reports were excluded. We included only articles in the English language. Matching our keywords, we found a total of 338 papers. We excluded 329 papers because they did not treat the imaging features of retroperitoneal ganglioneuroma, or they were labeled as reviews or case reports. Three articles were excluded because they were not in the English language. We included nine studies. The included studies performed a pre-operative diagnosis through CT or MRI. The radiological features considered were tumor size , shape, calcification, and enhancement ,22,23. TThe appearance of ganglioneuroma in imaging can be very variable because it depends on the composition of the lesion, in particular the presence of myxoid tissue, collagen fibers, and varied cells ,23,24,25In our case, the paravertebral site of the lesion and the heterogeneous aspect in T2-weighted sequences could have oriented towards a neurogenic nature, however, the predominantly cystic or fluid aspect and the considerable longitudinal non-invasive extension between retroperitoneal structures mislead to a lymphatic malformation. In the literature, it is reported that a ganglioneuroma could show cystic presentation due to the degeneration of a well-known solid form while maintaining a benign character: the distinguishing malignity character is the revelation of immature cells on histological examination .The differential diagnosis is still changing and includes neuroblastoma and ganglioneuroblastoma from the same cell line, ganglion cells, but also schwannoma, lymphangioma, lymphoma, or dysontogenic cysts ,23,24,25The malignant tumors, ganglioneuroblastoma, and gangliobastoma, often develop in children under 2 years old ,21. TheySchwannoma is the most common retroperitoneal tumor. It originates from the nerve sheath and it often has a round shape rather than the oval or elongated shape of ganglioneuroma. It may develop cystic degeneration or calcifications. It can show a different behavior from ganglioneuroma in T2-weighted images, with a central zone of low intensity ,25,26.Lymphangioma is more common in males and rarely presents enhancement, and it often appears as unilocular or multilocular thin-walled cysts ,25,26.Retroperitoneal mass MRI characterization requires a differential diagnosis algorithm that first excludes the most frequent histotypes, such as lymphomas, representing about 1/3 of retroperitoneal lesions, and more expansive and infiltrating sarcomas. The most frequent solid type of lesions should also be excluded: well-differentiated liposarcoma, whose distinctive MRI feature is the adipocytic component, and leiomyosarcoma, whose distinctive feature is the vascular invasiveness .On T2-weighted sequences, a lesion showing peripherical hyperintensity and central hypointensity is suspicious for a neurogenic origin, especially in paravertebral localization. Moreover, vascular enhancement is increased in the paraganglioma and reduced in the presence of a myxoid matrix.Finally, cystic lesions, especially if they are longitudinally extended and without invasion of nearby structures, are reliable in the first hypothesis of lymphatic malformations ,30.However, the absence of neuroblasts on histological examination is the only certain exclusion element for a malignant histotype, such as ganglioneuroblastoma or neuroblastoma ,31.Surgical resection of benign histotypes such as ganglioneuroma is the only effective therapy, even if it may be complicated by the involvement of surrounding structures ,33.The Transatlantic Australasian Retroperitoneal Sarcoma Working Group conducted a retrospective study on 328 patients affected by abdominopelvic and retroperitoneal ganglioneuroma. Most of the patients (64.6%) underwent surgery, while 35.4 underwent active surveillance. In the follow-up, the malignant transformation was rare, but three cases out of 328 patients (0.9%) evolved into neuroblastoma. However, the patients who underwent active surveillance had stable disease in more than 90% of cases. In a 3-years follow-up, 84.4% of patients who had undergone surgery were disease-free. Recurrence was considered as rare as malignant transformation, occurring in only four patients (1.9%) .In the last 20 years, minimally invasive surgery has been affirmed in general surgery, almost becoming the standard of care for benign but also for oncological diseases ,34,35.In this field, several studies have affirmed that minimally invasive surgery provides a shorter length of stay and a decrease in complications ,37,38. ICurrently, surgical procedures for retroperitoneal mass, and adrenal tumors above all, are safe and diffuse, and new technologies have been developed ,43,44,45Artificial intelligence has already been tested on retroperitoneal masses to improve diagnosis, prognosis, and management of treatment strategies ,47,48,49Concerning differential diagnosis, machine learning has been tested in a few studies to differentiate neuroblastoma from other tumors with a lower malignancy, like ganglioneuroma and ganglioneuroblastoma ,53,54.Due to the complexity of the anatomy of the retroperitoneum, AI also aims to help clinicians to perform CT-guided biopsies. This technique aims to avoid any damage to vascular structures ,56.As for other tumors, AI will be fundamental in recognizing the number of undifferentiated cells can be a key point in diagnosis and treatment and even the improvement of the standard technologies available .Further AI studies will focus on achieving an accurate diagnosis through non-invasive exam, avoiding sedation for children during MRI exams, as ultrasound, or with short MRI protocol improved by artificial intelligence software.This case reminds us that MRI characterization of a primitive retroperitoneal cystic mass should not exclude the occurrence of an atypical cystic presentation of benign tumors as ganglioneuroma or schwannoma; in the case of ganglioneuroma, the first suspicion sign should be the paravertebral localization.


Mature cystic teratomas are common benign ovarian tumors. They usually occur in young women, less than 40 years old. Our case report concerns a patient of perimenopausal age who came to the hospital complaining about mild abdominal pain, fever below 37.8°C, and diarrhea. The patient had an intrauterine contraceptive device inserted. Based on the clinical findings and imaging, a possible diagnosis of pelvic inflammatory disease was set, and intravenous administration of broad-spectrum antibiotics started immediately. The decision for performing laparotomy was taken after the fact that the clinical condition and blood tests of the patient had shown no improvement. Intraoperatively, the presence of a large twisted ovarian mass with signs of total necrosis due to adnexal torsion was detected. A histological examination of the surgical specimen confirmed the diagnosis of mature cystic teratoma in the right ovary. The postoperative course was uneventful. The presentation of the case follows a brief literature review of this rare medical condition regarding the diagnostic and therapeutic approach of these patients. Teratomas are germ cell tumors. In their typical form, they consist mainly of well-differentiated tissues derived from the endoderm, mesoderm, and ectoderm. Teratomas can be mature cystic which are benign, or they can be immature and characterized by malignant potential . The firThe mature cystic teratoma of the ovaries or dermoid cyst (synonym) is estimated to account for 10-20% of all ovarian neoplasms. Mature cystic ovarian teratomas in the majority of cases (more than 80%) occur in women of reproductive age, especially in women under 40 years of age . These aWith the occasion of the present case report, we must emphasize the difficulties that may arise in the differential diagnosis between torsion of mature cystic ovarian teratoma and pelvic inflammatory disease. Furthermore, we must underline the great importance of the early diagnosis and treatment of mature cystic ovarian teratomas complicated with complete adnexal torsion, especially when they concern young patients who wish to maintain the integrity of the female reproductive tract and achieve future pregnancy.A 49-year-old patient came to the emergency department of our hospital complaining about abdominal pain accompanied by a low fever (below 37.8°C). The onset of symptoms was five days ago. The location of the pain was in the lower abdomen, with mild intensity, without symptoms of peritoneal irritation. The patient did not report vomiting. She reported three bouts of diarrhea in the past two days. Her body temperature was 37.7°C, and her blood pressure and pulse rate were normal. The patient had three vaginal deliveries in her obstetric history, and her personal medical history was clear. She had an intrauterine contraceptive device placed four years ago and was removed during the gynecological examination and sent for culture. During the gynecological examination, a painful pelvic mass was found. Transvaginal ultrasound revealed a large inhomogeneous lesion of roughly 95x80 mm behind the uterus and in the pouch of Douglas, probably originating from the right adnexa Figure . The lefThe decision was made to admit the patient to our clinic and immediately start intravenous administration of broad-spectrum antibiotics to treat pelvic inflammatory disease. CT findings showed an enlarged edematous image of the right fallopian tube with opacification of the right ovary and a normal left ovary with the presence of small cystic follicles. More specifically, between the rectosigmoid and the uterus in the rectouterine pouch of Douglas, the presence of a large demarcated fluid collection with thick hyperintense walls and the presence of numerous septations in the pelvis around the fluid collection were depicted. The above imaging findings were probably attributed to an encapsulated pelvic inflammatory fluid collection that originated from the right adnexa Figure .The fact that non-improvement was made in the clinical condition and blood tests of our patient Table  48 hoursIntraoperatively, the presence of a large twisted ovarian mass was found, originating from the right ovary and not adhering to the adjacent tissues. The mass had distinct signs of total necrosis due to ipsilateral adnexal torsion Figure .After detorsion of the twisted ovarian mass and the reveal of necrotic, non-viable tissues, adnexectomy was performed. Histological examination of the surgical specimen confirmed the diagnosis of mature cystic ovarian teratoma Figures , 5.On microscopic examination, a cyst with squamous epithelium, increased vascularity and edema, and sebaceous glands and hair follicles was observed. Adipose tissue and cerebral tissue were also observed. Immature tissue or malignancy was not observed. On the fourth postoperative day, the patient was discharged from the clinic with a consultation for re-examination at the gynecology outpatient clinic of our hospital.Clinical diagnosis of mature cystic teratoma of the ovary is usually difficult. In most cases, mature cystic ovarian teratomas are asymptomatic. Although in ovarian torsion abdominal pain can have many variables, acute abdominal pain followed by vomiting may be associated with rupture of the ovarian mass or, more commonly, torsion of the ovarian pedicle . In our The significance of imaging in the evaluation of ovarian masses and adnexal torsion remains determinant. On ultrasound, the mature cystic ovarian teratomas appear as a simple cystic mass without solid components up to a complex solid cystic mass with typical imaging features of intratumoral fat components forms . CT and Furthermore, Doppler imaging of the pelvis is an important complementary ultrasound technique in the preoperative diagnostic approach of mature cystic teratoma after torsion of the ovarian vascular pedicle. The unilateral significant increase in the ovarian volume due to localized edema caused by venous stasis, the coexisting mass within the affected ovary, the presence of various concentrations of free fluid in the pouch of Douglas, and the lack of arterial or venous flow in the affected adnexa significantly increase the probability of adnexal torsion. However, it is useful to underline that the presence of flow on color Doppler ultrasonography cannot exclude adnexal torsion because it is possible to have torsion of the vascular pedicle with a viable ovary .Preoperative differential diagnosis between pelvic inflammatory disease and adnexal torsion with mature cystic ovarian teratoma in our patient was challenging. Rare cases of malignant transformation of mature cystic ovarian teratoma have been described in the literature, in which abdominal pain and vomiting were incorrectly attributed to adnexal torsion ,14. In aThe treatment is surgical. Torsion of mature cystic ovarian teratoma usually concerns patients of reproductive age who wish to preserve fertility and more frequently occurs in premenopausal women, but it can also happen to women of postmenopausal age. This makes the minimally invasive approach the optimal treatment option for these patients . Early dCystectomy is considered to be the treatment of choice in young women who wish to preserve ovarian function . ConfirmAdnexectomy  and hysterectomy, depending on the patient’s age, may be considered necessary in cases of complete adnexal torsion, making the ovary and fallopian tube non-viable . The lapTorsion of mature cystic ovarian teratoma can be misdiagnosed as a pelvic inflammatory disease. Adnexal torsion, particularly when accompanied by atypical clinical symptoms and imaging findings, can create a serious differential diagnostic challenge and significantly delay correct diagnosis. Early diagnosis and treatment should be a primary concern of modern obstetricians and gynaecologists, especially when it comes to young patients who wish to preserve fertility and achieve future pregnancy.


