Many types of dorsal neoplasm of early infancy are described in literature ranging from benign to aggressive. Some are more common while others quite unusual. Here, we describe a newborn with a lumbosacral soft tissue mass. Positivity of S-100 and vimentin was compatible with the neural cell line and the high proliferation rate of major activity cells (biopsy Ki67 20%) suggests an aggressive nature. An exclusively surgical approach was chosen and no clinical or radiological signs of recurrence have been observed after 2 years of follow-up. This case is atypical for location, histological pattern, radiological aspect, and clinical behavior. Diagnosis is hard to define and limited to a mesenchymal neoplasia with myxoid tracts. The described aspects raise concerns about clinical and therapeutic approach, classification, and radiological follow-up of sacral tissue masses in newborns. We present a peculiar and still unclear case of lumbosacral mass in a newborn. In literature, some types of soft tissue neoplasm are described, starting from the most frequent   to the mA newborn female was admitted to our hospital in her first month of life for a visible right lumbosacral paramedian mass. Pregnancy was uneventful except for non-insulin dependent gestational diabetes.She was a term delivery, with birth weight at the 97° percentile and head circumference above the 97° percentile. Brain echography in the first week of life, revealed no pathological findings. Clinical and radiological initial evaluations and the following follow-up did not show other pathological findings, except for hip dysplasia treated with hip abduction braces in the first months of life.After admission, X-rays did not reveal clear bone involvement and an echography found an oval, strongly vascularized, lesion 36 × 15 mm), apparently connected to the spinal cord in the lumbar region. However, MRI . Proliferation index (Ki67) was 20% and only few mitoses were observed. Therefore, the mass seemed to be a non-aggressive soft tissues neoplasia with unidentified histotype.Taking into account the child’s very young age and the histological characteristics of the lesion and considering that the macroscopic appearance suggested a good surgical resectability, a surgical approach was preferred to chemotherapy.Surgical excision of the mass was performed and then analyzed Figure .It was identified as a mesenchymal neoplasia, with myxoid tracts of uncertain classification, probably a chordoma . Immunohistochemical expression resulted as follows: vimentin and S-100 positive; CD34 weak and focal positive; desmin, EMA, NSE, cytokeratin, inhibin, Melan A, synaptophysin, chromogranin, GFAP, estrogen, and progesterone receptors negative. Molecular analysis excluded rearrangements of EWSR1 and ETV6 genes and the presence of EWSR1/NR4A3 and TAF15/NR4A3 fusion transcripts.Positivity of S-100 and the high proliferation rate (of major activity cells) also suggested a mesenchymal chondrosarcoma, but this possibility was excluded after further histological evaluation.Further evaluations performed by Senior Pathologists from two other Centers  confirmed the uncertain classification of this neoplasm.MRI performed 3 months later Figure  found a Three further MRI exams performed (each after 3 months) showed unvaried dimensions of the area and led to the second hypothesis. The fourth showed no pathological findings.Up to now, the diagnosis is soft tissue sarcoma and a specific evaluation concerning prognosis is not possible, so the child is following a strict oncological follow-up.The main types of soft tissue malignancy in infancy are rhabdomyosarcoma, congenital fibrosarcoma, and infantile hemangiopericytoma. Rhabdomyosarcoma is characterized by cellular necrosis and small round or large spindle cells . Congenital fibrosarcoma shows a better prognosis than the adult type and is characterized by violet like skin discoloration and hypervascularity . The lesion may cause bone deformity, but bone destruction is quite rare.Microscopically, the lesion is highly cellular with spindle-shaped cells organized in fascicles or herringbone patterns and is frequently related to a specific translocation t.Infantile hemangiopericytoma shows a more benign outcome than the adult type and is characterized by a hypervascularized soft tissue mass that sometimes causes bone invasion.Peripheral nerve sheath neoplasia is also reported and is mainly associated to type 1 neurofibromatosis .Transformation of a myxoid mesenchymal neoplasia of infancy to an undifferentiated sarcoma is reported in literature, so a long-term follow-up of these diseases is recommended .Nonteratomatous neoplasms located in the sacral region in children form a very heterogeneous group without a prevalent type. Diagnosis is often late, but complete surgical resection is associated with a good prognosis .Chordomas are nonteratomatous neoplasms, infrequent in infancy, and mainly localized in the cranial region. They are low-grade neoplastic lesions developing from notochordal residues. Sacral chordoma usually shows a worse prognosis due to the venous drainage density of the region.Surgical resection and radiotherapy are the common treatments, but recently also photon therapy has been suggested .Mesenchymal chondrosarcoma is a rare malignancy more frequent in young adults and prone to cause lung metastases. Surgical resection is the main treatment, while the efficacy of chemo and radiotherapy is not clear. Dantonello et al. reported that clinical and prognostic criteria of adults could not be extended to the pediatric population and suggest radiotherapy if complete surgical resection is not possible. They also proposed considering induction chemotherapy .Habrand et al. reported the case of a 9-year-old boy with a chordoma of the cranial-cervical junction, treated with surgical resection, radiotherapy, and photon therapy. Ten years after treatment, MRI revealed a local tumor progression, treated with subtotal resection and radiotherapy. A year later, the patient died because of a hemorrhage that could have been caused by tumor progression or radionecrosis of the internal carotid artery .Shinmura et al. reported the clinical case of a 3-year-old boy with a sacrococcygeal chordoma. When diagnosis was performed, it had infiltrated the bone and lung metastases were detectable on CT. Treatment was performed with chemo and radiotherapy, with little response. The child died 14 months after the first admission to hospital because of respiratory failure due to lung metastasis .Namini et al. reported the case of a congenital sacrum mesenchymal chondrosarcoma in a newborn. The neoplasia was surgically removed, but 10 days later many metastases on the back were noted. Chemotherapy was suggested, but the parents refused. The infant died 3 months later .Dorso-lumbar teratoma is a quite common location in early infancy, but this type of neoplasm usually shows a more typical histological pattern, characterized by mature and benign components, derived from all three germ layers. In infants, it is frequently associated with spinal dysraphisms or cord malformations. Radiologically, it shows mixed solid and cystic morphology, fat signals, and calcifications .A case of soft-tissue sarcoma associated with Sotos syndrome has been described by Hill et al. , but ourThe described clinical case is peculiar for two reasons: first, the histological aspect of the mass does not allow a clear identification of the neoplasm and so a prognostic evaluation cannot be performed. Second, according to literature, soft tissue malignancies presenting a similar macroscopic aspect and localization in children, usually are more aggressive and invasive than in this case. Because of these uncertainties, a strict follow-up was strongly recommended. The child’s age raises some concerns: muscular tissue is still growing, so a more accurate histological evaluation is difficult. Furthermore, the child must be protected from side effects due to follow-up exams . At the same time, the distinction between scar and neoplastic tissue is mandatory to avoid recurrences.Taking into account the favorable behavior of the lesion (compared to similar neoplasms presented in literature) and the unclear diagnosis , we decided to describe this case and to share our findings with other physicians involved in neonatal and child care, with the hope that this will lead to further progress in the field.Managing lumbosacral soft tissue masses in newborns and infants is a challenge involving many health professionals  because of the large range of possible diagnoses and the lack of standardized approaches.Patient’s parents approved the publication with a written consent form.SC and FS wrote the paper. MB revised the paper.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.


Ancient schwannomas (AS) are exceedingly rare variant of common schwannomas (CS). Only two cases involving the cauda equina region have been previously reported in literature. AS are typically associated with a higher histological degree of degenerative changes (Antoni B areas). It is of peculiar importance, according to our opinion, to outline that, because of their extremely slow growth (which explains the increase of the degenerative changes in respect to the CS) and their typical soft consistency in respect to their standard counterparts, AS usually imply an even better prognosis.  To the best of our knowledge, there are only 2 cases previously reported of AS involving cauda equina , 2, an eThe authors report a case of AS involving cauda equina, an exceptionally rare lesion for an extremely rare location, and review all the relevant literature in order to outline clinical, radiological, and pathological remarks of this rare condition.A 36-year-old man was admitted to our department complaining of one-year history of progressively worsening back pain with bilateral radiation to the anterior surface of the lower limbs, combined with paresthesias to both lower limbs and urinary incontinence for about a month. The neurological examination reported a bilateral fall of both lower limbs in Mingazzini maneuver and Patellar and Achilles hyporeflexia and bilateral sensory deficit of sacral dermatomes.The patient underwent a lumbar spine MRI scan with TSE, FS, T1w, and T2w sequences and contrast enhanced T1 (Gadolinium) which demonstrated a voluminous intradural-extramedullary tumor (1.9 cm ×  2.8 cm ×  2.0 cm), with solid and cystic areas of heterogeneous intensity signal Figures  with a sThe patient was operated on for D12-L1 laminotomy and en bloc resection of the lesion. Gross pathology was consistent with a spinal schwannoma . The posHistology showed a mesenchymal neoplasm, with an alternation of hypercellular (Antoni A) and hypocellular areas (Antoni B) presenting degenerative traits like multiple microcystic areas filled with a tenuously basophilic colloid material in H&E stain, surrounded by a loose hypocellular fibrillary stroma with rare necrotic areas.The neoplasm was composed of spindle-cells with pyknotic nuclei and rare mitotic figures and a modest degree of nuclear pleomorphism. The neoplasm showed a remarkable S-100 immunoreactivity and a scarce reactivity for GFAP staining. The Ki67 immunoreactivity demonstrated an extremely low proliferating rate (less than 1%) Figures . HistoloBefore writing this paper patient was consulted and gave informed written explicit consent to this report.Ancient schwannoma is a rare variant of schwannoma <1% of CS) [% of CS . Up to 2Cauda equina tumors are rare entities with a total incidence of less than 6% among spinal primary neoplasms ; CS counAnother case of AS was reported by Hayashi et al.  that wasThese tumors are typically associated with a long lasting history of mild low back pain, progressively worsening over years , 15. It Occasionally, the clinical onset may be a tumor hemorrhage , 17, locAs for any other primary spinal neoplasm, Gadolinium enhanced MRI is the current “gold standard” for radiological diagnosis , 3, and The radiological appearance of the capsule and of the central “core” of the lesion is important in differentiating a CS from its “ancient” variant , 5, 24. In AS, Antoni A and B areas are more finely admixed; thus, the resulting MRI appearance is of a circumscribed, rounded mass with heterogeneous contrast enhancement , 5.Because of the different pattern of contrast enhancement in respect to CS and the presence of multiple cystic areas, the differential diagnosis between AS and malignant neoplasms such as fibrous histiocytomas, malignant peripheral nerve sheath tumors, liposarcomas, or haemangiopericytomas can be quite difficult  before hMacroscopically, the AS is not different from the standard schwannoma: but there are remarkable histological differences.CS is a mesenchymal neoplasm composed of an alternation of bundles of spindle-shaped cells with pyknotic nuclei packed in pseudopalisades (Antoni A areas) and hypocellular areas with cells organized in ovular masses surrounded by a loose stroma (Antoni B areas); mitotic index is low but mitotic figures are commonly observed , 6, 7.On the opposite, AS has a higher degree of degenerative phenomena involving Antoni B areas: microcysts, calcifications, intravasal thrombosis, necrosis, and hyalinosis are common findings. Nuclear pleomorphism is more pronounced than CS , 6, 25.The aforementioned histological features are useful to perform the differential diagnosis between AS and mesenchymal malignant neoplasms. Furthermore the absence of local invasion involving the tumor capsule and neoplastic vessels and a low Ki67 immunoreactivity complete the diagnosis [As for other intradural-extramedullary spinal neoplasms , surgeryWe strongly prefer laminotomy over laminectomy to approach an intradural-extramedullary lesion because of the risk of iatrogenic postoperative instability and spinal deformity. The incidence of postoperative instability in thoracolumbar spine can involve up to 25% of patients receiving two or more levels of laminectomies  and postAncient schwannomas are benign lesions characterized by a prevalence of Antoni B areas heterogeneously and finely admixed with Antoni A areas. It exceptionally involves cauda equina and only two other cases with cauda equina involvement have been previously reported. Surgical treatment is the gold standard for this condition.


