A 53-year old man presented with seven months history of progressive weakness of extension of the digits and the thumb of the left hand. The wrist extension was normal and sensations were also intact. The patient had also been noticing a progressively enlarging lump on the lower anterolateral aspect of the left antecubital fossa for the last three months. Physical examination andelectro diagnostic studies revealed motor deficit along the posterior interosseous nerve (PIN) distribution with preservation of sensations. Also a soft tissue solitary lump (measuring 6×5 cm in its greatest dimensions) was palpable in the left antecubital fossa. The magnetic resonance imaging (MRI) of the forearm revealed a well-defined, non-enhancing, homogenous, fat intensity lesion in the left antecubital fossa, attached to the proximal radius. The patient underwent surgical excision of the lump with decompression of the PIN in the radial tunnel. Histopathology confirmed the diagnosis of parosteal lipoma. Although the diagnosis was elusive at the very outset, yet prudent clinical judgment, appropriate ancillary investigations and timely surgical intervention resulted in optimal functional recovery of the hand drop. There was complete motor recovery at 4-months follow up with no recurrence of the lipomaafter one year. The inciting cause is often unknown. The age group frequently affected is 40-60 years. The vast majority of patients present with a history of a slow-growing, painless and non-tender lump. Motor and sensory disturbances from compression of the adjacent nerves may occur during the course of its progression.3-6Parosteal lipoma is an extremely rare benign neoplasm composed of mature adipose tissue contiguous with underlying periosteal bone. It accounts for 0.3% of all lipomas.6-9The posterior interosseous nerve (PIN) arises from the bifurcation of the radial nerve in the proximal forearm. It is a pure motor nerve that innervates extensors of the forearm , brachioradialis, or anconeus) and the abductor pollicis longus (APL). Immediately distal to the bifurcation, the PIN travels through the radial tunnel, which is a 5-cm space dorsally floored by the capsule of the radiocapitellar joint, laterally bounded by the ECRL and extensor carpi radialis brevis (ECRB) muscles, medially bordered by the biceps tendon and brachialis muscle and volarly covered by the brachioradialis.6-9 To the best of our knowledge, there are only a handful of cases of PINS secondary to parosteal lipoma of the proximal radius reported in the published literature. Ours is the first case to be reported in this regard from any plastic surgical facility from Pakistan. This rarity prompts us to share our management experience.Within the confined space of the radial tunnel, there are five potential sites of PIN compression; i.e. a- the fibrous bands to the radiocapitellar joint between the brachialis and brachioradialis; b- the recurrent radial vessels (i.e. leash of Henry); c- the proximal edge of the ECRB; d- the proximal edge of the supinator (i.e. arcade of Fröhse); and e- the distal edge of the supinator. Compression of the PIN gives rise to either of the two distinct compression syndromes. i.e. The posterior interosseous nerve syndrome (PINS) and the radial tunnel syndromes (RTS). In contrast to PINS, the RTS is characterized by lateral proximal forearm pain with no discernible motor weakness. The management for both is the same.A 53-year old Pakistani male patient presented to our unit with seven months history of weakness of extension of the fingers and the thumb of the left hand. The difficulty in extension was of gradual onset and progressively worsening with the resultant gradual loss of manual dexterity. The wrist extension was normal, albeit with mild radial deviation of the hand on attempted extension. The patient was particularly concerned about his progressive hand drop. He also had been noticing a progressively enlarging lump on the lower anterolateral aspect of the left antecubital fossa for the last three months. The lump was painless, soft and slow growing. The patient had no history of any traumatic insult to the affected limb or any other concomitant illness. Neurological examination of the affected limb was performed. On the Medical Research Council scale , the power scores for the triceps, brachioradialis, and ECRL were all 5/5, however the scores for the ECRB, extensor digitorum communis, extensor carpi ulnaris, extensor pollicis longus and brevis, extensor indicis proprius, extensor digiti minimi and APL were all 2/5. The forearm supination was also weakened. There was no sensory deficit. There was a solitary, soft, well-defined lump in the left antecubital fossa, measuring approximately 6×5 cm in size. The lump was relatively fixed while the overlying skin was mobile. Positive Tinel signal on the PIN was elicit-able on tapping over the lump.Electromyography and nerve conduction studies revealed motor deficit of the PIN, consistent with the aforementioned findings on physical examination. The MRI of the forearm revealed a well-defined, non-enhancing, homogenous, fat intensity lesion in the left antecubital fossa. It was completely separate from the adjacent forearm muscles and attached to the proximal radius -3. The pWe undertook surgical exploration and excision of the lump under general anesthesia. An anterior approach was employed involving a curvilinear incision beginning proximal to the lateral epicondyle and continuing in the interval between the biceps and brachioradialis muscles and then curving 2 cm above the elbow flexion crease over the mobile wad and then medial to the border of the brachioradialis. The fascia along the brachioradialis muscle was divided and the muscle retracted laterally while retracting the biceps and pronator teres medially. The lump was carefully mobilized and excised by undocking the periosteal attachment. Care was taken to safeguard