Acquired digital fibrokeratoma is a rare, benign tumor that mostly occurs on the fingers and toes and may appear to be a supernumerary rudimentary digit. It generally affects adult men and appears as a dome-shaped papule although it can also be elongated or pedunculated. Trauma is believed to be a triggering factor in some cases. We report a male patient with an acquired digital fibrokeratoma on a finger, shaped like a cutaneous horn, and a history of minimal repeated trauma and spontaneous remissions not previously described in the literature. Acquired digital fibrokeratoma (ADFK) is a benign tumor that occurs on the fingers or toes and occasionally on the palms and soles, usually in adults .Clinically, ADFK typically presents as a small, single, skin-colored papule on the finger or toe. It is generally surrounded by a collar of slightly raised skin called the “fossa”, and is usually asymptomatic .Histopathologically, ADFK shows an epithelium with marked orthokeratosis and acanthosis. The network of interpapillary ridges appears thickened and generally branched. The enveloped dermis is composed of a thick bundle of interwoven collagen, which is oriented parallel to the longitudinal axis of the lesion. Elastic fibers are thin and few, if not completely absent, and the tumor tends to be highly vascularized. Curative treatment consists of complete surgical excision .A 47-year-old male patient, a welder by trade, presented approximately 15 years ago with a cutaneous tumor lesion on the lateral aspect of the second phalanx of the right middle finger. The lesion began as a firm, asymptomatic papule, which the patient initially traumatized repeatedly with the intention of removing it. The tumor grew and, although asymptomatic, it bothered him at work, for which it was necessary to wear gloves. He reports that this lesion has presented multiple subtotal spontaneous remissions, mainly in winter, leaving a papule from which the tumor re-emerges.Upon examination, the lesion presented like a 1 cm long cutaneous horn, emerging from a hyperkeratotic collarette  is classically associated with tuberous sclerosis; these types of tumors can be differentiated by their histopathological characteristics. Nonpigmented eccrine poroma can be differentiated by its histopathological characteristics on both their pigmented and non-pigmented variants: Pyogenic granuloma, which has a characteristic history of rapid growth rate, and a friable surface that can easily bleed with trauma, also has several dermoscopic characteristics. Keratoacanthoma has dermoscopic differences and histopathological characteristics not found in ADFKs. Cutaneous neurofibroma requires histopathological examination, usually with immunohistochemical stains, to diagnose. Enchondroma can be differentiated by its content's consistency, and by histopathological examination. Dermatofibroma has several dermoscopic characteristics and can be differentiated by histopathological examination. Aggressive digital papillary adenocarcinoma can only be differentiated by histopathological examination with immunohistochemical stains .To date, there have been no reported cases of spontaneous remission of ADFKs, but surgical excision is curative and recurrence is rare .The case we present coincides both clinically and histopathologically with the first descriptions of acquired digital fibrokeratoma. However, due to the varied locations reported, not always acral, we believe the term "acquired fibrokeratoma" to be more accurate to describe this type of tumor in other locations.We present this case to highlight the patient's history, who had a clear history of multiple traumas, and an unusual clinical course, with spontaneous remissions followed by new growth of the tumor. Both spontaneous remission and spontaneous recurrence have not been previously described in the literature.


Liposarcomas are a subtype of soft tissue sarcomas arising from lipoblasts, a mesenchymal cell lineage that commonly arises from deep tissues of the body. Though these are the most common subtypes, their early diagnosis still remains a challenge due to their varied presentation as a soft benign appearing growth. The pleomorphic variant has complex management due to its high recurrence rate and resistance to chemoradiation. Scrotal liposarcomas have been reported. But in the present case, a 69-year-old male who presented with a pedunculated swelling in the left groin mimicking a left hemi-scrotal swelling. It was a left para-scrotal pleomorphic liposarcoma which was totally extra-scrotal, and not related to the spermatic cord or testes. So, this is a rare case with a review of the literature presented here. Soft tissue sarcomas account for <1% of all solid malignancies of adulthood. The World Health Organization (WHO) has further classified these soft tissue sarcomas into 70 subtypes, of which liposarcoma is the most commonly detected subtype . Common In literature, intra-scrotal and para-testicular liposarcomas, spermatic cord liposarcomas have been reported. Para-scrotal which are totally extra-scrotal swellings or liposarcomas, not originating from the spermatic cord are rare.In the present case, the patient developed deep perineal fat tissue liposarcoma, and the patient presented as left scrotal mass but not from the scrotum; it was para-scrotal appearing like the scrotum, and so labeled as pseudo-scrotum. It was a left para-scrotal low-grade pleomorphic liposarcoma.A 69-year-old male presented with complaints of a painless left scrotal swelling since 15 years which was progressively enlarging since 6 months of admission. On general examination, the vital parameters were within normal limits. Detailed physical examination revealed an enlarged and pedunculated left para-scrotal swelling with a soft to firm vague mass within. The left testis was displaced into the right hemi-scrotum, giving the appearance of scrotal swelling Figure . ClinicaA contrast-enhanced computed tomography (CECT) scan of the pelvis was done which reported a pedunculated lesion with predominant fat density measuring 13.4 cm x 5.8 cm arising from the perineal region between the left scrotum and medial aspect of the left thigh. Its superior extent was broad-based extending into the whole anteroposterior perineum and was in continuity with the fat of the left inguinal, medial aspect of the gluteal region. Soft tissue densities and calcifications were noted Figure . The lefUltrasound-guided core biopsy was performed which was reported as normal adipose tissue cells with no atypia. The patient then underwent wide local excision of the tumor through an incision on the lateral aspect of the left hemi-scrotum Figure .A gross examination of the tumor revealed a firm capsulated tumor with a yellowish-white firm necrotic center with the surrounding adipose tissue Figure .Histopathological analysis of the specimen showed R0 resection margins with low-grade pleomorphic lipoblasts and interspersed spindle cells, suggestive of a pleomorphic liposarcoma. The mitotic index was 4 mitosis/high power field, hence was reported to be a low-grade tumor Figure . The post-operative period was uneventful. Post-procedure radiotherapy was given. A CECT pelvis was repeated at 6 months which showed no recurrence. Since then, the patient has been under regular follow-up and there has been no recurrence.Lipoma is known as universal swelling as it can occur at anywhere in the body. Prolonged lipomas may become malignant leading to liposarcoma. Pleomorphic liposarcoma is the rarest variant of liposarcoma, accounting for <5% of all cases . Its incIn literature, intra-scrotal and peri-testicular or para-testicular (inside scrotum) liposarcomas have been reported. But para-scrotal which was outside the scrotum and bigger than the scrotum, low-grade pleomorphic liposarcoma has not been reported in the literature.Accessory to clinical examination is CECT, magnetic resonance imaging (MRI), and biopsy of the lesion. The radiological investigations help in assessing the exact extent of the tumor, presence of calcifications, proximity of involvement of vital structures such as vessels, bowel, etc. On histopathological examination, these tumors show mononucleate/multinucleated giant cells, bizarre cells, and pleomorphic lipoblasts. Pleomorphic lipoblasts have hyperchromic nuclei, multiple nuclei, and univacuolated/multivacuolated cytoplasm .These pleomorphic lipoblasts form only 10% of the tumor mass, hence a negative pre-operative biopsy report can often be misleading as happened with the present case . TherefoWide local excision of superficial pleomorphic liposarcoma is the treatment of choice . EnsurinAdjuvant radiotherapy (RT) should be administered to manage micro-metastasis and in R1 resection cases. RT has been proven to increase the disease-free survival (DFS) period . Wan et Old age, large tumors (>10 cm), presence of high mitotic index (>10 mitosis/hpf), positive surgical margins, and deep soft tissue sarcomas have been proven to be worse prognostic indicators . SuperfiIn literature, many cases with para-testicular liposarcomas as scrotal swellings have been reported. The gold standard treatment for such tumors is radical orchidectomy followed by a multimodal approach including radiotherapy and chemotherapy ,9.In the present case, the patient had swelling in the para-scrotal region over 15 years and it started growing progressively leading to swelling bigger than the scrotum. On investigations, we suspected malignant conversion of the lipoma to liposarcoma which was confirmed postoperatively with histopathological report. A high index of suspicion is essential for such conversion of benign lipomatous swelling to liposarcoma. Patients with multiple lipomatoses should be primed for the possibility of such complications. Wide local excision is a must and can be followed by adjuvant chemoradiation with bad prognostic markers mentioned above.A high index of suspicion is essential to diagnose liposarcomas as they present clinically as benign growths. The decision to perform a radical surgery should be considered when there is a clinico-radiological suspicion of a neoplastic growth as the pre-operative biopsy may be misleading. The treatment for low-grade tumors is wide local excision alone. Adjuvant radiotherapy for large tumors (>10 cm) has been known to increase the disease-free survival period. Regular follow up required to diagnose recurrence at the earliest.