The pineal gland is a small pinecone-shaped and functionally endocrine structure located in the epithalamus region. Developmentally, the pineal gland is considered as a part of the epithalamus. It plays a role in the entrainment of the circadian rhythms of an organism by producing melatonin, a functionally important hormone. Lesions of the pineal region are rare compared to other parts of the brain. A lesion may be tumorous or non-tumorous in nature. The most common lesions are tumors that are pineal parenchymal tumors (PPT) in origin. Gliomas are the second most common tumors in the pineal region. We report a case of a high-grade oligodendroglioma, not commonly seen in the pineal region, in a 45-year-old male. The patient was suspected to have a mass in the pineal region on a computed tomography (CT) scan and histology confirmed the diagnosis of oligodendroglioma. This is a unique case because only five such cases have been reported so far. The pineal gland is a small endocrine gland located in the epithalamus region. It produces melatonin, which helps in modulating the sleep cycle. Malignancies of the pineal gland arising from itself are very rare. The spectrum of tumors arising in this region is broad. Almost more than 17 heterogeneous tumors can arise in this region.To our knowledge, only five cases of oligodendroglioma of the pineal region have been reported in the literature. This is the sixth case, which makes this incidence a unique presentation.A 45-year-old man presented to the outpatient department with complaints of a frontal headache for the last four months. His pain was 6/10 on the intensity scale. The headache usually occurred in the morning, was severe in intensity, was associated with dizziness, and usually responded to over-the-counter pain medication. For past four months, the patient reported visual disturbances in the form of blurry vision, but on thorough examination by the ophthalmology department, a peripheral visual defect was ruled out and magnetic resonance imaging (MRI) of the brain was advised.The patient underwent a brain MRI and was referred to the medicine outpatient department where it was revealed that the patient had a mass in the pineal region as shown in Figure His baselines including the complete blood count, basic metabolic panel, liver function test, urine detailed report and chest X-ray were within normal limits. The patient denied any family history of brain tumors. His past medical history included diabetes and hypertension for the last five years. Both were well controlled on losartan and Glucophage, respectively.Neurological consult was sought and the patient was advised to get a brain biopsy done to channel the diagnosis into effective treatment options. The sample was processed and sent for histology. It showed a neoplastic lesion composed of polygonal cells against a fibrillary background. These neoplastic cells had a round nucleus exhibiting a moderate degree of pleomorphism and surrounded by a perinuclear halo. Significantly increased mitotic activity was appreciated. The immunohistochemical analysis came with positive CD 57, CD99, CD 10, diffuse positive for GFAP , weak positivity for synaptophysin, and a high Ki-67 index (around 17 percent of cells). Cytokeratin and extractable nuclear antigen (ENA) immunohistochemical stains were also negative. Fluorescent insitu hybridization (FISH) performed on a small number of cells showed deletion of 1p and 19q.In the light of all these findings, a diagnosis of oligodendroglioma was concluded. The patient was offered radiation and chemotherapy options like procarbazine, methyl-1-(2-chloroethyl)-1-nitrosourea (CCNU) and vincristine . After a detailed discussion, the patient and his family declined both options. Surgical management was also proposed but the patient deferred it.Oligodendrogliomas, a type of glioma, are believed to originate from the oligodendrocytes of the brain or from a glial precursor cell. Overall, they constitute less than 20% of all intracranial tumors and less than 25% of gliomas . Their pThe pineal region is a complex anatomical site sheltering the pineal gland surrounded by the quadrigeminal plate and the confluence of the internal cerebral veins forming the vein of Galen . AlthougGliomas are a very rare subtype of pineal region tumors; oligodendrogliomas of the pineal region, being a subtype of gliomas, further contribute to exclusively rare tumors with very few reported cases reported till date , 9.As stated previously, the grade of the tumor determines the course of the disease and treatment, with the overall five-year progression-free survival rate being around 79% for low-grade tumors as compared to 39% for high-grade tumors , 9. Our To our knowledge, only five cases of oligodendroglioma of the pineal region have been reported in the literature. This is the sixth case, which makes this incidence a unique presentation. The findings of all previous five cases and the current case are compiled in Table We present this case of oligodendroglioma in the pineal gland as it is the sixth case reported till now. This case will serve to remind physicians about its occurrence and presentation. A headache as the initial presentation of many illnesses should be given due consideration and one should rule out every possible cause. This, in turn, would help us to design a management layout for such an exceedingly rare tumor.


Soft tissue metastases of prostate cancer to other sites are extremely rare, and, to our best knowledge, there have been no reports of metastasis to soft tissue of the hand. A 63-year-old man was diagnosed with prostatic cancer. During treatment, bone and soft tissue metastases to the right hand, appearing in the first web space, were observed. The tumor was resected, along with both the first and second metacarpal bones. The thumb was reconstructed by pollicization of the remaining index finger, enabling the patient to use the pollicized thumb for activities of daily living. This is the first case report of prostate cancer metastasizing to the soft tissue in hand. After wide resection, pollicization was able to reconstruct a functional hand and thumb. Prostate cancer has been found to metastasize to the bones, regional lymph nodes, and lungs, with several previous reports describing metastases to other sites. Here, we present the first case of a prostate cancer that metastasized to the soft tissue of the hand.During a routine check-up a 63-year-old man was found to have a high prostate specific antigen (PSA) concentration (7.9 ng/mL). Transrectal fine needle aspiration (FNA) of the prostate provided a definitive diagnosis of poorly differentiated adenocarcinoma , 8/8 cores affected). No metastases were detected, and treatment with both the nonsteroidal antiandrogen bicalutamide (Casodex) and goserelin (Zoladex) reduced his PSA level to 0.2 ng/mL within three months. Five years later, however, despite his PSA level remaining low, local extension to the bladder and metastasis to the S1 vertebra were detected. Furthermore, he developed a gradually enlarging painless mass in the first web space of his right hand, adversely affecting his activities of daily living. Therefore he was referred to our department.Physical examination showed a well-circumscribed, elastic soft mass located between the right thumb and index finger. This mass, which was palpable but not mobile, measured 5 × 4 × 3 cm in size. The skin over the mass was discolored, suggesting that the tumor had invaded the skin. Daily living was impaired due to restricted range of motion (ROM) of the thumb. Although no pain was associated with the mass, the patient experienced sensory disturbance of the right thumb .Laboratory tests showed elevation of alkaline phosphatase but low PSA level (0.036 ng/mL).A roentgenogram of the right hand showed enlargement of first metacarpal interspace, indicating noncalcification of the soft tissue mass. The metacarpal bones adjacent to the mass were normal without any bony destruction.Magnetic resonance imaging (MRI) of the right hand revealed a well-defined, clearly circumscribed mass, with iso- to low intensity on T1 weighted images and heterogeneously high intensity on T2 weighted images. After intravenous administration of gadolinium-based contrast agent, the mass was well enhanced peripherally, but the central region was poorly enhanced, suggesting necrosis. The mass was adjacent to the first metacarpal bone .Thallium scintigraphy showed marked accumulation in the right hand but no accumulation in other parts of the body.Computed tomography (CT) of the entire body revealed a lytic and sclerotic lesion of the sacrum, which was considered metastatic.Based on these findings, the patient was differentially diagnosed with a primary malignant soft tissue tumor, such as a myxoid liposarcoma or myxofibrosarcoma, or with a metastatic lesion of the adenocarcinoma of the prostate.An open biopsy of the mass in the right hand and a CT-guided biopsy of the lesion of the S1 vertebra were obtained to make a definitive diagnosis. Pathological examination of both lesions showed multinodular growth of small-sized but pleomorphic anaplastic tumor cells with numerous mitotic figures. Some tumor cells were plump with eosinophilic cytoplasm. A focal sheet-like arrangement was observed, with no distinct organoid structure. Immunohistochemical evaluation showed that the tumor cells were positive for cytokeratin AE1/AE3, CAM5.2, vimentin, prostate specific acid phosphatase, and androgen receptor and negative for PSA, S-100, CD34, CD68, and smooth muscle actin. Reticulin silver impregnation showed an epithelioid-like structure. These findings indicated that the tumor was an anaplastic carcinoma rather than a mesenchymal malignancy. The patient was therefore diagnosed with metastatic prostate cancer .The treatment of patient with cancer should be performed on a case-by-case basis, depending on the patient's prognosis and functional capabilities especially in patients with multiple metastasis. Generally, palliative treatment, such as radiation therapy, is chosen because of the dismal prognosis of patients with metastasis. In the present case, the prognosis was thought to be relatively better than the other cancer; in fact, the prognosis of prostate cancer with bone metastasis is reported as 3.0–3.5 years . FurtherAfter discussion of treatment options with the patient and his family, he agreed to wide resection of the hand tumor and systemic chemotherapy. en bloc. Wide resection of the tumor was accompanied by disarticulation of the carpometacarpal (CMC) joint, osteotomy of the proximal second metacarpal bone, disarticulation of the second metacarpophalangeal (MCP) joint, resection of the tendons and neurovascular bundles of the thumb and index finger, and resection of both the first dorsal interosseous and lumbrical muscles. Concurrently, the thumb was reconstructed by pollicization of the remaining index finger, and the skin defect was covered with a skin graft. The pathological findings of the tumor were the same as the specimen at the time of biopsy, and microscopically free margin (R0 resection) was achieved. After surgery, the patient underwent chemotherapy with docetaxel (DTX) and estramustine. The patient was able to use the pollicized thumb for activities of daily living, such as writing, six months after the operation (The tumor, along with the surrounding tissues, was resectedperation . ChemothThe most common locations of prostate cancer metastases are the pelvic lymph nodes and bone, followed by the lungs, bladder, and liver . Soft tiAs described previously, a prostate cancer tends to metastasize to the bone. However, acrometastasis, that is, bone metastasis distal to the elbow and the knee, are rare, accounting for approximately 0.1% of all cases with skeletal metastasis. Men were almost twice as likely to acrometastasis as women. The most prevalent primary diagnosis is lung, followed by colon, breast, and genitourinary tract , 5. In tMetastases of well-differentiated prostatic adenocarcinoma show typical pathological features and are not difficult to diagnose. In contrast, metastases of poorly differentiated prostatic adenocarcinoma may present a diagnostic challenge. Immunohistochemical staining, especially for PSA, is useful in assessing the prostatic origin of these less latter tumors. However, higher-grade poorly differentiated adenocarcinoma may lack both pathological features and diagnostic antigenicity, including for PSA . Our patTreatment of patients with advanced prostate cancer must strike a balance between efficacy and acceptable side effects. After assessment of disease risk, patients should be informed about the benefits and side effects of all options . In addiWe have reported the first case of metastatic prostate adenocarcinoma to the hand. Wide resection of the metastatic tumor and reconstruction of the thumb by pollicization of the index finger resulted in satisfactory clinical and functional outcomes.