the overlying stretched branches of the PIN. Only bipolar electric cautery was used during the dissection to avoid any further nerve damage. Once the lump was removed, the nerve branches were traced distally in the interval between the brachialis and brachioradialis and their integrity confirmed visually. The fibrous bands overlying the PIN and the edge of the ECRB were released. The arcade of Fröhse was divided. The mobile wad muscles were retracted to expose the superficial head of the supinator, which was then divided. Thus the adequacy of PIN decompression was ensured by releasing all the potential sites of PIN compression. After ensuring adequate hemostasis the wound was closed in layers -6. On gross pathology examination, the excised lump was a well circumscribed mass measuring 6×5×4 cm in size . The cut10-12In our patient the PINS was caused by a parosteal lipoma of the proximal radius. The published literature has highlighted three major groups of causes of non-traumatic isolated PIN palsy. These include space-occupying lesions (SOLs), the supinator syndrome, and isolated neuritis. It is important to differentiate between these causes because the treatment differs accordingly. A variety of SOLs have been reported in the literature and include lipomas, ganglia, intracapsular chondromas, synovial hemangiomas, myxomas, and synovial chondromatosis. The supinator syndrome occurs when the PIN is compressed by the proximal boundary of the supinator muscle.6 have classified PIN palsy into three types of (i) Type 1 (drop of fingers and thumb) caused by a simultaneous compression of the recurrent branch and the descending branch of PIN at the entrance point and within the supinator; (ii) Type 2 (drop of fingers) caused by compression of the recurrent branch only; and (iii) Type 3 (drop thumb) caused by compression of the descending branch only.6In our patient we found drop of all the digits and the thumb of the affected left hand as both the medial and lateral branches of the PIN were unduly stretched by the underlying parosteal lipoma. We termed it as “hand drop” in order to differentiate it from wrist drop of the radial nerve palsy. Suematsu Netal13-15In our patient we performed MRI scan as it is superior to the CT scan for the evaluating a parosteal lipoma. The lesion is identified on the MRI as a juxta-cortical mass with signal intensity identical to that of subcutaneous fat, regardless of the pulse sequence. Heterogeneity in these lesions may be present and corresponds to the pathologic components in the lesion. Areas with intermediate signal intensity on T1-weighted images that are high signal intensity on T2-weighted images will represent the cartilaginous components in parosteal lipoma. Fibrovascular septae may cause a lobulated appearance of the fat component, with low-signal-intensity strands on T1-weighted images that become higher in signal intensity on the long TR images (particularly with fat suppression).13-15Larger areas of bone production surrounded by the lipomatous components will also well demonstrated on MRI. Adjacent muscle atrophy, poorly demonstrated by CT scan, is better identified on MRI as increased striations of fat in the affected muscle and may be caused in part by the associated nerve entrapment. This finding is best appreciated on T2-weighted images because of the decreased signal intensity of normal muscle relative to fat. The MRI also best demonstrates the relationship of the lesion to the underlying native bone and the adjacent muscles and this information is important for surgical planning as the parosteal lipoma is usually firmly adherent to the underlying cortex at the site of surface bone production.1,5The definitive treatment of parosteal lipoma causing PINS is complete surgical excision of the lipoma, as we did in our patient. We carefully separated and retracted the overlying stretched branches of the PIN to ensure compete excision without causing any further damage to the nerve. Also we carefully employed bipolar cautery to avoid any risk to the nerve by monopolar cautery. The bony attachment of the parosteal lipoma to the proximal radius was separated using subperiosteal dissection with the help of an osteotome. The published literature has described surgical removal of the bony stalk of a parosteal lipoma by using either a subperiosteal dissection (which entails the separation of the lesion from the underlying bone using an osteotome) or segmental resection of the source bone. This latter part of the surgical excision is an additional step to the relatively easy dissection usually performed for a soft tissue lipoma elsewhere in the body.et al.16 who have also reported full recovery in 24 out of 25 patients who underwent surgical decompression of the PIN. However Vrieling et al.17 have reported relatively less favorable results among their patients. The difference in the reported outcomes is probably due to variability in the timing of the surgical intervention. Intuitively early surgical intervention is crucial for achieving optimal outcome. If surgery is unduly delayed for approximately 18 months, fibrosis of the affected muscles may occur, making tendon transfers the only viable option for achieving functional restoration.17We observed complete motor recovery of the PIN four months post operatively. Our outcome results conform to Hashizume Although the diagnosis of parosteal lipoma causing PINS happens to be an elusive one at the very outset, yet prudent clinical judgment, use of appropriate ancillary investigations and timely surgical intervention results in optimal functional recovery of the hand drop. Any patient presenting with hand drop (i.e. inability to extend the digits and thumb) with intact wrist extension and intact sensations, should be suspected of having PINS and a careful search for possible etiology in the cubital fossa be performed.The authors declare no conflict of interest.