Schwannomas are benign peripheral nerve sheath tumors typically found in the neck, flexor surfaces of the extremities, mediastinum, posterior spinal roots, cerebellopontine angle, and retroperitoneum. Pleural schwannomas are a type of neoplasm that arises from autonomic nerve fiber sheaths in the pleura and rarely originate in the thoracic cavity. These schwannomas tend to be asymptomatic, benign, and slow-growing neoplasms. Although pleural schwannomas commonly occur in males, our report highlights a unique presentation of a pleural schwannoma presenting as musculoskeletal-type chest pain in an adult female. Our patient's diagnosis of pleural schwannoma was supported after X-Ray, Computed Tomography (CT) Scan, and Positron Emission Tomography (PET) Scan imaging was complete. All imagining and immunohistochemical staining yielded pleural schwannoma as the final diagnosis. We aim to bring awareness to the necessity of imaging and histopathological staining in atypical clinical cases of pleural schwannoma. Our novel case highlights pleural schwannoma as a differential diagnosis for patients with intermittent, musculoskeletal-type chest pain. Schwannomas are rare tumors from Schwann cells. They commonly arise within the skull base, cerebellopontine angle, and posterior spinal roots . HoweverA 33-year-old female presented to her primary care complaining of intermittent left musculoskeletal-type chest pain she had been having for a few months. She was sent to radiology, where she underwent an X-ray and computed tomography (CT) imaging. This revealed a left-sided pleural-based nodule in the lateral mid-thorax area adjacent to her third rib. The nodule was smooth-edged and measured 11x19mm. The patient was referred to pulmonology. On clinical assessment, she reported no weight loss, fever, cough, or other symptoms besides intermittent pain, which she rated 3/10. Her physical exam was significant for tenderness on palpation of her left upper rib cage. She had no smoking history or history of exposure to chemicals or radiation, and her family history was positive for lung cancer in more than 1 family member. Her symptoms and imaging raised suspicion of sarcoma of sorts. Therefore, Positron Emission Tomography (PET) scan and bronchoscopy with a CT-guided core biopsy were ordered.The patient's PET scan Figure  showed mThe patient underwent robotic wedge resection via thoracotomy. The laterally placed chest wall schwannoma was seen protruding into the pleural space. The entire lesion was excised with some intercostal muscle margins in toto. X-ray images pre and post-surgery are seen in Figures The patient recovered well from surgery with no complications. She was seen on follow-up a month later. She reported no recurrence of her original pain, had no further symptoms, and was cleared to be seen as she needed in the future.Pleural schwannomas are one classification of peripheral nerve sheath tumors (PNST) that are benign neoplasms rarely found in the thoracic cavity and even less common in the pulmonary pleura. PNSTs are usually located in the posterior mediastinum and are rarely found in other mediastinal compartments . A studyThough other PSNTs can initially present with symptoms of pain, palpable/visible mass, or nerve palsy , pleuralThere are several possible explanations for the pain. First, the interaction of the pleural schwannoma with intercostal nerves causes the patient to have pain. Sughrue et al.  explaineDiagnosing pleural schwannomas is challenging, and radiological imaging can produce ambiguous results. Nonetheless, CT imaging showing solid, solitary, and well-defined pleural tumors should prompt adding schwannoma into the differential diagnosis . The defA case report published in 2014 illustrated that less than 20 cases had been reported in the medical literature . HoweverSchwannomas are a rare type of peripheral nerve sheath tumor. While most reside in the posterior aspects of the human body, such as the posterior mediastinum and retroperitoneum, pleural schwannomas have begun to be reported due to the increased collection of nerves in those regions as coincidental findings on radiologic imaging. The presentation of most pleural schwannomas is asymptomatic, making pinpointing this disease difficult through symptoms alone. Our patient presented with a few months of intermittent left-sided musculoskeletal-type pain that compelled her to present to a pulmonologist for evaluation. The peripheral pleural mass was identified and excised. To further identify this tumor accurately, physicians are encouraged to focus more on pulmonary and mediastinal sources of pain when a patient presents with chest pain, as many are trained and used to emphasizing cardiac causes. If recognized early, the treatment options and prognosis are excellent, prompting us as a healthcare community to evaluate all causes of chronic chest pain with an open mind and consider all options.


Case Description. A 31-year-old G2P0111 PPD 7 presented with intra-abdominal abscesses of unknown source. She was treated with broad-spectrum antibiotics, image-guided percutaneous (IR) drainage of the largest abscess, and surgical exploration with debridement. During surgery, she was diagnosed with spontaneous uterine rupture. The uterine defect was successfully repaired, and she was able to be successfully managed with fertility-sparing treatment. The patient ultimately did not require a hysterectomy. The final pathology was consistent with pyomyoma.  Pyomyomas are an infrequent complication of uterine fibroids and, in extremely rare cases, the cause of spontaneous uterine rupture. A few documented cases were managed conservatively with oral antibiotics and CT-guided drainage or myomectomy with fertility preserved. However, treatment more frequently involves IV antibiotics and a hysterectomy.  In a majority of cases, pyomyoma treatment requires a hysterectomy, and fertility is unable to be preserved. However, conservative management with IV antibiotics, IR drainage, and surgical debridement could be a fertility-preserving approach to the treatment of pyomyomas. Uterine leiomyomas, or fibroids, are benign monoclonal tumors that develop from the myometrium's smooth muscle cells and fibroblasts. They are very common in reproductive-age women and are quite common during pregnancy . The preUterine fibroids during pregnancy are typically asymptomatic. When they do cause symptoms, pain is the most frequent complaint. Uterine fibroids during pregnancy cause complications in roughly 2% of cases and include degeneration, preterm labor and birth, preterm premature rupture of membranes (PPROM), and pyomyoma , 4.A pyomyoma, or suppurative leiomyoma, results when a fibroid becomes infected and necrotic. Typically, the fibroid becomes infarcted during pregnancy or in the postpartum period due to hemorrhage or decreased blood flow, leading to necrosis and infection. The infection then spreads from the fibroid either directly to adjacent structures or by lymphatic or hematogenous spread –6. PreviThe patient was a 31-year-old G2P0111 who presented to her primary obstetrician 7 days after an uncomplicated vaginal delivery with a complaint of lower abdominal pain. Her pregnancy had been complicated by iron deficiency anemia, fibroid uterus , and a h3 μL, thrombocytosis with platelets of 703 × 103 μL, and hypokalemia with potassium at 3.0 mmol/L. Due to these collective findings, the patient was admitted for management with intravenous (IV) antibiotics, and interventional radiology (IR) was consulted. The patient was started on IV ampicillin and sulbactam, gentamicin, and clindamycin. A Jackson-Pratt (JP) drain was placed in the right lower abdominal quadrant by IR under CT guidance, with drainage of 900 cc of yellow, purulent fluid. The sample was submitted for culture and cytology. Infectious disease was also consulted, and the IV clindamycin was subsequently discontinued.Initial computed tomography (CT) of the abdomen and pelvis was significant for multiple, large loculated fluid-filled collections within the peritoneum measuring 21.4 cm by 21.1 cm by 16.5 cm with a separate fluid collection in the cul-de-sac measuring 7.0 cm by 5.0 cm by 8.9 cm ; previouOn the third hospital day, blood cultures had been negative for approximately 36 hours, and culture of the fluid drained grew Fusobacterium nucleatum. Her leukocytosis reached an apex of 22.7. Infectious disease escalated her antibiotics from gentamicin and ampicillin/sulbactam to piperacillin and tazobactam.During the following 3 days, her WBC count decreased and there was minimal output from the JP drain. Repeat imaging was ordered for evaluation of the abscess prior to removal of the JP drain. Despite improvement in her WBC count and JP drain output, repeat CT imaging of the pelvis and abdomen showed only a small decrease in the fluid collected from the right lower quadrant near the JP drain. Overall, the fluid collection remained large, measuring 17.8 cm by 8.5 cm by 14.2 cm with the fluid collection in the cul-de-sac measuring 6.1 cm by 4.9 cm by 7.5 cm . IR was The patient was consented and underwent a diagnostic laparoscopy that was converted to an exploratory laparotomy. Upon initial visualization of the intra-abdominal cavity, extensive adhesions were noted, and general surgery was consulted intraoperatively. However, despite lysis of adhesions, they were unable to obtain adequate visualization of the pelvis. The decision was made to convert to laparotomy. During lysis of adhesions, multiple small bowel serosal tears were found. A majority of the serosal tears were able to be repaired, but two areas were noted to be too significant for repair. Ultimately, the patient required a small bowel resection. The surgeon also performed drainage and debridement of intra-abdominal/pelvic abscess and fluid.After a bowel resection and extensive irrigation of the abdominal cavity, the pelvic cavity was able to be inspected. The uterus was localized and was noted to have a 3 × 3 cm defect extending more than 50% into the myometrium in the left anterior fundus. This was the location of the previously described fibroid, which was no longer identifiable. This finding was concerning for spontaneous uterine rupture. The uterine rupture was considered to be the likely source of the abscess formation. The uterine defect was subsequently closed in an interrupted fashion using 1 chromic suture. A sample of the debridement was obtained and sent to pathology for evaluation. After further exploration, no other sources for abscess formation were identified. Two 19 FR Blake drains were placed, one located posterior to the uterus and the other within the left gutter, in close proximity to the uterine repair.The Blake drains remained in place until POD 4, and she had her diet advanced as tolerated, from clear liquids to a regular diet by the general surgery team. The patient was transitioned to p.o. amoxicillin by infectious disease and was discharged on postop day 5 in stable condition. She was instructed to complete a 5-day course of amoxicillin and to follow up in one week with both the obstetric and general surgery teams. After discharge, her postoperative recovery was uneventful, and she did not require any further hospital admissions or reoperations.The pathology report of the surgical debridement noted severe acute and chronic inflammation and necrotic tissue with adjacent fibrous/smooth muscle tissue showing changes consistent with infarct. The overall architecture of the fibrous/smooth muscle tissue was consistent with the uterine wall. These findings confirmed our suspicion that the patient experienced a fibroid infarction during the postpartum period, which created a pyomyoma that lead to a spontaneous uterine rupture and the formation of intra-abdominal abscesses.Pyomyoma is an extremely rare complication of uterine fibroids during pregnancy or the postpartum period. One study reported that only 13 cases of pyomyoma related to pregnancy have been documented since 1945 . While pManagement of pyomyoma includes IV antibiotics and, in most cases, surgical management. A majority of patients require a hysterectomy and fertility is unable to be preserved. There have been a few documented cases where pyomyoma has been managed more conservatively with antibiotics and CT-guided drainage or myomectomy, in order to preserve fertility . SpecifiWhile pyomyomas are extremely rare, they are associated with high morbidity and mortality. Due to their rarity, a high index of suspicion must be present from physicians in order to achieve a correct diagnosis and provide prompt treatment. Findings that should raise suspicion for pyomyoma would be signs of sepsis and pelvic pain in the setting of a fibroid uterus. Ultrasound and CT imaging can help guide the diagnosis, but it is ultimately a surgical diagnosis. Conservative management in order to preserve fertility is reasonable to consider and can be successful, as demonstrated by our case.