Esophageal duplication cysts (EDCs) are rare developmental anomalies. They may occur anywhere along the esophagus with the predominant location in the thoracic segment. Presently, most are diagnosed prenatally or in early childhood. The prevalence of EDCs is estimated at 1 in 8200 live births. Usually, cysts are asymptomatic in the neonatal period, but they may cause respiratory distress or feeding difficulties depending on the size and location of the lesion.This report presents a female neonate with a cyst located in the right pleural cavity recognized prenatally. Computed tomography confirmed the diagnosis and revealed a round cystic mass in proximity to the left lung base. Thoracoscopic cyst excision was undertaken on day 15 after delivery. The postoperative period was uneventful. Histological cyst examination confirmed the diagnosis of foregut duplication.This case underlines the importance of early diagnosis and treatment of EDC, before symptoms and complications arise, and confirms that surgery in the neonatal period is safe and effective. This anomaly seems to result in impaired development of the vertebrae, spinal cord, muscles, and gastrointestinal tract. In 1944, Bremer proposed a theory of faulty recanalization during the solid stage of development in the foregut. Another explanation for gastrointestinal duplications was suggested by Lewis and Thyng, who claimed that these duplications are persistent gut diverticula, which normally disappear prenatally.4 EDCs are commonly divided into 3 types: cystic, tubular, and diverticular.5 Most of them elongate in the lower part of the thoracic esophagus, although cervical and abdominal cysts have been reported as well.6 The majority of patients are asymptomatic and the lesions are usually found incidentally. However, respiratory distress, dysphagia, retrosternal pain, hemoptysis, and infection can occur in the case of large cysts with rapid growth or an unfortunate location.3 Early surgical excision is always recommended to prevent a symptomatic course of EDC. Technical progress in surgical instrumentation with its minimization allows for the use of minimally invasive techniques in the treatment of this mediastinal cyst. Classical thoracotomy has been slowly squeezed out by video-assisted thoracoscopy and the age of patients treated successfully with this approach has diminished. Here, we report thoracoscopic excision of an EDC performed on a neonate on the 15th day after birth.Esophageal duplication cysts (EDCs) are an uncommon congenital anomaly, and account for 20% of gastrointestinal duplications. The etiology of this entity is explained by several theories.The female neonate weighed 3770 g with 10 Apgar points in the 37 weeks of gestation and was admitted to the Department of Pediatric Surgery and Traumatology in Lublin on the first day of life due to a congenital thoracic cystic mass discovered by prenatal ultrasonography. Upon admission, the neonate was in general good condition. Chest and abdominal radiographs revealed a round mass in the right lung field. On the second day after birth, a chest computed tomography (CT) scan was performed. It showed a cystic mass measuring 35 mm in proximity to right lung base Figs. .The initial diagnosis of duplication of the esophagus or a bronchogenic cyst was made. The elevated bilirubin level decreased significantly during a period of 2 weeks and on the 15th day after birth, thoracoscopy was performed.The patient was in an almost prone position with a slight elevation of the right side of the thorax. The first 3 mm camera port was inserted below the tip of the scapula and 5 mm positive pressure insufflation was started. The second 3 mm port was located paravertebrally and the camera was changed to this port. Under direct visualization, the third 3 mm port was inserted at the anterior auxiliary line. The large cystic mass filled almost the entire pleural cavity, so initial puncture was necessary. The lesion was connected to the inferior part of the esophagus and was excised using a hook with electrocoagulation. The continuity of the esophagus was confirmed by pumping the air through the esophageal tube while the esophagus at the site of resection was submerged in a 0.9% saline solution. The thoracic tube was left in the right pleural cavity. Postoperative recovery was uneventful. A control chest radiograph was performed and the thoracic tube was removed on the fourth postoperative day. The excised mass was a unilocular cyst with a smooth thick wall. Histological examination showed boundless smooth muscle cells with a collagenous stroma within the cyst wall. The internal surface was lined with a ciliated epithelium. Based on these data, a final diagnosis of a foregut duplication cyst was established. On the seventh postoperative day, the patient was discharged in general good condition. The infant remains symptom-free 1 year after surgery. We got the parental consent to use clinical data of their child for scientific purposes .7 Diagnosis of EDC can be challenging because of its rarity and difficult differential diagnosis. It has to be distinguished from many mediastinal masses such as bronchogenic cysts, mature cystic teratomas, pericardial cysts, congenital cystic adenomatoid malformations, and neurogenic tumors.8 These mediastinal masses may have similar appearance by ultrasonography, x-ray and even CT visualization.9 Magnetic resonance imagination (MRI) is the most sensitive and relatively precise tool to make the diagnosis. It can reveal the characteristic structure of a cyst with a thin wall and fluid content and exclude concomitant anomalies such as spina bifida.8 In our case, there was no possibility of performing MRI, so we based our diagnosis on a CT chest scan.EDCs are rare congenital malformation. The incidence is estimated to be 1 in 8200 live births with male prevalence. EDCs mainly elongate in the thoracic segment of the esophagus, accounting for 30% of posterior mediastinal masses in children. A duplication in the cervical or abdominal part of the esophagus is also possible, but this occurs less frequently.1 Most of patients are asymptomatic prenatally and after birth, although 80% of them develop symptoms during childhood.10 Cough, stridor, feeding difficulties, and chest pain may occur, depending on the size, location, and growth of the EDC. Some cases of cardiac arrhythmia and bleeding from the cyst with an ectopic gastric mucosa have been reported.9 In more than 80% of patients, diagnosis is established before the age of 2 years, when the symptoms usually appear.4 Early diagnosis and treatment seem to be crucial for an asymptomatic course of the entity. In recent years, most mediastinal masses have been diagnosed prenatally, although a differential diagnosis with some similar lesions remains difficult in this evaluation. Some studies have suggested prenatal magnetic resonance imaging to further differentiate cystic mediastinal lesions revealed in prenatal ultrasound examinations.1 However, a histological examination is ultimately required to confirm the clinical diagnosis. There are 3 main criteria of an EDC: (1) it must be attached to or within the esophageal wall, (2) it is covered by 2 muscle layers, and (3) it is lined with a squamous, cuboidal, columnar, pseudostratified, or ciliated epithelium.11EDCs are often an incidental finding during routine radiological investigations.12 This procedure should be undertaken as soon as possible in children diagnosed with an EDC, because they are expected to develop symptoms if they have not done so already.13 The number of studies supporting the thoracoscopic approach has increased quickly. Most of them present cohorts of patients with foregut duplication cysts, which are divided into groups of patients with EDCs and bronchogenic cysts after surgery.15 The biggest study that we found was performed by Bratau et al15, published in 2005. This research compared 16 cases of thoracotomy and 11 cases of thoracoscopy undertaken on children with foregut duplication cysts. Thirteen cases of EDCs were finally recognized in this cohort. Bratau et al observed a shorter duration of drainage and length of stay in cases of thoracoscopy. These authors also noted a lower incidence of intraoperative complications such as tracheal and esophageal injury during thoracoscopic excision of the mass. Nowadays, it is commonly said that video-assisted thoracoscopy reduces postoperative pain, shortens the recovery period and allows early hospital discharge compared with open surgical resection via thoracotomy. The cosmetic outcome with minimal skin scarring is undoubtedly another advantage of this method.11 The age of children who have undergone thoracoscopy is continually decreasing. There are some cases of infants diagnosed with an EDC and treated by thoracoscopy reported in the literature. The course of surgical treatment is supported by the ongoing development of pediatric endosurgical instruments.16Surgical excision remains the treatment of choice in EDCs.We have performed a successful thoracoscopic EDC excision in a newborn on the 15th day after birth. We decided to operate at this time because of the size (35 mm) of the mediastinal mass, which was expected to cause symptoms and significant lung compression in the short term. The patient was diagnosed with a mediastinal cyst during a prenatal ultrasound evaluation and the CT chest scan after birth confirmed this finding. This early diagnosis enabled early surgical intervention. The tolerance to thoracoscopy procedure in our patient was excellent. The postoperative period was uneventful, and the newborn was discharged within 1 week after surgery. One year of follow-up revealed no symptoms or recurrences (chest radiograph was performed). The histological examination confirmed the diagnosis of a foregut cyst. Based on these data and clinical findings, a final diagnosis of and EDC was established. Searching the literature (PubMed) using the MeSH terms esophageal, duplication, newborn, child, and thoracoscopy, we found only a few cases of such an early thoracoscopic intervention in the case of an EDC.EDCs are uncommon congenital anomalies. Their early diagnosis and minimally invasive excision via thoracoscopy seems to provide a good outcome. This approach should be considered as the treatment of choice for EDCs.