The neurofibromatosis is a frequent and polymorphic genetic disorder. The severity is related to the complications. The degeneration of neurofibroma is a very rare complication of neurofibromatosis. In the literature, a few cases of solitary neurofibroma, which turned into a malignant tumor were reported. In our case, we described a very rare clinical case of neurofibrosarcoma in the scalp, and surgical treatment. One of the more morbid complications is malignant degeneration of plexiform neurofibromas.5 The malignant Schwannoma is one of the forms of degeneration of the neurofibroma in neurofibrosarcoma.6 Here, we described a very rare clinical case of neurofibrosarcoma in the scalp, and surgical treatment.Neurofibromatosis is characterized by café-au-lait spots, lentigines and neurofibromas; with eye signs like Iris hamartoma; also CNS glioma and bone signs like dysplasia, pseudoarthrosis and scoliosis.We report our experience with a forty-three years old patient, known carrier of Neurofibromatosis type 1, who presented to us with a large occipital tumor. Biopsy revealed presence of malignant Schwannoma . The tre7-9 Histologically, the neurofibrosarcoma is characterized by the presence of fusiform cells, probably derived from Schwann cells. Collagen fibers are rare. There is a presence of coarse reticulin fibrils, in parallel rows between fusiform cells which is very characteristic of the lesion. Signs of malignancy are represented by pleomorphic cells, giant cells, mono or polynuclear cells, an excess of mitoses, an invasion of surrounding tissues, and vascular invasion.10,11The degeneration of neurofibromas is a rare complication of neurofibromatosis, and the lifetime risk for a patient of NF1 is of the order of 3 to 5 percent.12 The classic treatment of cephalic and scalp involvement of the malignant Schwannoma is not codified. It is variable from patient to patient, depending on the location and the level of involvement of structures. Whatever the histological grade, treatment consists of wide local excision, complete with radiotherapy for residual microscopic disease. First line chemotherapy can be tested to facilitate resection, however the rate of response is only 25-30 percent.13The degeneration of a plexiform neuroﬁbrome must be considered specifically because there is usually a rapid increase in size and volume of the lesion. There is usually also presence of pain, induration and neurologic signs. 5-year survival rates range from 48 to 58%. Recurrence rates vary from 38 to 45 percent and overall survival at 10 years does not exceed 20 to 40%. Hence, frequent monitoring is essential.13 Surgical excision followed by suitable reconstruction is essential.14 The surgical reconstructive options are based on the extent of the disease and the resection margins, the viability of the surrounding local tissues, the local and the regional or microvascular flap options as well as the hemodynamic status of the patient. Neurofibromatosis is a common pathology, however degeneration of plexiform neurofibromas remains a serious and often underestimated complication. Prompt evaluation, wide surgical excision and reconstruction is essential to minimize mortality and morbidity.The challenge in reconstructive surgery is to adapt processes to deal with the various problems posed by this disease.