Background: This retrospective case series study aims to demonstrate a salvage technique for the treatment of carotid blow-out syndrome (CBS) in irradiated head and neck cancer patients with a vessel-depleted neck. Methods: Between October 2017 and October 2021, two patients (N = 2) with CBS were treated at our institution in a multidisciplinary approach together with the Department of Vascular Surgery. Patients were characterized based on diagnoses, treatment procedures, and the subsequent postoperative course. Results: Surgical emergency intervention was performed in both cases. The transition zone from the common carotid artery (CCA) to the internal carotid artery (ICA) was resected and reconstructed with a xenogic (case 1) or autogenic (case 2) interposition (end-to-end anastomosis). To allow reconstruction of the vascular defect, an additional autologous vein graft was anastomosed to the interposition graft in an end-to-side technique, allowing arterial anastomosis for a free microvascular flap without re-clamping of the ICA. Because of the intraoperative ICA reconstruction, none of the patients suffered a neurological deficit. Conclusions: The techniques presented in the form of two case reports allow for acute bleeding control, cerebral perfusion, and the creation of a vascular anastomosis option in the vessel-depleted neck. Carotid blowout syndrome (CBS) is a rare and life-threatening complication in patients treated for head and neck cancer, most commonly with a history of prior surgery and radiotherapy. CBS is the result of vessel wall necrosis that can occur as a result of surgical and adjuvant tumor therapies, chronic inflammation, and fistula.The general incidence of CBS in oncological procedures in the head and neck region is 3–4.5% ,2, whileCarotid artery rupture occurs mainly in the common carotid artery (CCA), near the bifurcation (60–70% of cases). A much smaller proportion also occurs in the internal carotid artery (ICA) . In geneThe main risk factor for the development of CBS is previous radiation therapy after tumor surgery . PatientBased on this, it is understandable that surgical procedures that result in damage to the adventitia increase the likelihood of CBS. Patients who underwent neck dissection surgery showed an eight-fold increased risk of CBS . In partThe general incidence of CBS in re-irradiated patients with tumor recurrence is 0–17%. In these cases, tumor ingrowth into the CCA is an additional risk factor for CBS ,13. In gAnother risk factor is chronic inflammation in the sense of bacterial infection, which leads to thrombosis of the vasa vasorum of the arterial wall and has an increased susceptibility to the negative influence of inflammatory mediators in contaminated wound areas . In addiDue to the extremely high mortality rate of 76%, CBS is a feared complication . In emerThe aim of the present study was to present clinical examples of techniques that can be used for the surgical management of spontaneous and intraoperative CBS. The techniques presented in the form of two case reports allow acute hemorrhage control during permanent cerebral perfusion  and the creation of a vascular anastomosis option in the avascular neck.®, Meierhofer AG, Munich, Germany) and the digital patient files .This interdisciplinary retrospective case series was performed at the Department of Oral and Maxillofacial Surgery in cooperation with the Department of Vascular Surgery (University Clinic Erlangen). Patients were identified by screening the digital clinic documentation system  with infiltration of the mandible. She had previously undergone resection of an OSCC on the left cheek  and a second OSCC in the alveolar bone of the left mandible  L0 V0 Pn0 G2 R0). Postoperative interstitial brachytherapy with up to 50 Gy in 2008/2009 and definitive concurrent radiotherapy  and chemotherapy with 5-FU and cisplatin (10–12/2014) with maintenance chemotherapy with cisplatin (01/2015) were initially performed. In 10/2015, a recurrent tumor of the OSCC in the area of the anterior floor of the mouth and the alveolar crest of the left lower jaw (rpT4a pN0 (0/15) L0 V0 Pn0 G2 infiltration depth 0.5 cm, R0) was resected and reconstructed with a microvascular anastomosed upper arm graft.The present recurrent tumor (rpT4a L0 V0 Pn0 G3 Rx), newly detected in July 2017, was treated with a partial mandibular resection (continuity resection). The mandible was reconstructed with a reconstruction plate  and a free microvascular M. latissimus dorsi flap. The postoperative course was very complicated due to recurrent circulatory disturbances of the flap and cervical wound healing disorders, which were covered by a musculocutaneous anteriolateral thigh flap (ALT).® , as autologous vessel grafts of adequate size were not available.During the postoperative inpatient period, spontaneous erosive bleeding of the left CCA/ICA required emergency surgery. After systemic heparinization and clamping, the ruptured carotid bifurcation was resected, and the ICA was reconstructed with an on-table pericardial tube graft  was placed end-to-side on the pericardium to allow arterial anastomosis of a soft tissue graft. An upper arm flap was used to fill the cervical soft tissue defect resulting from the resection of the necrotic tissue mentioned above .The patient was discharged from the hospital on the 36th day after the operation. After discharge, the patient attended our six-weekly tumor follow-up in our outpatient clinic.A 65-year-old Caucasian male presented to our clinic in February 2021 with a suspected malignancy at the floor of the mouth, a significantly worsened general condition, and a weight loss of 10 kg within a few weeks. The staging CT scan showed a cT4a N2b OSCC on the right floor of the mouth. In March 2021, surgery was performed with a temporary tracheotomy, neck dissection of levels I-III on both sides, and resection of the floor of the mouth and the mandible from the left to right jaw angle. The mandible was reconstructed with a reconstruction plate  in combination with a microvascular latissimus dorsi transplant, as osseous reconstruction was rejected by the patient.Histopathological evaluation revealed a pT4a pN2c (2/43) L0 V0 Pn1 OSCC with bone infiltration of 1.4 cm, G2, R0. Due to the tumor size and lymph node involvement, the postoperative interdisciplinary tumor board recommended adjuvant radiochemotherapy to optimize tumor control. However, the planning CT showed signs of early recurrence, and definitive radiotherapy  and adjuvant chemotherapy were recommended, which were delivered from May 2021 to July 2021 with a total cumulative dose of 70 Gy to the oral floor and lymphatic drainage target volume. The recommended concurrent chemotherapy was rejected by the patient.However, during the course of radiotherapy, a significant intraoral dehiscence with significant exposure of the reconstruction plate was observed, leading to severe aesthetic and functional impairment. Due to the risk of further complications, removal of the reconstruction plate was recommended. The patient has now requested osseous reconstruction of the mandible for future implant-prosthetic rehabilitation. Removal of the reconstruction plate alone, with the risk of permanent tracheostomy due to possible soft tissue collapse, was categorically rejected.Because of the expected intraoperative soft tissue deficit due to previous radiotherapy, extraoral cervical soft tissue reconstruction with a microvascular ALT transplant in combination with a free fibula transplant was planned.The procedure was performed using a “three-team approach”. One team prepared the cervical vessels and removed the osteosynthesis material, while the second and third teams harvested the ALT and the free fibula transplant.The preparation of the neck was extremely difficult due to extreme fibrosis of the soft tissues and significant scarring. Despite careful preparation, a wall weakness of the CCA was observed, leading to a wall defect with massive bleeding (CBS). As direct suture reconstruction was not possible, a vascular surgeon was consulted at once.Systemic heparinization was initiated, followed by preparation of the carotid bifurcation and ligation of the external carotid artery (ECA). The area of perforation, which included parts of the ICA and parts of the CCA, was resected. To ensure cerebral perfusion, an intraluminal shunt system  was placed until an approximately 5 cm long venous segment of the greater saphenous vein was prepared and anastomosed to the ICA and CCA as an arterial interposition (end-to-end). To allow microvascular reconstruction of the cervical soft tissue dehiscence, an additional 2 cm-long vein graft was anastomosed to the interponate in an end-to-side technique, which could be used for arterial anastomosis of the ALT graft. This avoided further cross-clamping of the ICA during microsurgery. Due to the desolate cervical vascular status, the fibular graft was not completely harvested, transferred back to the lower leg, and replanted with osteosynthesis. The postoperative course was uneventful. No neurological deficit was noted. The patient was discharged on postoperative day 13 with a permanent tracheostomy tube. Postoperative angiography of the cervical vessels showed adequate perfusion . PostopeThe aim of this case series was to demonstrate techniques that allow for the management of CBS while at the same time enabling microvascular soft tissue reconstruction of cervical defects using free flaps. Two patient cases are presented.The two cases have in common that the preoperative anatomical and soft tissue situations were extremely challenging. Both cases showed previous operations, wound infection/dehiscence, a previously irradiated neck (cases 1 + 2) and partial microvascular free flap necrosis (case 1). Both cases presented with CBS and cervical soft tissue defects too large for local management while at the same time presenting “vessel depleted necks” with no suitable vessels for microvascular anastomosis.The literature describes different techniques for the handling of mass bleeding resulting from the erosion of the carotid bifurcation. The number of treatment techniques for acute CBS is more limited. Here, vascular ligation represents the ultima ratio, and a series of possible consequences should be considered. The direct neurologic consequences of ligation of the ICA are not predictable, and range from an asymptomatic course to a disabling major stroke .Patients with a high risk for CBS often also show a “vessel depleted neck” due to multiple prior surgical and radiochemical interventions. In the same patient group, as is the case here, a microcvascular flap treatment may be necessary due to the size of the defect. In order to make microvascular tissue transfer possible, individual solutions range from simple interposition grafts to extracorporeal perfusion devices ,22,23,24As the standard vascular network defined by the ECA branches and the jugular vein is inaccessible in those cases, other recipient vessels for microvascular anastomoses have to be chosen.For arterial anastomoses, branches of the subclavian  and axillary arteries  as well as the superficial temporal artery have been described in the literature ,26,27.For venous drainage, the accessory veins of the target artery can be prepared. Another option is to use the cephalic vein because of its reliable drainage, consistent anatomy, long pedicle, and high flow to the low-pressure system .As mentioned above, CBS can occur under unfavorable conditions that require new strategies.In addition to patient survival, the aim is to solve the acute bleeding problem, provide an anastomosis option for microsurgical defect treatment, and allow sufficient cerebral perfusion to prevent neurological damage. All these requirements were met with the two techniques described in this report.In both cases, the choice of vascular graft depended on the presence/location of a suitable donor site, e.g., the presence of a microvascular donor site despite the “vessel depleted neck”.In case 1, a xenograft was used to manufacture a neo-ICA. In case 2, V. saphena magna was harvested by extending the surgical access to the ALT flap. The xenograft could be manufactured in length and diameter as required. The saphenous vein has nearly the same caliber as the ICA, and the length could be varied as required. This way, graft positioning was unproblematic, and no risk of kinking existed.In case 2, a collateral circulation bridging the resected CCA/ICA gap was created using an intraluminal shunt. Installing the shunt takes about 5–8 min when performed by an experienced surgeon. During this period, the CCA as well as the ICA/ECA are clamped. The anastomosis of the additional lateral graft to the interposition graft for subsequent arterial flap anastomosis was performed before re-opening the clamps in order to avoid additional clamping of the interposition graft.With the techniques described, simultaneous management of both acute bleeding (CBS) and soft tissue reconstruction was possible. In the treatment of the CBS, an almost optimal situation for microsurgical transplant anastomosis was constructed. Both patients experienced no further bleeding or neurological complications; cervical soft tissue reconstruction was successful.ICA/CCA reconstruction with an autologous or xenogeneic vascular graft and an additional vascular branch for subsequent microvascular flap anastomosis is an effective method for the combined treatment of CBS and cervical soft tissue defects in patients with a “vessel depleted neck”.