Case Report. A nine-year-old patient with a diagnosis of tuberous sclerosis  that showed calcifications at the brain level. Besides, the case showed the Vogt triad . The patient clinically showed a volume increase of hard consistency, without suppuration and no sessile that included the following teeth 73, 74, and 75. Cone beam computed tomography (CBCT) was obtained, and it displayed a delimited unilocular lesion. After surgical excision, the histopathological report was desmoplastic fibroma (DF). It was observed that the patient had an aggressive recurrence of DF at four months after surgery treatment. Due to these clinical findings, resective osseous surgery and curettage were carried out. It is uncommon to find these two pathologies together (DF and tuberous sclerosis). Since DF is a benign pathology but very invasive and destructive, it is necessary a constant follow-up examination due to a high recurrence frequency. Tuberous sclerosis (TS) is a rare disease described by Von Recklinghausen of neurocutaneous autosomal dominant origin, which is characterized by the development of benign tumors. This disease exhibits a triad known as the Vogt triad, the full triad that includes seizures, mental retardation, and cutaneous angiofibroma occuring in only 30% of reported cases. It is described by the presence of hamartomatous benign tumors, neurofibromas, and angiofibroma, which can be developed in the eyes, kidneys, heart, skin, brain, lungs, and progressive intracranial calcifications. There is a family history of the disease in 50% of all affected subjects, and the birth prevalence is as high as 1 in 6000 birth cases [Oral manifestations of TS occur in 11% of affected subjects, and oral abnormalities include the presence of delayed eruption, bifid uvula, enamel hypoplasia, cystic hyperostosis, hyperplasia, enamel pitting, haemangioma, multiple osteomas, cleft lip and palate, and desmoplastic fibromas (DF) , 4. The A nine-year-old patient with tuberous sclerosis, who was diagnosed at five months of age, presented the Vogt triad . There was not a family history of tuberous sclerosis, epilepsy, nor mental retardation. However, cone beam computed tomography (CBCT) showed multiple radioopacities throughout the brain, which were diagnosed by the neuropediatrician .In March (2016), the patient attended to the dental clinic at San Luis Potosi University, oral examination revealed a facial asymmetry, and this increase of volume has no movement with a measure of about 8 mm in diameter overlying the left mandible from the canine to the second molar of the primary dentition. In addition, the enamel of permanent anterior teeth showed pitting or irregularities. Radiographs  and a cone beam computed tomography  were taken. The tomography shows the presence of a lesion of approximately 5.6 × 9.6 × 8.3 mm, and the cortical bone was covered with an incomplete fibrous capsule. Radiographic examination of the mandible revealed a large, round, radiolucent lesion with edges well circumscribed Figures . TherefoBefore the clinical procedure, the caregivers received an informed consent form where ethical principles were taken into consideration based on the Declaration of Helsinki on Ethical Principles for Medical Research Involving Human Subjects (version 2013). After the acceptance of the treatment by their parents, asepsis with iodopovidone was performed in the patient in the intervention site; local infiltration of lidocaine containing 2% of epinephrine  was carried out. Deciduous teeth 73 and 74 were removed, and then a full-thickness flap technique was performed; debridement and surgical enucleation of the lesion (10 × 15 × 13 mm) were carried out, ensuring that no fibrous tissue was left in the area. Subsequently, the flap was repositioned and sutured with Vicryl 4-0 . The biopsy was sent to the pathology department, and the following week, the patient attended to his follow-up appointment for stitches removal Figures .The histopathological showed a DF, constituted by a proliferation of connective tissue with the presence of fusiform fibroblasts deposited between dense hyalinized collagen bundles . Once thIn the clinical follow-ups, radiographically was observed that the first premolar (34) during the first three months, and there was no visible lesion . HoweverAn osseous resective surgery with safe margins and curettage of the surgical site was performed with a subsequent use of the orthodontic button onto the left mandible canine. It was placed a closed chain without traction . FinallyThere is little information about the combination of desmoplastic fibroma and tuberous sclerosis; this combination is uncommon , 10, 11.It has been observed radiographically that relapsing lesions are unilocular or multilocular; the patient showed a unilocular radiolucent area at four months of the first surgery, coronal to the erupting dental organ 33 , 11. DF Establishing a diagnosis of DF is difficult by imaging studies alone, since many tumors resemble DF. The differential diagnosis includes a wide range of lesion from benign lesions  to malignant lesions (osteosarcoma). In addition, DF includes pathologies from soft tissues (desmoplastic fibroma) to bone tissues (bone desmoid tumor). Besides, due to its high recurrence, osseus resective surgery with safe margins and curettage should be considered as the optimal treatment. The follow-up examination is an important issue to detect recurrence; it should be done monthly for the first year.In the present case report, it was observed that the patient had an aggressive recurrence of DF at four months after the first surgical resection; it was necessary a second surgery that included safe margins and curettage. However, most reported cases did not mention the relapse time, just a high rate of recurrence. Besides, it is uncommon to find these two pathologies together (DF and tuberous sclerosis). Furthermore, it is necessary an excellent follow-up control due to a high recurrence frequency. The DF is benign pathology but is very invasive and destructive.


Vascular anomalies accompanied with the diploic veins are rare. Among them, sinus pericranii, which is characterized by abnormal connections between intra- and extracranial venous systems, is relatively common. Besides sinus pericranii, a few cases of subepicranial varix with connections to diploic veins have been reported, but these varices had no connections to intracranial venous sinuses. Herein, we present a rare case of an expanding venous sac in the diploe which communicated with the intracranial sinus but not with the extracranial venous systems.An adult woman presented to us with a minor transient headache. Although no abnormal appearances were found on her scalp, imaging studies showed a club-shaped venous sac in the left parietal diploe that communicated with the superior sagittal sinus and diploic veins on the medial and lateral sides, respectively. It was revealed that the lesion had expanded as compared with a previous computed tomography image. Surgery was performed to intercept venous supply from the diploic veins, and the lesion was filled with thrombi. In a follow-up of 15 months, there was no recurrence of abnormal venous flow. Histological examination showed the endothelial lining in the membranous wall of the sac, which is typically observed in sinus pericranii. However, no communication with the pericranial veins of the scalp was identified on the imaging studies and intraoperative observation. Accordingly, it was diagnosed as another entity “intradiploic varix”.The abnormal connection between the intracranial and the diploic venous systems via the large venous sac was surgically treated. It was pathologically similar to sinus pericranii, but anatomically considered to be another form of venous anomaly. In cases of expanding lesions, surgical treatment is recommended. The diploic venous system is located between two compact tables of the skull. It communicates with the dural sinuses and pachymeningeal veins along the inner table, and with the pericranial veins on the outer table via the emissary veins (Hershkovitz et al. A 76-year-old woman with no previous medical history, including that of head trauma, presented to us with a minor transient headache and was examined. The results of the patient’s general and neurological examinations were normal. No abnormal appearances were found on her scalp.Radiography showed the presence of a linear bone deficit in the left parietal diploe Fig. . The lesThe patient opted for surgical treatment. During surgery, a portion of the skull that was assumed to be on the lesion was exposed via a linear skin incision. Neither substantial changes nor deficits were found on the skull surface, but a part of the surface that was located around the lateral end of the lesion contained a reddish-blue tinge, suggesting the thinning of the outer table of the skull. The bluish part of the outer table of the skull was removed to expose a membrane of a sac-like structure which was filled with liquid. The membrane was not pulsating. The sac had several communications with the diploic veins that we assumed to be feeding vessels. By interrupting these inflows, the sac deflated. A section at the end of the sac was cut out and sent for histological examination. There was copious bleeding from multiple interdiploic venous channels, and it was controlled by the bone wax. On the other hand, bleeding from the edge of the sac was not so heavy, suggesting that main feedings no longer remained. The edge was then ligated and the scalp was closed in layers.Histopathological study of the lesion showed a membranous wall that consisted mainly of fibrous tissues with scattered spindle cells Fig. a. No oncThe post-operative course of the patient was uneventful. MRI showed that there was an intensity change in the inside of the sac on T2-weighted images. The main part of the inside of the sac showed low intensity prior to surgery Fig. d but chaThe diploic venous system is connected with both the intra- and extracranial venous systems via the dural sinuses and the emissary veins, respectively. It is involved in various vascular anomalies. Although its occurrence is rare, abnormal arteriovenous shunts to the diploic vein can be lethal. For instance, dural arteriovenous fistulas can cause intracerebral hemorrhage (Yako et al. The patient in this case report also had a varix-like venous sac in the diploe that was continuous with the diploic veins, but it was covered by the outer table of the skull and isolated from the extracranial structures. Because of this distinct lack of communications with the extracranial venous system, it belonged to neither sinus pericranii nor subepicranial varix. Accordingly, it was diagnosed as another entity of venous anomaly that should be termed “intradiploic varix”. This entity has not reported previously maybe because it should be asymptomatic in general.On the other hand, from a pathological viewpoint, as our histological study showed the endothelial lining, which is typically observed in sinus pericranii (Bollar et al. Another significant point in our case was the expansion of the lesion during the 5-year period and the surrounding hyperosteosis. Assuming that the pathology is similar to that of sinus pericranii, the intravarix pressure would increase and decrease intermittently in response to the intracranial pressure. For sinus pericranii, this intermittent pressure change leads to a change in the size of the scalp swelling. However, in our case, because the venous sac was covered by the cortical bone, it may have gradually eroded the diploe and simultaneously induced reactive hyperosteosis.In regards to the treatment, the patient opted for surgery despite the lack of symptoms because the lesion had been expanding and it was necessary to differentiate it from neoplastic diseases. Intradiploic varices that have a low risk of being lethal do not always need to be treated, but in cases of expanding lesions such as in our patient, treatment is recommended to avoid further erosion of the skull. Aside from direct surgical interventions such as resection or venous occlusion, endovascular occlusion may also be an option, based on the history of sinus pericranii treatment. However, there is still insufficient evidence to support it (Brook et al. The abnormal connection between the intracranial and the diploic venous systems via the large venous sac was diagnosed as a new entity of venous anomaly that should be termed “intradiploic varix”. It could be considered pathologically similar to sinus pericranii. This case suggests that invasive treatments are recommended for expanding lesions, even if they are asymptomatic.