A 68-year-old female was diagnosed with giant cell tumor of bone (GCTB) metastatic to her lungs. The patient was treated with IV denosumab for the course of 4.5 years for these metastases. The metastatic tumor burden decreased significantly after only 3 months of therapy. The size of the metastases has been stable for over 4 years. Denosumab therapy has promise in the treatment of GCTB, including pulmonary metastasis. However, the long-term role of denosumab for pulmonary metastases is yet to be determined.  Giant cell tumor of bone (GCTB) is a benign yet aggressive lytic tumor of bone occurring in the metaphysis and epiphysis of long bones. The most common locations involved are the distal femur, proximal tibia, and distal radius . Such deThe mainstay of treatment of GCTB pulmonary metastasis is surgical . Since pThe histologic composition of GCTB consists of sheets of neoplastic spindle shaped mononuclear cells with high levels of RANK Ligand (RANKL) expression, intermixed with large osteoclast giant cells , 12. RANOur case is a 68-year-old female with a past medical history of hyperlipidemia and insulin-controlled type 2 diabetes mellitus who presented with right knee pain and swelling. She denied any history of trauma. Radiograph and magnetic resonance imaging were concerning for GCTB. She underwent curettage and cementation of the right distal femur lesion and histological examination confirmed the suspected diagnosis of GCTB. Approximately one year later, she developed local recurrence of the tumor, which was also curetted with additional use of cement. One year after the second surgery, a routine chest radiograph showed lung lesions suspicious of metastatic disease . At the After discussion at a tertiary center musculoskeletal tumor board the patient was started on denosumab therapy. Treatment was initiated with a loading dose of 120 mg every week for three weeks. She was then transitioned to receive a dose of 120 mg every four weeks. She was also placed on daily calcium and vitamin D supplements.At 3 months after the initiation of treatment she had repeat CT imaging of her chest. The scan showed the RLL mass had decreased considerably in size, now measuring 2.6 cm in its greatest dimension. There was also a decrease in size of the other pulmonary lesions . No new Forty-eight months after initiating treatment, CT imaging showed the stable RLL mass as well as the smaller pulmonary lesions to be unchanged in size .We present a case of metastatic GCTB successfully treated with denosumab for 4 years. A literature search was able to find only one similar case. This was in a 17-year-old female who was followed for only three months while on denosumab . Our patDosing of the denosumab was also different in the two cases. In our patient, we followed the treatment regimen first described by Thomas et al. which consists of three loading doses of 120 mg seven days apart followed by one dose every four weeks . FurtherA denosumab safety study conducted by Chawla et al. showed low rates of hypocalcemia, osteonecrosis, neutropenia, hypophosphataemia, anemia, back pain, and pain in the extremities , 21. PatWhile denosumab has proven to be effective in reducing metastatic tumor size through interfering with osteoclastic differentiation, it does not target the neoplastic stromal cells, which will continue to proliferate with discontinuation of therapy. It is unknown if patients require chronic denosumab therapy or if the rate of neoplastic stromal cell proliferation can be reduced sufficiently with initial treatment that patients can stop therapy without progression of disease . Recent Additionally, there is a theoretical increased risk of pathologic fracture with chronic use of medications similar to bisphosphonates. Therefore, we had considered discontinuing denosumab after three years of therapy. However, due to a report in the literature of critical hypercalcemia following cessation of denosumab , the lacOther questions concerning denosumab therapy remain. More studies are needed to determine its application in all the stages of GCTB. We are hopeful denosumab can be a reasonable alternative to surgical treatment for pulmonary metastasis . The freDenosumab therapy has promise in the treatment of GCTB, including pulmonary metastasis. However, there is still much to do in defining its use in treating the different stages of GCTB. Also needed are long-term studies further defining its optimum dose, side effects, and how the disease progresses after discontinuation.


Marginal zone lymphoma (MZL) describes a heterogeneous group of B-cell lymphomas derived from marginal zone cells found in the spleen's white pulp and surrounding germinal centers. To date, only one case of spinal primary MZL has been reported. We report a second case. A 67-year-old man without special medical history was referred to our clinic because of progressive paralysis first noticed 2 months before hospitalization. On examination, his paralysis concerned the lower limbs. No reflex deficits were identified. No further neurological deficits were found. Spinal MRI in sagittal sequence in weighting T2 (A) and sagittal sequence (B); coronal (C) and axial (D) T1-weighted after gadolinium injection showed dorsal compression by an extensive posterior epidural tissue process from T6 to T8 in continuity with left pleural neoplastic thickening through the inter-vertebral homolateral foramens. A laminectomy with resection of the intra-ductal lesion was performed, the examination of which found a MZL. In an extension study, thoraco-abdomino-pelvic CT showed medial and celiac lymphadenopathy, a tissue sleeve of the left costo-vertebral gutter extended to a height of 9 cm. An osteo-medullary biopsy revealed a marrow of normal wealth without sign of malignancy. The lymphoma was classified in bone IV stage. The patient was treated according to the 2013 National Adult Non-Hodgkin's Lymphoma protocol. He received 8 RCHOP cures. The evolution was marked by the improvement of neurological signs with no lesion of evolutive suspicious nature at the thoracic and abdominopelvic stages at the end-of-treatment scan.