MKKS gene on chromosome 20. Individuals with this condition may have extra fingers or toes, fused labia or undescended testes, and, less commonly, severe heart defects. Diagnosis involves a physical examination and genetic testing, while treatment focuses on symptom management, including surgical intervention if necessary. The prognosis varies depending on the severity of associated complications. In a recent case, a 27-year-old woman with fetal hydrometrocolpos gave birth to a female neonate with extra digits on both hands and feet, fused labia, and a small vaginal opening. The neonate also had a large abdominal cystic mass, and echocardiography revealed a patent foramen ovale. Genetic testing confirmed an MKKS gene mutation, and the hydrometrocolpos required surgical management. Early diagnosis and intervention can improve outcomes for individuals with this syndrome.McKusick-Kaufman syndrome is a rare genetic disorder that affects limb development, genital formation, and heart function. It is caused by mutations in the  MKKS gene [McKusick-Kaufman syndrome (MKS) is an autosomal recessive condition that includes genital malformations, limb abnormalities, and heart defects . The conKKS gene . These sKKS gene . The limKKS gene .A 27-year-old pregnant woman visited the antenatal clinic at 28 weeks of gestation with an obstetric score of G5P4A0. She had no significant past medical or surgical history. An ultrasound scan was conducted which showed a single viable fetus with polydactyly in both hands and a large fluid-filled structure in the fetal pelvis. The amniotic fluid volume was normal, and no other structural abnormalities were detected. A referral was made for a level II ultrasound scan, confirming polydactyly Figure .There were no other anomalies detected, and the fetal heart was normal.Based on the ultrasound findings, fetal hydrometrocolpos was diagnosed, and the patient was informed about the possibility of an underlying genetic disorder. Amniocentesis was offered, but the patient declined. The patient was managed conservatively with regular ultrasound monitoring of fetal growth and amniotic fluid volume.At 39 weeks of gestation, the patient presented in spontaneous labor, and a female neonate was delivered via cesarean section with an APGAR score of eight at one and five minutes. As mentioned above, the patient had a history of four pregnancies and deliveries, with no abortions or any other medical interruptions of pregnancy. Her prenatal history and laboratory reports were normal. At 35 weeks of gestation, an ultrasound showed an abdominal mass and a dilated lateral ventricle of the brain.The postnatal examination showed that the baby was 44 cm long, with a head circumference of 35 cm and a weight of 2.8 kg. The baby had respiratory distress and required 2 L/minute of nasal oxygen. Physical examination revealed the presence of an extra digit on both hands and feet, giving her a total of 12 fingers and 12 toes. The extra digits were well-formed and had functional joints , genital malformations, and heart defects. It was first described in 1964 by Victor A. McKusick and Robert L. Kaufman. MKS is caused by mutations in the The most common limb abnormality observed in MKS is polydactyly, ranging from a small extra digit to a fully formed finger or toe. Genital malformations in females typically involve fused labia and a small vaginal opening, while males may have undescended testes. Heart defects, although less common, can be severe and may require surgical intervention .We can contemplate a differential diagnosis of MKS and Bardet-Biedl syndrome (BBS) because genital abnormalities and limb abnormalities are commonly present in both disease entities . BBS is MKKS gene, which is diagnostic for MKS [Diagnosis of MKS is typically based on physical examination and genetic testing. Individuals with MKS often exhibit multiple physical abnormalities, including polydactyly, genital malformations, and heart defects. Genetic testing can confirm the presence of mutations in the  for MKS .Management of MKS involves a multidisciplinary approach, with treatment aimed at managing the specific symptoms of the condition. Surgery may be recommended to correct genital malformations and prevent long-term complications. Individuals with congenital heart defects may require cardiac surgery to correct the abnormalities .The prognosis for individuals with MKS varies depending on the severity of their symptoms and the presence of associated complications. Early diagnosis and intervention can improve outcomes for affected individuals, highlighting the importance of timely identification and collaborative treatment. Recent advances in medical technology and research have shown promise in improving the management of MKS. For example, targeted therapies and gene therapies have the potential to address the underlying genetic mutations that cause the condition .In essence, this case offers a thorough examination of MKS and stresses the significance of genetic testing which can aid in early diagnosis and collaborative treatment. Because this is a rare condition, very few cases have been published and very few studies have been done. It would be helpful if more articles or cases would have been discussed to gain more information and emphasize the importance of further investigation and effective treatments. Additionally, the study highlights the possible benefits of novel treatments and the necessity of further investigation and medical attention to enhance the quality of life for people impacted by this uncommon condition.MKS is an autosomal recessive condition distinguished by limb abnormalities, genital malformations, and heart defects. The prevalence of this disorder is about 1 in 14,000 individuals. The diagnosis is based on genetic testing and physical examination. Treatment requires a multidisciplinary approach aimed at managing specific symptoms. The prognosis varies depending on the severity of the heart defects and other associated complications. Early diagnosis and intervention can improve outcomes for affected individuals. This case report offers a thorough analysis of MKS and emphasizes the importance of further investigation and medical attention to enhance the quality of life for individuals impacted by this infrequent condition.