Hibernomas, especially located in the mediastinum, are extremely rare benign tumors, which are important to consider in the differential diagnosis of a heterogeneously enhancing mass with areas of fat attenuation on imaging of an often incidentally discovered mass. Other common possibilities in the differential include malignant tumors, such as liposarcoma, hence histopathology is usually required to confirm the diagnosis. Hibernomas often follow the distribution of sites of persistence of brown fat in adults, and intrathoracic locations are unusual. We present a very rare case of a mediastinal hibernoma in a 53-year-old woman. She presented to the emergency department with severe, progressive right neck and shoulder pain with radiation down her arm and was found to have a right apical posterior mediastinal mass on imaging. Initial radiographs of the shoulder showed a soft tissue mass within the apical right hemithorax. Further imaging with CT revealed a well circumscribed, heterogeneously enhancing mass with areas of fat attenuation. Pathology confirmed the diagnosis of mediastinal hibernoma, and the mass was completely excised. Fourteen months after surgery, the patient had a normal chest radiograph, and thirty-two months after surgery, she remains asymptomatic. Hibernomas are rare, benign tumors composed of brown fat. Hibernomas can occur in multiple sites, including the head, neck, trunk, and extremities. Only a few mediastinal hibernomas have previously been documented in literature. These tumors enhance heterogeneously and often contain macroscopic fat, which can result in misdiagnosis as liposarcoma. While imaging can provide useful clues, the diagnosis needs histopathologic confirmation. We present a very rare case of a mediastinal hibernoma with excellent radiology-pathology correlation.A 53-year-old woman with no significant past medical history presented to the emergency department with a ten-day history of progressive, severe right neck and shoulder/scapular pain that was worse with movement and radiated down her right arm to her fingers. NSAIDs, range of motion exercises, and trigger point injections had provided only minimal relief. She denied recent trauma, had no infectious symptoms, and reported no smoking history.Right shoulder radiographs revealed a right superior mediastinal mass . FurtherMesenchymal neoplasms, lymphoma, and teratoma were considered in the differential diagnosis based on imaging. Microscopically, the CT-guided biopsy showed multivacuolated and granular eosinophilic cells with small centrally located nuclei consistent with hibernoma. No lipoblasts or cytologic atypia was seen to suggest liposarcoma. Due to the unusual location, large size, and possible mass effect on adjacent structures, total excision was performed by a right video-assisted thoracoscopy. The surgeon described a dense, fatty, lobulated mass located in the posterior mediastinum with extension into the lower neck and without direct invasion of surrounding structures. The resection specimen measured 8 × 5 × 3 cm. Grossly, it was described as a circumscribed, yellow to tan, lobulated mass . HistoloOn follow-up, the patient had transient right vocal cord paralysis, likely due to the close proximity of the resected mass to the recurrent laryngeal nerve, with return of full vocal cord mobility about one month after surgery. No CT evidence of recurrence was seen on a chest CT four months after surgery, and a chest radiograph fourteen months after surgery was normal. Thirty-two months after surgery, the patient remains asymptomatic.Tumors composed of brown fat are called hibernomas, a term that originated due to the similarity to brown fat in hibernating animals . HibernoSince these tumors are hypothesized to arise from tiny remnants of fetal brown fat, it is logical that common sites for hibernomas are areas known to contain brown fat in adults , 3. In tIt is important to recognize hibernomas from imaging and pathology as benign tumors, not to be confused with malignant tumors like liposarcomas. On CT, hibernomas are well circumscribed, heterogenous lesions with mixed low attenuation fatty components and enhancing soft tissue components. It is essential to note that hibernomas are hypervascular, a key imaging differentiation from liposarcomas , 5. On MGross pathology is typically described as a tan colored, well-defined, soft, lobulated mass that can be encapsulated . HistoloAlthough the masses are most commonly asymptomatic, they can grow to large sizes and become symptomatic. Therefore, treatment with complete surgical resection is common. Incomplete excision can lead to local recurrence of the tumor . Overall


Schwannomas usually occur in adults being between the second and fifth decades, and such neoplasms are extremely rare in a pediatric population. In addition, they are not normally found in the retroperitoneal region. Here, we present a pediatric case of a retroperitoneal schwannoma in an adrenal location where the tumor was not able to be preoperatively differentiated from other benign or malign adrenal gland tumors. In our opinion, this tumor can be included in the differential diagnosis of a nonfunctioning retroperitoneal adrenal mass in children. Schwannomas are usually seen in adults between the second and fifth decades of life . They arHere, we present a pediatric case of a retroperitoneal schwannoma in an adrenal location where the tumor was unable to be preoperatively differentiated from other benign or malign adrenal gland tumors.A 14-year-old boy was referred to our institution with a retroperitoneal left adrenal mass that was detected on abdominal ultrasound during an investigation into complaints of intermittent abdominal pain and vomiting for about one year. A physical examination of his abdomen found no palpable mass but revealed tenderness in the left lower quadrant. The patient's medical history revealed rectal and intravesical ulcerations medicated 5 years previously and a tonsillectomy 1 year previously, and he had spotty skin pigmentations dissimilar to café au lait spots.The patient's routine blood tests were normal and peripheral blood smear, lactate dehydrogenase, ferritin, neuron-specific enolase, adrenocorticotropic hormone (ACTH), cortisol and urine vanillylmandelic acid (VMA), homovanillic acid, and metanephrine values were within the normal ranges. No pathological findings were found in the results of a chest X-ray, bone marrow aspiration, and biopsy. Ultrasonography demonstrated a hypoechoic 30 × 37 mm solid lesion close to the medial upper pole of the left kidney causing a change in kidney contour. A dynamic computed tomography (CT) scan of the abdomen confirmed the ultrasound findings and revealed a 32 × 40 mm homogeneous mass in the left adrenal region with a regular contour . AbdominDuring surgery, the abdomen was opened in a transperitoneal way via a left subcostal incision; entering through the bursa omentalis, the pancreas was deviated cranially and a 4.5 cm mass that was immobile, stiff, and adherent to the surrounding tissue was completely excised, including part of the adrenal gland. Histopathological examination showed features of a benign schwannoma. The mass was solid and had smooth surface capsule. There were yellow and gelatinous areas as well as hemorrhages . In the The clinical course was uneventful and the patient was subsequently discharged on postoperative day 11. During the first year after the operation the patient was controlled using ultrasound and no problems were detected. After this period, the patient was lost to follow-up.Schwannomas are tumors of the nerve sheaths that have a generally benign nature , 4–6. ThA search of the medical literature reveals that schwannoma cases originating in the adrenal medulla are very rare. Often, retroperitoneal schwannomas, particularly those located in the juxta-adrenal space, cannot be diagnosed preoperatively when they mimic adrenal lesions. In Poland, out of 1,111 adrenal incidentaloma cases that were reviewed, only 2 appeared to show schwannomas . Our casThe symptoms of a benign schwannoma are usually nonspecific and change according to the location and size of the lesion . RetropeIn general, malignant schwannomas are diagnosed in patients who have neurofibromatosis type 1 and neurofibromatosis type 2 . The colOther neurogenic tumors such as paraganglioma and pheochromocytoma are considered additional pathologies in the differential diagnosis of retroperitoneal masses , 4. If tHormone-releasing tumors must be excluded by the clinician if an adrenal lesion is present, particularly pheochromocytoma. Because hemorrhage, infection, and seeding of the tumor cells are known risks and are lethal if there is a pheochromocytoma, biopsy is not recommended for diagnosis. Distinguishing among adrenal schwannomas based on diagnostic imaging studies can be difficult . The staBecause malignancy cannot be excluded with analyses performed pre- or perioperatively and because schwannomas are insensitive to radiotherapy and chemotherapy, complete resection of the tumor is recommended , 8. BeniAlthough Antoni A and Antoni B areas are seen in various ratios in classic schwannoma, varying morphologies have been described, including ancient, cellular, melanotic, plexiform, glandular, and epithelioid subtypes . AccordiIn view of the above, we believe that this tumor can be included in the differential diagnosis of a nonfunctioning retroperitoneal adrenal mass in children.