Pseudomyxoma peritonei (PMP) and extragastrointestinal stromal tumors (E-GISTs) are both rare entities. Most of the time, PMP is associated with an appendiceal tumor. An ovarian mucinous tumor can mimic appendiceal metastases. E-GIST is a mesenchymal tumor that can arise from the omentum, retroperitoneum, mesentery, or pleura. We present a case of an 87-year-old woman with mucinous carcinomatosis and acute intestinal occlusion submitted to an emergency laparotomy. She has found to have a borderline mucinous tumor of the ovary from the intestinal type with several lesions of pseudomyxoma peritonei and an E-GIST from the epiploons retrocavity (intermediated risk). In the literature, no relation was found between these two rare tumors. E-GIST was an incidental finding in the context of a mucinous carcinomatosis. Mucinous carcinomatosis or pseudomyxoma peritonei (PMP) is an accumulation of mucinous fluid in the abdominal cavity. Most cases are caused by mucin-producing tumors of the appendix and ovary . MucinouWe present the case of an 87-year-old woman with a relatively good performance status brought to the emergency room with progressive asthenia, weight loss, and abdominal distension for the last two months. She had a previous diagnosis of diabetes mellitus under oral antidiabetics and hypercholesterolemia and no previous surgical procedures. On physical examination, she was pale, emaciated, afebrile, and with abdominal distension for ascites without tension. Edema in the lower members was also present.On laboratory workup, a minor microcytic anaemia was detected; renal function, liver markers of cytolysis, and inflammatory markers were normal. Tumor markers, such as carcinoembryonic antigen and carbohydrate antigen 19.9 and 125, were within normal range. She was admitted for further workup.Abdominal ultrasound described a mass between the stomach and pancreas with about 9 cm and large volume ascites. Abdominal CT scan described a lobulated mass anterior to the pancreas with 8.5 × 9.5 cm, signs of chronic liver disease, and confirmed large volume ascites . EndoscoOne week after admission, the patient had persistent vomiting and dyspnea caused by increased abdominal distension. An emergency laparotomy was performed. A total of 12 L of mucinous ascites  was remoPseudomyxoma peritonei is a clinical condition more frequently originated in a perforated mucinous tumor of the appendix. The accumulation of gross mucinous ascites can lead to adhesions and cause intestinal obstruction.The clinical course is insidious, and multiple recurrences are common. Surgery with cytoreductive therapy and intraperitoneal chemotherapy is the approach with better outcomes described.A primary ovarian tumor rarely causes PMP and the most frequently associated subtype is the mature teratoma , 9. It iSome available data suggested that “borderline” mucinous tumor from the ovary can be originated from a primary appendiceal lesion. When the lesion presents benign and borderline areas and smooth surface and is unilateral and limited to the ovary, these characteristics point to ovarian origin. Pathologists use a panel of immunohistochemical markers for ovarian origin  even though some combinations can be shared with other tumors . Metastatic lesions are more common and usually present as small diffuse lesions with an infiltrative pattern of stromal invasion .In the case presented above, the appendix was not resected because it did not show abnormal appearance and a unilateral lesion in the ovary was resected.The other tumor removed was characterized as an E-GIST, a mesenchymal tumor that originates from interstitial cells of Cajal and arises outside the GI tract. While some authors consider that E-GISTs may originate as primary tumors from mesenchymal stem cells of the greater omentum and mesentery, others consider that these tumors derive from the gastrointestinal tract like the other GISTs and may represent a metastatic stage .The tumor could be asymptomatic for a months or years and sometimes can be palpable. The presence of symptoms is associated with a large lesion. Most of cases are diagnosed through CT scan. The lesions are usually single, well delimited, and with low density .n = 15) followed by the retroperitoneum (n = 13) and omentum (n = 8).Yi et al.  publisheSeveral case series studied grading systems for these tumors. Usually, tumor size and mitotic rate are used to classify the other GISTs. As we discussed above, E-GISTs are more frequently symptomatic only when they are already large masses. Therefore, size is not probably as useful for grading as for the other GISTs. Mitotic rate, cellularity, and Ki67 expression seem to be the most significant prognostic factors. In most cases, E-GISTs are large tumors associated with high proliferative rates, sometimes with lymph node and distant metastasis, which entails a worse prognosis , 16.Yamamoto et al.  defined Surgical resection is the treatment of choice. After the surgery, patients are selected for treatment with inhibitors of tyrosine kinase that follows the NIH criteria: tumor size, mitotic rate, and anatomic location. Our patient's tumor was classified as moderate risk, and no adjuvant treatment was given.No relationship between a borderline ovarian mucinous tumor and an E-GIST was found in the literature , 18. In