We describe the first case in literature of malignant mesothelioma of the tunica vaginalis that has shown partial response to systemic immunotherapy (ipilimumab‐nivolumab) post orchiectomy, warranting further investigation in a trial setting.We present a case report of an 80‐year‐old ex‐smoker with a rare diagnosis of metastatic mesothelioma of the tunica vaginalis, managed with immunotherapy. The patient, with no known history of asbestos exposure, presented with a left scrotal mass and pain. Scrotal ultrasound confirmed a large paratesticular mass, and computed tomography (CT) of the chest, abdomen, and pelvis revealed a bilobed mass in the left scrotal compartment without associated inguinal or abdominopelvic lymphadenopathy, and an indeterminate, subcentimeter, bi‐basal subpleural nodules. He underwent a left orchiectomy, and histopathology confirmed the diagnosis of a paratesticular mesothelioma. Postoperatively, the patient had a positron emission tomography (PET) scan showing a new right pleural effusion as well as increasing size of the lobar and pleural nodules bilaterally, all metabolically active and suggestive of progressive metastatic disease. The patient was commenced on ipilimumab and nivolumab immunotherapy, a regimen indicated for malignant pleural mesothelioma; however, the efficacy on paratesticular mesothelioma is not known. After 6 months of treatment, the patient demonstrated a partial response to immunotherapy, with a reduction in the size of known pleural nodules and effusion.Literature review suggests that diagnosis requires a high index of suspicion and patients commonly have metastatic disease at the time of diagnosis. Orchiectomy is a common management modality. However, the role, regimen, and benefits of systemic therapy are unclear, warranting further studies investigating management strategies. His medical and surgical history were significant for prostate cancer in 2000, managed with prostatectomy and active surveillance, hypertension, and paroxysmal atrial fibrillation.3Testicular tumor markers, including serum beta–human chorionic gonadotropin (β‐hCG), alpha–fetoprotein (AFP), and lactate dehydrogenase (LDH) were all within normal limits. Ultrasound examination of the left scrotum revealed a 66 × 26 × 38 mm mixed hyperechoic mass with increased vascularity and was suspicious for a neoplastic process. Computed tomography (CT) scan confirmed the sonographic findings, including a bilobed mass measuring 31 mm Figure  and  26 Subsequently, the patient underwent a left orchiectomy. The macroscopic view of the resected specimen confirmed the presence of a bilobed tumor compressing the testicle Figure . HistopaPostoperatively, the patient underwent positron emission tomography (PET) with 358 MBq 18F‐FDG, and low dose CT scanning, confirming metastatic disease. Focal activity in the left inguinal region was suspicious of residual nodal malignant involvement Figure , both of4Based on these investigations, the large paratesticular mass was diagnosed as a malignant epithelioid mesothelioma of the tunica vaginalis with metastases to the left inguinal node, lung, and pleura.5There is no standard systemic treatment for this rare cancer. However, given the progressive and metastatic nature of the disease and the patient's preference to have systemic treatment, he was offered ipilimumab and nivolumab immunotherapy. This occurred following discussions of potential immune‐related toxicity from a treatment regimen with unclear benefits due to lack of efficacy data among patients with mesothelioma to testis or tunica vaginalis, as these were excluded in the landmark trial CheckMate 743.6The patient presented to hospital with acute onset dyspnea and new oxygen requirement 1 week after cycle1 day1 (C1D1) of ipilimumab and nivolumab immunotherapy. CT scan of the chest demonstrated an increase in the size of the right‐sided pleural effusion since imaging one month ago Figure , indicat7Malignant mesothelioma of the tunica vaginalis is a rare diagnosis and the management strategies are not standardized in clinical guidelines. A literature search on PubMed reveals that there are 119 papers published globally discussing this rare diagnosis. We reviewed 18 of these cases, diagnosed between 1994 and 2022, to highlight common risk factors, propensity for early, recurrent or metastatic disease, poor prognosis and lack of available standard treatment. Analysis of these 18 cases revealed that the mean age of diagnosis is 56 years (range: 14–81), and the disease most commonly manifests with scrotal swelling (89%) and ultrasound confirmed hydrocele (78%). Of the reported risk factors, in this patient cohort, one patient had a background of asbestos exposure, one patient had a smoking history and one patient had trauma history. Thus, our findings corroborate the literature review by Segura‐Gonzalez et al.,n = 12), cytokeratin , vimentin , and epithelioid membrane antigen .When reported, testicular tumor markers were within the normal range for all patients. Ultrasound was the most common initial imaging modality, with 72% (13) of patients receiving sonography input. Of the other imaging modalities, 61% (11) of patients underwent a CT scan (pre or postoperatively), 17% (3) of patients received magnetic resonance imaging (MRI), and 17% (3) of patients underwent PET scans. Histopathology was diagnostic in all of cases. Immunohistochemical markers were reported in 15 cases, and of these there was positivity for calretinin  of patients had metastatic disease, and the majority of patients had nodal involvement, two patients had lung metastasis  and one patient had rectal wall thickening Figure . Due to Following surgery, some patients were managed with multimodal measures. Three patients underwent systemic therapy and the treatment regimen for all of these patients was cisplatin and pemetrexed. These chemotherapy regimens predated the CheckMate 743 trial, which concluded that immunotherapy with ipilimumab and nivolumab demonstrated better survival rates and decreased treatment‐related adverse events in comparison to cisplatin and pemetrexed.n = 3) developed recurrence. The most common site of recurrence was nodal , along with the skin and the lung.Risk factors for poor disease outcome include older age at the time of diagnosis, tumor size greater than 49 mm, tumor necrosis, high mitotic index, angiolymphatic invasion, and biphasic cell type (epithelioid and sarcomatoid components).Considering the rarity of this tumor, a multidisciplinary evaluation with medical, surgical, and radiation oncologists, along with urologists, should be utilized.8In patients who present with a scrotal mass and/or hydrocele, malignant mesothelioma of the tunica vaginalis should be considered as a differential, even in the absence of documented risk factors.Many patients have metastatic disease, most commonly nodal involvement, at the time of presentation.There is no standard of treatment; however, commonly utilized management options include orchiectomy to remove the primary tumor, even in the setting of metastatic disease, plus or minus lymph node dissection.The majority of cases recur after surgery. While patients may require systemic therapy, the treatment regimen is unknown.We describe the first case of metastatic mesothelioma of the tunica vaginalis in literature which has shown partial response to systemic immunotherapy (ipilimumab‐nivolumab) post orchiectomy. This may pose a new treatment option for a rare disease and warrants further investigation in a trial setting.Referral for genomic profiling may provide therapeutic or trial recommendations for this rare disease, and may even uncover a mutation of germline origin.Kritika Mishra: Conceptualization; data curation; formal analysis; investigation; methodology; writing – original draft; writing – review and editing. Shihab Siddiquee: Formal analysis; writing – review and editing. Anna Rachelle Mislang: Conceptualization; methodology; resources; supervision; writing – review and editing.Open access publishing facilitated by Flinders University, as part of the Wiley ‐ Flinders University agreement via the Council of Australian University Librarians.The authors declare no conflicts of interest.Written informed consent was obtained from the patient to publish this report in accordance with the journal's patient consent policy.


A nulliparous woman declined a hysterectomy for her 30 cm fibroid uterus in 2018, recommended for radiological suspicion of sarcomatous change, and instead attempted alternative medical remedies. She re-presented 5 years later; computerized tomography imaging identified a massive leiomyoma measuring 42 × 38 × 50 cm, with a 30 cm fluid-filled pocket, and enduring concern of possible sarcomatous change.She underwent debulking surgery. Intra-operatively, the mass was arising from a stalk at the uterine fundus. It was adherent to the anterior abdominal wall, pelvic side walls, small bowel, large bowel, uterine fundus, and the left ovary, with multiple large feeding vessels. A 10 cm segment of small bowel was excised, and a side-to-side anastomosis created. A total abdominal hysterectomy, bilateral salpingo-oophorectomy, and omentectomy were then completed. The estimated blood loss was 3.5 L; requiring transfusion of 10 units of blood and 4 units of fresh frozen plasma. 8 L of fluid drained from the mass; after removal, total estimated weight of the mass was 45 kg. She was discharged 11 days later, weighing 51 kg, after being admitted at 96 kg. Histology confirmed high-grade leiomyosarcoma .Leiomyosarcomas of the uterus are rare; affecting around 6 per million people per year in the United States . StagingThere have been large leiomyosarcomas reported in the literature; the largest being 57 kg [