Final diagnosis was IPEH associated with an organizing thrombus. Dentists should be aware about this rare benign vascular lesion, whose final diagnosis is achieved only after histopathology analysis. Surgical removal is the treatment of choice and no recurrence is expected.Intravascular papillary endothelial hyperplasia (IPEH) is a benign lesion of the skin and mucosa of vascular origin characterized by reactive proliferation of endothelial cells. A 76-year-old woman was referred presenting a painless nodule on the lip. Intraoral examination revealed bluish submucosal nodular proliferation, measuring 10 × 5 × 5 mm, affecting the lower labial mucosa. The lesion had a firm consistency and it was not fixed to the adjacent tissues. The main differential diagnoses were mucocele/mucus retention cyst, sialolith, or salivary gland neoplasia. An incisional biopsy was performed and during the intraoperative procedure an encapsulated red-bluish nodular mass was observed. Microscopic analysis revealed papillary endothelial proliferation in the center of the lesion and fibrin admixed with inflammatory cells in organization peripherally. There was no nuclear atypia, mitotic figures, or necrosis. The endothelial cells were CD34 positive, with low Ki-67 proliferation index (4%).  Intravascular papillary endothelial hyperplasia (IPEH) was first described by Masson in 1923 . The mosα-SMA) highlighted the smooth muscle cells in vessel walls  and the oral site most frequently involved is the lower lip (40.5%), although nearly any other oral site can be affected . IPEH usVarious other benign and malignant vascular proliferative disorders should be considered in the histopathological differential diagnosis; in particular the papillae fuse to form an anastomotic vascular pattern that may mimic the appearance of angiosarcoma –12. Noneα-SMA highlighted the vessel walls, whereas D2-40 (podoplanin) was negative. This latter excluded a lymphatic origin in the current IPEH case.Some immunohistochemical markers can be useful in the identification of the IPEH lesions. Moreover, the Ki-67, a cellular marker for proliferation, may help to predict the biological behavior of IPEH lesions , 13. MorThe treatment of choice for IPEH is total excision, and recurrence is extremely rare . SclerotAs mentioned above, the clinical features of IPEH are nonspecific; hence, the histopathological examination, sometimes supplemented by immunohistochemistry, is required for establishing a correct diagnosis. Regarding its histopathological resemblance to low-grade angiosarcoma, oral IPEH lesions should be recognized, especially by dentists, preventing misdiagnosis and unnecessary aggressive treatment.


We report a case of hemosiderotic dermatofibroma presenting as a brown‐black‐colored nodule with peripheral extensions, which mimics melanoma. Histopathology showed completely benign features with no atypia or mitosis. Nodular extensions of childhood dermatofibromas may be related to the growth of the child not necessarily pointing to a malignant process. Dermatofibroma (DF) is a common benign fibrohistiocytic tumor of the skin and has a slight female preponderance A 12‐year‐old boy presented to our clinic with a firm, nodular mass on the medial aspect of the right knee with a history of about 1 year. The lesion was not pruritic or painful, but mild tenderness was present. The patient stated that the size of the nodule had gradually increased over the last 3 months. Physical examination revealed a dark brown‐black nodule, measuring about 1 × 1 cm, with two small nodular extensions from the periphery (Figs Dermoscopy revealed focally localized blue‐red pigmentation areas on a white structureless background and pinkish red area at the periphery surrounded by brown pigmentation Fig. . The lesDermatofibroma is a benign dermal fibrohistiocytic tumor with a slight female predominance The common variant of DF is characterized by a well‐circumscribed, red‐brown nodule typically located on the legs DFs can display a variety of dermoscopic patterns. A peripheral delicate pigment network and central white scar‐like patch is the typical and most common dermoscopic feature of DF Hemosiderotic DF is considered as a precursor stage of aneurysmal DF by some authors Dermatofibroma is generally known as a benign mesenchymal neoplasm of the skin, but high cellularity, increased atypia, and proliferative activity can be seen histopathologically especially in the cellular, aneurysmal, and atypical variants Hemosiderotic DF, which is a rare variant of dermatofibroma, has never been reported in children until now as far as we are aware. Our case was also interesting as the lesion clinically gave the impression of a rapidly growing melanoma with its dark brown‐black color and peripheral spreading, but no features pointing to a malignancy were detected on histopathological examination, thus forming a clinicopathological discordance. The clinical appearance was attributed to the DF lesion's evolution with the growth of the patient. It must be kept in mind that DFs can enlarge with peripheric nodular extensions in childhood, not necessarily pointing to a malignant process. Our case also reveals that hemosiderotic DFs must be considered in the differential diagnosis of pigmented nodular lesions in children.The authors declare that there is no conflict of interest regarding the publication of this article.EMA: involved in the acquisition of clinical and histopathological data, design and draft of the manuscript, and revision and approval of the final manuscript; MT and AK: interpreted the histopathological data, designed and drafted the manuscript, and approved the final manuscript; FK: designed and drafted the manuscript, and approved the final manuscript.


FGFR3-PHGDH fusion. We describe this fusion and our rationale for pursuing personalized, targeted therapy for the patient’s tumor that may potentially play a role in the treatment of similar cases.Approximately 1–5% of pediatric intracranial tumors originate in the thalamus. While great strides have been made to identify consistent molecular markers in adult oligodendrogliomas, such as the 1p/19q co-deletion, it is widely recognized that pediatric oligodendrogliomas have a vastly different molecular make-up. While pediatric thalamic or “central oligodendrogliomas” are histologically similar to peripheral pediatric oligodendrogliomas, they are behaviorally distinct and likely represent a cohesive, but entirely different entity. We describe a case of a 10-year-old girl who was diagnosed with an anaplastic glioma with features consistent with the aggressive entity often diagnosed as central or thalamic oligodendroglioma. We performed whole-exome (paired tumor and germline DNA) and transcriptome (tumor RNA) sequencing, which demonstrated an  Due to the essential functions of the thalamus and surrounding structures, as well as the difficult surgical approach to the midline, biopsy and resection were rare in the past. But with improved surgical techniques, total or partial resection is now more frequently performed.7Tumors originating in the thalamus comprise ~1–5% of pediatric intracranial tumors.9 Unfortunately, it is also understood that while pediatric tumors may resemble adult tumors, they are often genetically dissimilar.10 Notably, while 50–70% of adult oligodendrogliomas demonstrate a 1p/19q co-deletion, this is only rarely seen in children with oligodendrogliomas.11 While pediatric thalamic or “central oligodendrogliomas” are histologically similar to peripheral oligodendrogliomas, they are behaviorally distinct and likely represent a cohesive, but entirely different entity.15 Approximately 2/3 of central oligodendrogliomas are anaplastic  at biopsy.As the field of neuro-oncology became increasingly proficient in detecting molecular alterations within tumors, precision medicine for high-risk brain tumors has rapidly expanded in the clinic. Molecular analysis of adult gliomas has already begun to demonstrate specific molecular markers that designate prognostic and therapeutic benefit.Overall, the biological basis for this behavior is not well understood and very little is known about the molecular attributes of pediatric central oligodendrogliomas. Here, we present a pediatric patient who was diagnosed with a thalamic anaplastic glioma with features consistent with the aggressive entity often diagnosed as central oligodendroglioma. Sequencing of the patient’s tumor led to the identification and therapeutic targeting of a fibroblast growth factor receptor (FGFR)-containing fusion.A 10-year-old girl without a significant past medical history was presented, with a 3-week history of right-handed weakness and right facial drooping. A neurologic examination was significant with right-sided facial weakness and arm weakness. Magnetic resonance imaging (MRI) of the brain revealed a heterogeneously but minimally enhancing, 5.1 × 3.8 × 3.4 cm ovoid mass involving the left thalamus, upper brainstem, and internal capsule and extending posteriorly to the cerebellar peduncles. The left lateral ventricle appeared distorted with displacement of the septum pellucidum and third ventricle. Peripheral edema extended to the left caudate, bilateral hypothalami, bilateral temporal cortices, and right thalamus  clinical trial ACNS0831 and underwent multi-agent induction chemotherapy with carboplatin, vincristine, cyclophosphamide, and etoposide. A follow-up MRI 3 months later showed interval progression in the size of the thalamic tumor  mutation, KIAA:BRAF fusion, and BRAF v600E mutation. Additionally, the tumor was positive for loss of 1p, but negative for co-deletion of 19q. At this point, a diagnosis of “anaplastic glioma with clear cell features” was rendered with the comment that these features were consistent with the aggressive entity often diagnosed as central or thalamic oligodendroglioma in the pediatric population. The patient was treated with conformal fractionated external beam radiation therapy (RT) for 6 weeks. During RT, the patient developed moderate headaches that were treated effectively with dexamethasone. At the end of this treatment, the patient had some improvement in her clinical exam and a post-RT MRI showed a partial response.Based on the patient’s poor response, further pathology review was performed at multiple centers, as well as centrally at COG to clarify her diagnosis. Pathology showed fragments of densely cellular glial neoplasm composed of relatively uniform, regularly distributed, ovoid cells with round, centrally located nuclei, stippled chromatin, and clear-to-eosinophilic cytoplasm Fig. c. The ba16 Nucleic acid preparation, high-throughput sequencing, and computational analysis were performed using standard protocols in our sequencing laboratory in the Michigan Center for Translational Pathology, which adheres to the Clinical Laboratory Improvement Amendments (CLIA).Due to the rarity and poor prognosis of this diagnosis, the patient was enrolled on PEDS-MIONCOSEQ, which was approved by the University of Michigan’s Institutional Review Board. PEDS-MIONCOSEQ is a precision oncology study involving whole-exome (paired tumor and germline DNA) and transcriptome (tumor RNA) sequencing and genetic counseling. Clinically integrated sequencing was performed according to previous published methodology.FGFR3 copy gain with an additional activating in-frame fusion of FGFR3 with phosphoglycerate dehydrogenase (PHGDH) and increased FGFR3 expression (see FBXW7 (p.R465H) mutation was seen that has been described before but was of unclear significance in this case.18 The activating in-frame fusion of FGFR3-PHGDH is a canonical FGFR3 fusion.19 The fusion occurs at the very 3’ end of FGFR3, with the extracellular domain, transmembrane domain and kinase domains intact  penetration, and confirmed that no relevant clinical trials were available.Sequencing confirmed 1p loss on copy number analysis and revealed an 23 The patient weighed 75 kg and was initially started on a treatment regimen of 15 mg of ponatinib daily, with a goal dose of 45 mg orally. After 2 months, the patient’s dose was increased to 30 mg daily for the second cycle. After two cycles, the patient had demonstrated a partial response , as well a case report describing ponatinib use in a pediatric patient, we ultimately decided to initiate ponatinib for this patient.24 Even with current treatment modalities, the prognosis for pediatric HGGs remains poor.27 Fortunately, HGG are relatively rare, comprising less than 20% of pediatric brain tumors.28 The median survival for pediatric patients with HGG is less than 2 years.29 Oligodendrogliomas comprise about 9% of all gliomas and are classically classified in adults as a glioma harboring a 1p/19q co-deletion.30 In adult glioma, 1p/19q co-deletion frequently co-occurs with a mutation in IDH1.31 Recent studies have distinguished clinical and molecular sub-types of grade II–IV glioma based on the status of IDH1 mutation, 1p/19q co-deletion, and telomerase reverse transcriptase (TERT) promoter mutations.32 While great strides have been made to identify consistent molecular markers in adult oligodendrogliomas, it is widely recognized that pediatric oligodendrogliomas have a vastly different molecular make-up.11 The current study supports other molecular data, which demonstrates that partial loss or alteration of 1p is common in pediatric central oligodendrogliomas.3 This finding is unique compared to hemispheric pediatric oligodendrogliomas, which rarely demonstrate partial loss or alteration of 1p.34 Thus, it is imperative to discover new molecular markers to guide the treatment of pediatric central oligodendrogliomas.Gliomas are the most common malignant human brain tumors, and high-grade glioma (HGG) encompasses those with the worst prognosis, including anaplastic oligodendrogliomas (WHO grade III), anaplastic astrocytomas (WHO grade III) and glioblastomas (WHO grade VI).FGFR3 copy gain with an activating in-frame fusion of FGFR3-PHGDH. The critical signaling domains of FGFR remain intact, allowing for the potential utilization of FGFR inhibitors against this fusion. Gene rearrangements of FGFR have been associated with cholangiocarcinoma, breast cancer, bladder cancer, thyroid cancer, and prostate cancer.35 In addition, a sporadic FGFR1 mutation has been observed in two medulloblastomas, one low-grade glioma, and three glioblastoma cases.36 FGFR inhibitors were demonstrated to be effective in the clinical treatment of tumors with FGFR mutations.19Molecular analysis of our patient’s tumor demonstrated an 37 PHGDH has also been shown to induce proliferation and invasion in glioma cells.38 The molecular configuration of PHGDH has been described to primarily be composed of two substrate-binding domains, an NAD+/NADP+-binding domain, and a regulatory domain.39 The fusion we report would only leave a portion of the regulatory domain intact. No previous reports of an FGFR3-PHGDH fusion have been reported; however, our finding strengthens the role of PHGDH as a relevant tumor driver in human gliomas.Interestingly, PHGDH initiates a pathway that allows for the de novo synthesis of serine and has been recognized in an oncogenic and prognostic role in breast cancer, melanoma, cervical cancer, non-small cell lung cancer, and colon cancer.FGFR3 expression, the FGFR tyrosine kinase pathway was deemed as the likely tumor driver, encouraging our team to implement FGFR inhibitor therapy with ponatinib.This patient was a poor candidate for surgical resection of the tumor given that the tumor had infiltrated the patient’s bilateral thalami. This, in combination with the patient’s molecular findings, caused us to consider targeted therapies. Given that the patient’s tumor demonstrated increased 40 Ponatinib has also demonstrated potent inhibitory activity against FGFR1-4 with an IC50 of <40 nM.41 While ponatinib has not been tested in oligodendrogliomas, it has been shown to have in vitro activity against glioblastoma cells and has caused tumor reduction and apoptosis in a murine xenograft glioblasoma model.23 Importantly, in a murine model, ponatinib demonstrated excellent CNS penetration with a brain to plasma concentration ratio of 0.88.22 The ability of ponatinib to cross the BBB and achieve levels that are above in vitro IC50 levels ultimately influenced the decision to pursue treatment with ponatinib rather than pazopanib.42 Recently, a publication raised the question of CNS penetration in a case report of an adult with CNS-positive leukemia who failed to respond to ponatinib.21 As per our knowledge, this is the first published use of ponatinib for a primary CNS tumor. Future studies are warranted to further clarify its role in patients with brain tumors harboring FGFR alterations.Ponatinib is a tyrosine kinase inhibitor (TKI) that was initially approved to treat patients with chronic myelogenous leukemia (CML) and Philadelphia chromosome-positive acute lymphoblastic leukemia (ALL) that were resistant to BCR/ABL TKIs.FGFR3-PHGDH and our rationale for pursing personalized, target therapy for the patient’s tumor with ponatinib. Our work demonstrates the benefit of pursuing clinically integrated sequencing in high-risk pediatric brain tumors, strengthens the role of PHGDH as a relevant driver of human glioma, and reports a FGFR3-containing fusion that may play a role in similar cases.In conclusion, we report molecular data for a case of pediatric central oligodendoglioma with a fusion of Consent was obtained from the family of the patient at the time of enrollment for the sequencing of her tumor. The methods were performed in accordance with relevant guidelines and regulations and approved by the University of Michigan’s Institutional Review Board.All relevant data are available from the authors.Supplementary Materials