Primary salivary gland–type tumors of lung are rare. Epithelial–myoepithelial carcinoma (EMC) of the lung is a minor salivary gland–type tumor subtype.We report a very rare case of EMC located in the peripheral left lower lobe that was diagnosed in a 58-year-old man and this is the first study in which we summarize all the patients with primary peripheral lung EMC concerned with the clinical features. Informed consent was obtained from the patient.Chest computed tomography displayed an anomalous soft tissue mass with slightly lobular borders in the peripheral segment of the left lower lobe and closed to the visceral pleura. The surgery was performed by using video-assisted thoracic surgery. Grossly, the tumor was solitary, well-circumscribed, and unencapsulated endobronchial lesion. A microscopic examination revealed that it was circumscribed, although the tumor borders may show single cells or clusters of cells proliferating away from the main tumor mass. The inner tubular layer showed epithelial cell characteristics, whereas the outer layer exhibited myoepithelial cell characteristics. Immunostaining for P40, P63, and cytokeratin 5/6 was positive. However, the anaplastic lymphoma kinase-V, thyroid transcription factor-1, synaptophysin, chromogranin A and napsin A were negative.Literature review showed that most of patients with peripheral EMC were asymptomatic. Computed tomography and magnetic resonance imaging scans are able to indicate the presence of peripheral EMC. Pathological analysis is an effective method to clarify the diagnosis. Surgery is a regular treatment method. To facilitate the preoperative diagnosis and avoid the misdiagnosis of such a rare disease, more cases will need to be reported. Epithelial myoepithelial carcinoma (EMC) of the lung is a minor SGTT subtype. About 120 cases have been reported in the world literature, most of which were located in salivary glands, except for a few cases occurring in unusual locations such as trachea and bronchus.–4 EMC is pathologically characterized by a dual cell population, including an inner layer of cuboidal epithelial cells that are peripherally bounded by a layer of myoepithelial cells.,6 Herein, we report a very rare case of EMC located in the peripheral left lower lobe that was diagnosed in a 58-year-old man and this is the first study in which we summarize all the patients with primary peripheral lung EMC concerned with the clinical features.Primary salivary gland–type tumors (SGTTs) of lung are rare, accounting for 0.1% to 0.2% of all lung tumors.2A 58-year-old man was referred to our hospital for a routine health check. He denied symptoms, including chest pain, cough, and dyspnea. He was a nonsmoker. Physical examination revealed normal breathing sounds in both lung fields. Laboratory findings were within normal limits. His pulmonary function tests revealed normal performance. Chest computed tomography (CT) displayed an anomalous soft tissue mass with slightly lobular borders in the peripheral segment of the left lower lobe and closed to the visceral pleura Fig. A and B. As a diagnosis was not established, surgery was scheduled. We approached the tumor by using video-assisted thoracic surgery (VATS). The surgery was performed in the lateral decubitus position. Grossly, the tumor was solitary, well-circumscribed, and unencapsulated endobronchial lesion, measuring 1.3 × 1.1 × 1.2 cm. A microscopic examination revealed that it was circumscribed, although the tumor borders may show single cells or clusters of cells proliferating away from the main tumor mass . This tumor does not seem to be related to cigarette smoking.,10 As seen in Table Epithelial–myoepithelial tumors are rare neoplasms that occur more commonly in salivary glands, where they represent approximately 0.5% of primary tumors.,18 On MRI, the T1-weighted sequence showed the mass mostly with isosignal intensity to muscle tissue and partially with slightly higher signal intensity. The T2-weighted sequence of the mass showed heterogeneous high signal intensity, along with clear visualization of the septa and capsule-like structures. Contrast-enhanced MRI showed strong heterogeneous enhancement of the mass. Zhu et al reported that positron emission tomography–CT images show an oval nodule with slight but obvious increased (18)F-fluoro-2-deoxy-d-glucose uptake in the lobe. Most of the previously reported cases of pulmonary EMC have formed intraluminal polypoid masses occluding the lumen of the bronchus and they can be found by fiber-optic bronchoscopy. There are 6 tumors located in pulmonary parenchyma in our summary (cases 1–6). The patient we present in this report is the seventh reported case of a tumor located in the left lower peripheral pulmonary parenchyma. In this case, fiber-optic bronchoscopy examination is normal and chest CT displayed an anomalous soft tissue mass with slightly lobular borders in the peripheral segment of the left lower lobe and closed to the visceral pleura.As these tumors are rare, criteria for diagnosing EAC radiologically do not exist. EMC can be dependably diagnosed by CT, magnetic resonance imaging (MRI), and positron emission tomography–CT. Some reports showed that the margin of the mass was relatively well defined, and the internal CT density indicated a mixture of cystic and solid components.,21 However, in this case, the tumor presents as an intraparenchymatous mass without apparent connection with bronchus. Its size varies, ranging from 1 to 16 cm in diameter.