Antrochoanal polyp (ACP) is a well-known benign sinonasal polyp first described in the early 20th century. ACP mostly presents as a unilateral mass and is treated solely with surgical excision.We report a rare case of a middle-aged man presenting with nasal obstruction, rhinorrhea, and sleeping disturbances, eventually diagnosed with bilateral ACPs. After confirming the diagnosis with imaging and biopsy studies, the patient was treated conservatively, with marked improvements in his symptoms during 2–3 months of regular follow-ups. A review of the relevant literature regarding the presentation, diagnosis, and outcome of this rare entity is presented, highlighting its controversial etiopathogenesis.Presenting symptoms of ACP in most cases is unilateral progressive nasal obstruction. The occurrence of ACP bilaterally is rarely encountered in clinical practice. Diagnosis is mainly clinical and is achievable via nasal endoscopic examination and supported by computed tomography imaging. Treatment remains to be surgical, with 2 years of regular follow-ups being advised to detect any recurrence.This case report adds to the scarce data pool on bilateral ACPs and highlights the necessity of prudent and timely diagnosis of this uncommon entity to avoid unnecessary investigations and lengthy medical or surgical treatment. Additionally, a trial of medical therapy may provide symptomatic relief for patients who do not qualify for surgery. HighlightsAntrochoanal polyp (ACP) is a benign solitary lesion that originates in the maxillary sinus and may occur in adults and children.The etiology of ACP remains speculative, and their bilateral occurrence is extremely rare.ACP harbor ambiguous clinical and radiological features, entailing a considerable high index of suspicion to provide a timely diagnosis.Successful ACP treatment depends on complete surgical excision to eliminate the risk of recurrence.A frugal approach through a trial of medical treatment may provide symptomatic relief in patients who are otherwise unfit for surgery.Antrochoanal polyp (ACP) is a benign lesion arising from the mucosa of the maxillary sinus and extending through the natural or accessory ostium into the middle meatus, protruding posteriorly through the choanae into the nasopharynx (NP)A 44-year-old male smoker (15 pack-years) with no significant surgical, medical, or family history was referred to our rhinology clinic with a 1-year history of right-sided nasal obstruction, rhinorrhea, postnasal drip, snoring, mouth breathing, sleep disturbances, occasional sternutation, and itching. He denied other sinonasal symptoms, such as epistaxis, hyposmia, headache, and facial pressure. Endoscopic examination of the right side revealed a solitary nasal polyp originating from the middle meatus occupying the NP and almost entirely obstructing the right choanae. The left side showed a much smaller polyp that barely extended from the middle meatus, with marked left nasal septal deviation at the same level. Bilateral hypertrophy of the inferior nasal turbinates (HIT) was also noted Fig. . ConsentBased on the recorded history, endoscopic nasal examination, and CT findings, a diagnosis of bilateral ACPs was made. The patient was then counseled for functional endoscopic sinus surgery (FESS), but he refused and decided to undergo medical treatment. The Arabic Sino-Nasal Outcome Test (SNOT)-22ACP constitutes a minority of all benign nasal polyps, as it typically represents 4–6% of all nasal polyps in the general population; however, the rate peaks up to 33% among childrenA comprehensive description of ACP was first provided by Killian in 1906. However, he could not trace its origin until 1909, when Ion Kubo established that ACP originated from the maxillary antrumThe quest behind comprehending the etiopathogenesis behind ACP and its origin remains controversial, with many reported hypotheses and observations. The most lingering theory to date is the presence of an antral cyst as the origin of ACP. A summary of the etiopathogenesis of ACP and possible mediators of antral cysts is depicted in Figure et al.et al.To substantiate this theory, a radiological study by Bidkar Moreover, diagnosing ACP is mainly clinical; however, certain diagnostic challenges remain because of the rarity and ambiguity of its clinical and radiological signs; therefore, meticulous endoscopic examination, a high index of suspicion, CT imaging, and postoperative histopathological findings are indispensable for an accurate diagnosis. In turn, this may spare patients from prolonged, unnecessary medical treatment and/or aggressive surgery.The treatment of ACP is always surgical, with the FESS technique being the most preferred and effective procedureIn our case, the patient refused the surgical approach and was continued on the medical regimen while being monitored for the progression of his symptoms before and after treatment using the SNOT-22 score. After receiving adequate medical therapy that targeted his other sinonasal symptoms, including HIT, postnasal drip, allergic rhinitis, and quality of sleep, the patient’s symptoms and scores improved significantly. Nevertheless, the size of the ACP did not change. This is mainly because of the lack of inflammatory markers in ACP compared with that in chronic rhinosinusitis with nasal polypsn=6 vs. 6), with a mean age of 12–48 years. The predominant presenting symptoms were nasal obstruction and rhinorrhea, which is consistent with the symptoms of our case. Histological reports of all cases revealed the presence of benign inflammatory nasal polyps. Almost all patients were treated with complete surgical excision, except for our case. FESS is the most commonly used technique with no postoperative complications. No recurrence was reported during the mean follow-up period of 9.4 months  in both the title and abstract. Our search yielded 35 articles published in PubMed with no language restrictions, of which 11 were included in this study. Unilateral ACP cases and noncase reports were excluded. For analysis purposes, we also included our case. Data on the presenting symptoms of bilateral ACP, histological findings, treatment, complications, recurrence, and follow-up were collected for all cases. The cases included an equal number of male and female patients : not applicable.firasalmarri@gmail.com, ORCID: https://orcid.org/0000-0002-8981-411XFiras K. Almarri, Department of Otorhinolaryngology – Head and Neck Surgery, Ad Diriyah Hospital, Riyadh Third Health Cluster, Ministry of Health, Riyadh, Saudi Arabia, Tel: +966548104118, E-mail: Not commissioned, externally peer-reviewed.


Multifocal cystadenomatous oncocytic hyperplasia of the parotid gland is an extremely rare non-neoplastic lesion. We present a case involving a 73-year-old female with a painless, small mass in her right preauricular region for the past two months. Fine needle aspiration suggested a diagnosis of mucoepidermoid carcinoma. Following further investigations, the patient underwent an uneventful right superficial parotidectomy without neck dissection. Histopathological examination of the excised superficial parotid tissue revealed multifocal cystadenomatous oncocytic hyperplasia with negative surrounding margins. The patient’s subsequent follow-ups in the outpatient department were satisfactory, with no reported issues, concerns, or evidence of recurrence. Oncocytes are the main cells in oncocytic salivary gland lesions . These cAccording to the World Health Organization classification, oncocytic lesions are classified into three types: oncocytosis, oncocytoma, and oncocytic carcinoma . OncocytMAOH is a rare pathological condition of parotid neoplasms, characterized by oncocytic proliferation of the parotid gland’s duct system . In thisA 73-year-old female with a past medical history of type 2 diabetes presented to the outpatient clinic in the Department of Otorhinolaryngology - Head and Neck Surgery at Farwaniya Hospital in Kuwait with a small, asymptomatic, non-painful preauricular mass on the right side present for two months. A comprehensive head and neck examination, including lymph node palpation, revealed nothing remarkable except for a mass measuring 2.5 centimeters. She had initially undergone fine needle aspiration cytology (FNAC) for this mass, the results of which suggested mucoepidermoid carcinoma Figure .She underwent a contrasted CT scan of the neck, which showed a well-defined, non-homogenous lesion of the right parotid gland with no infiltration of the surrounding right parotid tissue Figure . AdditioA fluorodeoxyglucose positron emission tomography scan was also performed and found unremarkable for metastasis.Results from her routine laboratory investigations, including full blood count, renal function test, and coagulation profile, were within reference ranges. The patient was admitted and consented to right superficial parotidectomy. The operation also included taking specimens from the anterior, posterior, inferior, superior, and deep margins, a right deep lobe specimen, and an upper cervical lymph node. All specimens were sent for histopathology evaluation and returned negative results. The histopathology report of the right superficial parotid gland revealed multifocal cystadenomatous oncocytic hyperplasia Figure .Postoperatively, the patient had an uneventful three-day hospital stay and was subsequently discharged. Her frequent six-month outpatient clinic visits were satisfactory, with no new issues or concerns reported.In this case report, we presented a 73-year-old female patient diagnosed with the rare condition of MAOH of the parotid gland. Oncocytic transformation results from metaplastic development in reaction to detrimental alterations occurring within the cells or due to the natural aging process. These changes result in the depletion of functional mitochondrial enzymes, prompting a compensatory response characterized by the proliferation of mitochondria. This increased mitochondrial presence is accountable for the distinctive microscopic alterations observed in oncocytic cells [FNAC might not accurately identify whether an oncocytic neoplasm is benign or malignant . In our The standard and cornerstone treatment for MAOH is parotidectomy with facial nerve sparing. Patients should be followed up frequently for any local recurrences arising from residual tumors in case of incomplete resection. A peripheral residual clearance of about 5 mm should be performed during such procedures . ParotidIn conclusion, we report a rare case of MAOH in the parotid gland, highlighting the diagnostic challenges due to its similarities with various benign and malignant neoplastic lesions. This case emphasizes cytology's limitations and histopathology's importance for establishing a definitive diagnosis. The standard treatment for MAOH is parotidectomy with facial nerve sparing, and careful follow-up is crucial for monitoring local recurrences. Further studies are needed to determine this condition's exact pathophysiology, risk factors, and prevalence.