A 58-year-old man with advanced-stage pancreatic adenocarcinoma presented with fatigue and dyspnea. Examination revealed tachycardia (102 b/min) with mild tenderness in right upper quadrant. His hemoglobin (Hb) was 7.9 g/dL (10 days prior to presentation 12.2 g/dL), International normalized ratio (INR), platelet count was normal, and the stool guaiac test was negative. On admission, abdominal computed tomography (CT) scan showed hepatic metastatic lesion with a rupture and hemoperitoneum communicating to the subdiaphragmatic space. This rapid progression of anemia along with presenting symptoms and CT imaging were attributed to diagnosis of spontaneous rupture of liver metastasis from pancreatic adenocarcinoma. Patient received blood transfusion and hemoglobin was monitored in successive intervals. His general condition and anemia improved with conservative management and he was discharged in 3 days. Repeated CT after 4 months showed resolving hemoperitoneum and stable hemoglobin levels. The patient deceased 9 months after being diagnosed. A literature search revealed limited data regarding the incidence and management of spontaneous rupture of metastatic lesion secondary to pancreatic adenocarcinoma which has been managed conservatively and thus we are reporting our experience. Spontaneous ruptures secondary to malignant liver metastasis are uncommon occurrences that clinically present with similar symptoms/signs as the more common spontaneous rupture of liver secondary to hepatocellular carcinoma (HCC). HCC is consistently reported to have a poor prognosis and, due to its prevalence, continues to be the most lethal and life-threatening hallmark of advanced disease, attributing to 25–75% mortality in the acute phase HCC . Hepatic3; prothrombin time (PT): 14 s; INR: 1.08; activated partial thromboplastin time: 27.7 s; stool guaiac test that was negative; LFT's and RFT's that were within normal limits. 10 days prior to this presentation, his Hb and Hct levels were 12.2 g/dL and 37.7%, respectively, suggesting rapid progression of anemia. Abdominal contrast enhanced CT was immediately performed and revealed an enlarging hepatic metastatic lesion (78.9 × 68.8 mm) with a rupture , splenic artery, ascitic fluid, and diaphragm which was confirmed by biopsies at aforementioned sites. In the interim, he underwent several lines of chemotherapy, radiation, and molecularly targeted therapy. Surgical procedures included wedge resection of the left lung lesions, cervical corpectomy and fusion, abdominal paracentesis, and gastrostomy tube placement. Past medical history includes type 2 diabetes mellitus and tubulovillous adenoma for which he underwent polypectomy. On admission, the patient was in minimal distress with tachycardia (102 beats/min), blood pressure of 99/62 mmHg, and mild tenderness in the right upper quadrant of abdomen. Laboratory data revealed hemoglobin (Hb): 7.9 g/dL; hematocrit (Hct): 24.9%; platelet count: 210,000/mm rupture  and hemo rupture . Previou rupture . Follow- rupture .Considering the guarded condition and vitals being the lower limits of normalcy, the medical team consisting of a hospitalist, interventional radiologist, and oncologist decided upon managing the patient's treatment conservatively. The patient was given a blood transfusion; Hb and Hct were monitored serially in successive intervals during hospitalization. Subsequently, his general condition and anemia improved (Hb to 9.9 g/dL and Hct to 30.9%). Throughout his hospitalization, the patient's condition was stable and improved solely with conservative management. Since this approach was undertaken, there was no undue delay in resuming the patient's immunotherapy for pancreatic cancer. Repeated CT after 4 months showed resolving hemoperitoneum and stable Hb. The patient deceased 9 months after being diagnosed with hemoperitoneum secondary to metastatic hepatic rupture.Pancreatic cancer is one of the deadliest cancers, evidenced by the fact that its mortality equals its incidence . Early dPivotal clinical features of hemoperitoneum indicating hepatic metastatic rupture include history of malignancy, abdominal pain, hypotension, severe anemia, elevated liver enzymes, and, in extreme cases, surgical abdomen. Choi et al. reported that peripheral location of the tumor, protruding, contour, discontinuity of hepatic surface, and surrounding hemoperitoneum are helpful diagnostic indicators of ruptured HCC . The afoTreatment of hemoperitoneum secondary to spontaneous rupture of metastatic liver tumor depends on several factors, including tumor size, location, and severity of exsanguination. The major objective of treatment is to control the hemorrhage quickly and effectively; this can be accomplished by hepatic wedge resection/lobectomy or suture ligation of the bleeding source/hepatic artery. Recent studies suggested that a two-staged therapeutic approach in managing ruptured hepatic lesion consists of initial management with conservative approach, hemostasis achieved via transarterial embolization or surgery, followed by staged hepatic resection . TranscaHowever, conservative treatment focuses on achieving hemostatsis by correcting coagulopathy, close monitoring, and follow-up medical imaging to confirm hemostasis after initial resuscitation . A studyIn conclusion, we convey that spontaneous metastatic liver rupture secondary to advanced pancreatic adenocarcinoma is rare and has a poor prognosis. Clinical findings and CT are of great help in making a quick diagnosis of hemoperitoneum due to metastatic liver rupture. Although the literature supports invasive intervention (extrapolating from data for HCC) in advanced-stage pancreatic cancer, conservative treatment alone  can be employed to combat the sequelae of intraperitoneal hemorrhage and improve survival with fewer complications and minimal interruption or delays in necessary chemotherapy or cancer-directed treatments.