–24 Microscopically, it is a multinodular lesion presenting a bicellular pattern of proliferating epithelial cells with eosinophilic centrally located nucleus and myoepithelial cells with abundant clear cytoplasm. Immunohistochemically, the epithelial component is positive for keratin and carcino-embryonic antigen (CEA), while myoepithelial component is positive for S-100, p63, and CD10. Epithelial cells are positive for TTF-1, unlike myoepithelial cells. As seen in our case, P40, P63, and cytokeratin 5/6 were positive and the anaplastic lymphoma kinase-V, TTF-1, synaptophysin, chromogranin A, and napsin A were negative.In most reviewed cases, an evident relation and connection with the bronchial tree is observed. Adenoid cystic carcinoma is characterized by bands or cylinders of hyalinized or mucinous stroma separating or surrounded by nests or cords of small epithelial cells. When the cylinders occur within masses of epithelial cells, they give the tissue a perforated, sieve-like, or cribriform appearance. Such tumors occur in the mammary glands, the mucous glands of the upper and lower respiratory tract, and the salivary glands. They are malignant but slow-growing, and tend to spread locally via the nerves.The differential diagnosis of this disease includes pleomorphic adenomas and adenoid cystic carcinomas. Pleomorphic adenoma is a benign, slow-growing tumor, most commonly of the salivary gland, occurring as a small, painless, firm nodule, usually of the parotid gland, but also found in any major or accessory salivary gland anywhere in the oral cavity. Their study indicated that the tumors could have malignant potential, based on the malignant behavior of EMC of the salivary gland. EMC of the salivary gland demonstrated a local recurrence rate of 23% to 80% and a 14% to 25% rate of metastasis. The biological behavior of pulmonary EMC is still unclear. Pelosi et al found out that the protein p27/kip-1 is a cyclin-dependent kinase inhibitor that blocks cell cycle in G0 and G1. Arif et al showed the direct in vivo evidence that p27/kip-1 also functions as an oncogene. An abnormal subcellular location of p27/kip-1 into the myoepithelial cell would provoke the loss of its growth inhibition function through the lack of restriction of proliferation of myoepithelial component.Although a variety of names have been attached to these tumors, the term EMC of the bronchus is preferred because these tumors are potentially malignant, despite the majority behaving in an indolent fashion. Malignant EMC of the lung has been debated by many authors, and Wilson and Moran suggested that this entity be identified as a carcinoma to convey the malignant potential of these tumors to clinicians and patients. None of the reported cases had evidence of disease during their follow-up periods. The patient we describe is still alive without recurrence or metastasis 8 months after undergoing pneumonectomy by VATS. VATS has become a widely accepted alternative for the resection of thorax tumors. Compared with open thoracotomy, this minimally invasive procedure was related with many advantages, such as less trauma and faster recovery. With the development of thoracoscopic instruments and techniques, the application of uniportal VATS has become the regular method, especially for the peripheral pulmonary neoplasm. Adjuvant radiotherapy is also required in the case of 4-cm or larger primary tumors or positive surgical margins. Chemotherapy is used in patients with advanced disease or surgically unresectable disease, with various agents alone or in combination .Although optimal therapy for pulmonary EMC has yet to be defined, a clinical course similar to that of salivary EMC may be expected. However, one of the characteristic features of salivary gland EMC is the long interval between diagnosis and recurrence and metastasis. Considering the similar histopathological features of pulmonary EMC and salivary EMC, recurrence and metastasis can also be expected in pulmonary EMC in the long term. It appears that lobectomy or pneumonectomy and sometimes sleeve resection may be the treatment of choice. The follow-up periods of the reported cases range from 6 to 36 months.4Primary lung tumors mimicking the salivary gland–type neoplasms are extremely rare. Primary peripheral EMC is an uncommon tumor in this group, and only 6 cases have been reported. Most of patients with peripheral EMC were asymptomatic in our summary. CT and MRI scans are able to indicate the presence of peripheral EMC. Pathological analysis is an effective method to clarify the diagnosis. Surgery is a regular treatment method. To facilitate the preoperative diagnosis and avoid the misdiagnosis of such a rare disease, more cases will need to be reported.