In situ follicular B cell neoplasm, previously known as follicular lymphoma in situ, is a neoplastic proliferation of follicular lymphoma‐like B cells confined to the germinal centers. Herein, we report a case of a woman in her 70s who initially presented with several enlarged abdominal lymph nodes. Seven months later during follow‐up, a solitary pulmonary nodule was detected. As it was close to the hilum, lobectomy was performed. The intraoperative frozen section showed fibrosis and a collection of lymphocytes and macrophages. Therefore, the lymph nodes were sampled. Station 4 and 10 lymph nodes exhibited similar tumor cells and were immunohistochemically positive for CD10 and BCL2. Thus, the patient was diagnosed with in situ follicular neoplasm and is currently under observation. In situ follicular neoplasm is typically a slowly progressive neoplasm; however, it can present as a rapidly enlarging pulmonary nodule complicated by pulmonary aspergillosis. We report a case of a woman in her 70s who initially presented with several enlarged abdominal lymph nodes. Seven months later during follow‐up, a solitary pulmonary nodule was detected. As it was close to the hilum, lobectomy was performed. Tumor cells of the specimen were immunohistochemically positive for CD10 and BCL2. Thus, the patient was diagnosed with in situ follicular neoplasm and is currently under observation. In situ follicular neoplasm is typically a slowly progressive neoplasm; however, it can present as a rapidly enlarging pulmonary nodule complicated by pulmonary aspergillosis. In situ follicular neoplasia (ISFN) is a neoplastic proliferation of follicular lymphoma‐like B cells confined to the germinal centers. Occasionally, it is detected as an incidental finding following lymph node resection for another lesion.Herein, we report a case of ISFN in a patient who underwent pulmonary resection for a pulmonary nodule for which no definitive diagnosis had been made.A woman in her 70s with a suspected pancreatic cyst was referred to the gastroenterology department at our institute. Abdominal ultrasound revealed no pancreatic cyst; however, several enlarged intra‐abdominal lymph nodes with nonspecific inflammatory changes were visualized. Chest computed tomography (CT) performed 4 months later did not show any apparent nodules; however, a repeat CT performed 7 months after detected a 1 cm lung nodule (Figure The intraoperative frozen tumor section showed only fibrosis and lymphocyte and alveolar macrophage collections instead of malignant changes; thus, the lymph nodes were sampled. The postoperative course was uneventful. The results of the pathological examination revealed an Aspergillus‐like fungal mass in the center of the tumor. The surrounding area was infiltrated by lymphocytes forming numerous lymphoid follicles. Based on several immunostaining results, ISFN, previously known as in situ FL, was diagnosed Figure . Both stThis case demonstrates two important points. First, in situ follicular neoplasm can manifest as a rapidly enlarging pulmonary nodule. In the present case, enlarged lymph nodes were already observed in the abdominal cavity; however, they did not change significantly during the disease. The pulmonary nodule was observed on the follow‐up CT. The interval between the first CT scan when no nodules were observed, and the second CT scan when they were observed, was 3 months, indicating a fast growth rate. ISFN is described as a slow‐progressing tumor, but the pulmonary nodule in the present case had an extremely fast growth rate. When pulmonary nodules rapidly increase in size, as in this case, the differential diagnosis is infection or malignancy rather than a benign tumor or ISFN. Moreover, in the current case, the pathological examination revealed an Aspergillus‐like fungal mass in the resected specimen. Therefore, pulmonary aspergillosis should be included in the differential diagnosis of the enlarged pulmonary nodules, which could explain the absence of enlarged intra‐abdominal lymph nodes. Furthermore, if ISFN had been considered in the differential diagnosis, a definite diagnosis would require resection.ISFN does not require aggressive treatment; however, careful follow‐up is needed. The rate of ISFN observed in resected lymph nodes is approximately 2%–3%, and the frequency of progression to overt FL is <5%.Conceptualization, Tsuyoshi Uchida and Ryunosuke Koizumi; Methodology, Tsuyoshi Uchida; Investigation, Naoki Oishi, Ryunosuke Koizumi and Harunobu Sasanuma; Formal Analysis, Tsuyoshi Uchida and Ryunosuke Koizumi; Resources, Tsuyoshi Uchida and Ryunosuke Koizumi; Writing ‐ Original Draft, Tsuyoshi Uchida and Yuichiro Onuki; Writing ‐ Review & Editing, Hirochika Matsubara and Aya Sugimura; Supervision, Hiroyuki Nakajima.The authors declare that they have no conflicts of interest.


Osteosarcoma commonly occurs in the intramedullary cavity of long bones such as the femur, tibia, and humerus in children and adolescents. Osteosarcoma occurring as a primary tumor in the chest wall is rare. Only a limited number of such cases have been documented in the existing literature. Herein, we present radiologic and pathologic findings of a high-grade surface osteosarcoma of the rib mimicking a neurogenic tumor in a 44-year-old woman. A 44-year-old woman visited an outpatient clinic due to a nodular lesion detected on a chest X-ray screening. She did not complain about any symptoms. She was never a smoker. She was previously healthy. She had no notable family history of cancer. According to her chest CT scan, the nodule was an extrapleural lesion located along the inner surface of left eighth posterior rib. It measured about 1.8 cm in its maximum diameter. The nodule showed homogeneous contrast enhancement with a smooth well-demarcated margin. Focal cortical erosion of the left eighth rib was noted. Given the imaging features and the location of the lesion, a neurogenic tumor such as schwannoma was suspected. Nevertheless, upon closer examination of a magnified CT image using a bone window setting, the presence of bony spicules oriented perpendicularly to the rib cortex was observed, indicating a potential sunburst-like periosteal reaction. This finding raises concerns about the likelihood of malignancy .We performed a video-assisted thoracoscopic excision of the nodule, along with adjacent ribs, for diagnostic and therapeutic purposes. Upon gross examination of the specimen, it was noted that the lesion abutted the eighth rib, with a very small portion of the nodule connected to the rib bone. Upon microscopic examination, the nodule was identified as a highly cellular spindle cell tumor. In a part of the lesion, woven bone with endochondral ossification was noted, suggestive of a high-grade osteosarcoma. A tiny part of the tumor was connected to the medullary cavity of the rib bone, indicating the potential presence of endosteal extension. Upon higher magnification, it was evident the malignant cells were infiltrating the medullary trabeculae . Based oOsteosarcoma was reported to primarily involve the rib in only 1.3% of cases ,3. BasedWe presented the radiologic and pathologic findings of a high-grade surface osteosarcoma that emerged in the intercostal space. Despite its resemblance to a neurogenic tumor, the detection of a discreet periosteal reaction in the adjacent rib cortex on CT images prompted the consideration of a potential malignancy.


It seems that schwannomas of the tongue base originate from branches of the glossopharyngeal, vagus, or hypoglossal nerves. Additionally, complete trans‐oral surgical excision is an efficient method to remove them. Tongue Schwannoma is rare, and a definite diagnosis is made only with histopathological examination. In the current study, we present a case of tongue schwannoma in a 21‐ year‐old male case, with radiological and histopathological investigations. For more detailed evaluations, the patient was referred to the ENT ward of Taleghani Hospital of Shahid Beheshti University of Medical Sciences, Tehran, Iran. The patient's chief complaint was swelling of the base of the tongue  was requested for more evaluation. According to the MRI findings, soft tissue mass with irregular and smooth border in the right posterior aspect of the tongue measuring about42.5 × 40 × 26 mm mildly low on T1 weighted images (T1WI) and high on T2 weighted images (T2WI) with homogenous enhancement after IV Gd was seen  and non‐encapsulated, located below the mucous membrane's basal stratum.In the present case study, the patient's chief complaint was swelling at the base of the tongue. According to a systematic review study by Sitenga et al. (2017), in 87% of patients with schwannoma of the tongue, the swelling was the most common symptomatic complaint.4Tongue Schwannoma (Neurilemmomas) is rare, and a definite diagnosis is made only with histopathologicalinvestigations. It seems that schwannomas of the tongue base originate from branches of the glossopharyngeal, vagus, or hypoglossal nerves, but the issue is open to discussion. Additionally, Trans‐oral surgical excision is an efficient method to remove most of the schwannoma at the base of the tongue.Mahboobe Asadi: Data curation; writing – original draft. Malihe Mohseni: Writing – original draft. Fatemeh Jahanshahi: Supervision; writing – original draft; writing – review and editing. Arash Esmaeili: Data curation; writing – original draft. Zhaleh Mohsenifar: Data curation; writing – review and editing.This study has no financial source and support.There is no conflict of interest to declare.The present study complies with ethical and research standards involving humans. This article does not contain any studies involving animals performed by any of the authors.Written informed consent was obtained from the patient for publication of this case report and the accompanying images. A copy of the written consent is available for review by the editor‐in‐chief of this journal.


Dear Editor,A 17-year-old boy without medical antecedents presented to the dermatology clinic with a six-month history of papular lesions on his lower extremities. Dermatologic examination revealed three asymptomatic, firm, yellow-brown papules, scattered on the lower extremities A–C. On dDermatofibromas are common fibrohistiocytic tumors that are mostly diagnosed clinically. However, variants of dermatofibroma sometimes present significant clinical and dermoscopic challenges, and they are best diagnosed by histologic examination.2Dermatofibroma is a very common fibrosing cutaneous soft-tissue tumor, typically diagnosed in young to middle-aged adults. Most patients present with a firm, solitary 0.5–1 cm papule, nodule, or plaque, usually brown in color, on the lower extremities.Lipidized dermatofibroma is a poorly recognized variant of dermatofibroma. It was first reported as lipidized or “ankle-type” fibrous histiocytoma by Calonje and Fletcher in 1994.5A recent study evaluating 13 cases revealed three dermoscopic patterns of lipidized dermatofibroma. In the total yellowish homogenous area pattern, the yellowish area involves the whole lesion. The atypical pattern is associated with irregular or centrally located yellowish homogenous areas. The third pattern contains the combination of a central white network and a peripheral delicate pigment network.The main differential diagnosis of our patient was Juvenile Xanthogranuloma (JXG). JXG is the most common non-Langerhans cell histiocytosis and typically presents as a solitary well-demarcated, dome-shaped yellowish papule or nodule, mostly on the head and neck of young children.Lipidized dermatofibroma rarely presents in young adults as small papular lesions. This uncommon clinical presentation of lipidized dermatofibromas could be easily mistaken for juvenile xanthogranuloma, cutaneous mastocytoma, or eruptive xanthomata. However, typical dermoscopic and histopathological findings point to the diagnosis of lipidized dermatofibroma, and no further investigations are performed. Lipidized dermatofibromas should be kept in mind in the differential diagnosis of lesions representing yellow areas in dermoscopy.None declared.Tugba KevserUzuncakmak - Participated in data collection; analysis and interpretation and critical literature review; preparation and writing of the manuscript. Muazzez CigdemOba - Participated in data collection; analysis and interpretation and critical literature review; preparation and writing of the manuscript. MehmetSar - Preparation and writing of the manuscript. ZekayiKutlubay - Participated in data collection; analysis and interpretation and critical literature review.None declared.