A previously healthy 10-year-old female presented to a local emergency department following three days of nausea and vomiting diagnosed with a solid pseudopapillary tumor. Solid pseudopapillary neoplasms are a rare form of pancreatic cystic neoplasm that typically presents in young females in their 20–30s and are very rare in children. These neoplasms often present as an asymptomatic tumor found on incidental imaging. When symptomatic they most commonly present with abdominal pain and can also cause a palpable abdominal mass, weight loss, gastrointestinal obstruction, and nausea and vomiting. Timely diagnosis of this rare neoplasm is very important because complete resection of the tumor is the definitive treatment and leads to an excellent long-term survival. A previously healthy 10-year-old female with no significant past medical history presented to a local emergency department following 3 days of nausea and vomiting with mild diffuse abdominal pain. Her mother reports that the patient had nausea over a 3-day period with frequent episodes of nonbloody, nonbilious emesis. The first day of illness she was vomiting every 20 minutes. The following 2 days she vomited only after eating and drinking. Due to her persistent emesis, her oral intake had significantly decreased by time of presentation. Although normally energetic, she developed significant fatigue. There was no change in mental status. The mother initially believed that her symptoms were infectious in nature as several of her peers also had nausea and vomiting. She was subsequently brought to the emergency department on day 3 of illness due to her fatigue, worsening diffuse abdominal pain, and parental concern for dehydration.On physical examination, she was afebrile with tachycardia to 120 and mild hypertension of 131/84. On abdominal exam she demonstrated periumbilical and right sided tenderness to palpation. She had normoactive bowel sounds without guarding or rebound tenderness. Psoas and obturator signs were negative. No masses were palpated and she did not have hepatosplenomegaly. The remainder of her physical exam was unremarkable.Mother denied family history of pancreatic cancer. Maternal aunt had pancreatitis requiring surgical intervention; further details are unknown.A complete blood count, comprehensive metabolic panel, and lipase were obtained, all of which were normal other than a significantly elevated lipase of 393. An abdominal ultrasound was obtained which showed a well-defined round heterogeneous mass in the head of the pancreas  without Mother denied family history of pancreatic cancer. Maternal aunt had pancreatitis requiring surgical intervention; further details are unknown.The patient underwent a magnetic resonance cholangiopancreatography (MRCP) the following day for further characterization of the lesion and to evaluate the biliary system. MRI demonstrated a 2.6 × 2.6 cm pancreatic head mass  with hetFour days later an endoscopic ultrasound with fine needle biopsy was performed. Pathology results were consistent with solid pseudopapillary tumor. The patient was discharged until a planned readmission 4 days later at which time she underwent a laparoscopic radical Whipple's (pancreaticoduodenectomy) for removal of the pancreatic mass. The surgery was completed successfully without complication.Pathology of the removed specimen demonstrated solid pseudopapillary tumor of the pancreas with extensive necrosis and negative margins. She recovered well from the procedure and was discharged 6 days later. At her follow-up appointment a month later, she had completely recovered without any sequelae. The mother reports that “it is like nothing ever happened.”Solid pseudopapillary neoplasms (SPNs) are categorized as a pancreatic cystic neoplasms and SPNs are the least common neoplasm within this group . Cystic While more symptomatic initial presentations were seen in the past, incidental diagnosis has become the most frequent presentation due to the widespread availability of sensitive cross-sectional imaging . When sySolid pseudopapillary neoplasms are a rare form of pancreatic cystic neoplasm that typically presents in young women and are very rare in children, with an incidence rate of 0.005–0.01 cases per 100,000 . These l


Myxomas are rare benign soft tissue tumors. The kidney is an unusual location for this tumor. For this reason, less than 15 cases of renal myxoma have been reported in the English literature. There are no specific clinical and radiological features reported for this tumor that allow a preoperative diagnosis enabling a conservative treatment.We report another case of renal myxoma found in a 50-year-old Moroccan woman who presented with a right dull flank pain. An abdominal computed tomography scan objectified a suspected malignant renal mass. Thus, radical nephrectomy was performed. Histopathology of the specimen revealed the typical appearance of a myxoma.The objective of this report is to add another case report of this rare benign renal tumor to the literature. This benign tumor with excellent prognosis has no specific preoperative features that could justify a conservative management; a radical approach remains the therapeutic option for now. Myxomas are rare benign soft tissue tumors. Renal myxomas are rare; very few cases have been reported in the English literature. There are no specific clinical and radiological features reported for this tumor that allow a preoperative diagnosis enabling a conservative treatment. We report a case of renal myxoma diagnosed after radical nephrectomy had been performed for a suspected malignant renal tumor. We present a computed tomography (CT) scan and the histopathological findings of the case.A 50-year-old Moroccan woman presented to our hospital for assessment of a right dull flank pain that had begun a year ago. She had a medical history of hypertension and type 2 diabetes mellitus and was under calcium channel blocker and antidiabetic medication respectively.A physical examination found no palpable mass in her abdomen but a slight right flank pain. All laboratory investigations were within normal limits. An abdominal CT scan objectified a hypodense well-defined mass in the mid-portion of her right kidney. It measured 4 × 3.5 cm and was slightly enhanced after intravenous contrast measuring 61 Hounsfield unit , and Ki-67 but stained negatively with pancytokeratin and CD 34 (Fig. She underwent radical nephrectomy for a suspected malignant renal tumor. The specimen was sent for histopathological examination:Thus, the final diagnosis of renal myxoma was made. She is well and asymptomatic at 3 months follow up.Myxomas are rare benign soft tissue tumors that mainly occur in the heart and skin, although various anatomical locations have been described for this tumor . Renal mThere are challenges with respect to its preoperative diagnosis as no specific clinical manifestation has been reported so far due to its rare occurrence, which limits its study. Documented cases of this tumor have shown nearly equal prevalence in males and females. Although the majority of reported cases of renal myxomas have been diagnosed incidentally, flank pain is the main clinical presentation in symptomatic patients for this tumor as in our case . UltrasoMyxomas of the kidney are very rare benign soft tissue tumors. Preoperative diagnosis of this disease allowing conservative treatment remains a challenge due to its very rare occurrence and its lack of specific clinical and radiological manifestations. Histopathological examination after radical nephrectomy remains the sure diagnostic tool for this disease. Advanced imaging modalities and investigations will probably help in diagnosing and managing this disease. This benign tumor has a good prognosis with no known recurrence.


Spontaneous regression has been defined as occurring when the malignant tumor mass partially or completely disappears without any treatment or as a result of a therapy considered inadequate to influence systemic neoplastic disease. Recently, studies have implicated immunological responses as likely being involved. We report a case of a patient with squamous cell carcinoma of the lung who experienced spontaneous regression following biopsy without other intervention.A 57-year-old white man was referred to our pulmonary clinic after an incidental finding of a nodule in the lower lobe of his left lung. Thoracic computed tomography revealed a 2.0 × 1.4 × 1.5 cm spiculated nodule in the superior segment of the left lower lobe. Workup identified the mass as a squamous cell carcinoma that was clinically staged as T1M0N0. The patient deferred treatment of this lesion. He undertook no significant lifestyle or medical changes. Three months later, computed tomography revealed that, compared with the initial study, the solitary mass had decreased in size to 1.6 × 0.9 × 0.9 cm. Follow-up computed tomography 1 year after the original workup demonstrated that the nodule had stabilized to its smaller size.Studies have shown that immunological response can be initiated by trauma to an area. Because the tumor regression became evident in our patient only after the tissue biopsy, his immune response to the surgical procedure seems to be a plausible contributor to the spontaneous regression. Further understanding of spontaneous regression can potentially impact the identification of neoplastic drug targets or even the course of a patient’s treatment plan and goals. Spontaneous regression (SR) of a tumor was originally defined as occurring “when the malignant tumor mass partially or completely disappears without any treatment or as a result of a therapy considered inadequate to influence systemic neoplastic disease” , 2. SR oA 57-year-old white man was seen in the pulmonary clinic of our institution after an abdominal/pelvic computed tomographic (CT) scan for microscopic hematuria incidentally demonstrated a nodule in the left lower lobe (LLL) of his lung. The patient reported periodic chronic cough but denied any hemoptysis or any other alarming symptoms. His comorbidities consisted of hypertension, hyperlipidemia, latent syphilis, obesity, and diabetes mellitus type 2 with peripheral neuropathy. His daily medications included aspirin, lisinopril, hydrochlorothiazide, gemfibrozil, metformin, glipizide and amitriptyline. He reported a greater than 40-year history of smoking an average of two packs of cigarettes per day. He lived alone at home and was able to perform all of his activities of daily living. He reported that both his parents had died from lung cancer due to tobacco use. His physical examination and laboratory results had no pertinent abnormal findings.A thoracic CT workup in April 2016 revealed a 2.0 × 1.4 × 1.5 cm spiculated nodule in the superior segment of the LLL  had evident findings, indicating that the mass was potentially quite extensive/severe or metastatic. Our patient’s cancer was clinically stage I. Patients in other case reports had more advanced stages , 7. CaseThe case of our patient meets the SR criteria discussed in this report. The workup of the nodule revealed squamous cell carcinoma of the lung in a very early stage. Surgical resection is the standard of care and was recommended to the patient multiple times, but he declined. It is inadvisable to recommend watchful waiting in a case such as this one, but our patient’s refusal of surgery allowed for the opportunity to follow this tumor after biopsy. Repeat imaging 3 months after biopsy with no further treatment revealed shrinkage of the mass. During this period, the patient also denied any changes in his daily medications or lifestyle. Because the tumor regression became evident after the tissue was biopsied, the patient’s immune response to the surgical procedure seems to be a plausible factor in the occurrence of SR in his primary lung tumor. Our patient with a tumor that would typically be resected but was followed with imaging after biopsy demonstrates the potential for regression following insult or damage to a tumor, and this case report adds to the growing body of knowledge that implies the possibility of effective and potentially novel immunological treatments of cancer.