The Currarino triad is a rare hereditary syndrome comprising anorectal malformation, sacral bony defect, and presacral mass. Most of the patients are diagnosed during infancy.A 44-year-old man was diagnosed with Currarino triad, with a huge presacral teratoma and meningocele. One-stage surgery via posterior approach was successful.Treatment of the presacral mass in the Currarino triad, diagnosed in adulthood, is challenging. Multidisciplinary management and detailed planning before surgery are important for a satisfactory outcome. The Currarino triad was first described by Currarino et al. in 1981 . In moreA 44-year-old man complaining of intermittent constipation for 3 years presented to his local hospital with an increased severity of constipation of 3-month duration. Computed tomography (CT) scan showed a large presacral mass measuring 9 cm in diameter, and he was then referred to our hospital. His past medical history was significant for colostomy performed just after birth for anal atresia and anorectoplasty with stoma closure when he was 3 years old. He had occasionally, several times a year, suffered from incontinence. In his family history, his father died of lung cancer at the age of 65, and his mother was 80 years old and healthy. He had no siblings or children. He had no family history of hereditary diseases. He was not receiving regular medications.Physical examination of the abdomen showed a surgical scar extending from the left upper to lower quadrants without palpable masses. At the perineum, the rectal mucosa was anastomosed to the skin by the previously performed anoplasty. Digital rectal examination showed that the aperture of the rectum (plastic anus) was hard and that the lumen of the rectum was narrow. An elastic hard presacral mass was palpable at 4 cm from the anal verge. He could voluntarily constrict the anal sphincter. The rest of the physical examination, including urinary and genital systems, did not reveal any abnormalities.Laboratory analyses were normal, including tumor markers .Anorectal manometry revealed an impaired anal function; the maximum static pressure was 22 mmHg , and the voluntary contraction pressure was 43 mmHg .Colonoscopy and contrast enema showed that the lower rectum was stenotic but with intact mucosa.CT scan showed a partial defect in the right side of the sacrum with deformity, a 60 × 30 × 21-mm meningocele which was protuberated anteriorly from the level of the S3 vertebra, and a 90 × 85 × 68-mm cystic mass which was located anterior to the meningocele, compressing the rectum ventrally (Fig. Fluorodeoxyglucose-positron emission tomography showed no abnormal uptake. Myelography showed an inflow of the contrast agent into the meningocele, but not into the presacral tumor.Resection of the presacral mass was performed, based on a diagnosis of Currarino syndrome associated with presacral teratoma and meningocele. A postsacral midline incision was made with the patient in the jackknife position. After S3-4 laminectomy, the meningocele was dissected by ligating the root at the level of the dura. Then, the removal of the coccyx with an extended incision provided excellent visualization of the teratoma (Fig. Pathological examination revealed a mature teratoma Fig. . The cysOral intake was started on the fourth postoperative day. He was discharged from the hospital on the 15th postoperative day with no morbidity. His constipation dramatically improved postoperatively. No urological or rectal complications, or lower extremity neuropathy, were observed.The presacral mass associated with the Currarino triad is reportedly a teratoma, hamartoma, neuronteric cyst, anterior meningocele, or a combination of these lesions . TreatmePosterior, transabdominal, and combined approaches were previously reported for the surgical resection of presacral tumors. Table In our case, a posterior approach was the best to avoid spinal leakage or possible meningitis caused by injuring the meningocele. Closure of the meningocele from the posterior side was necessary before removal of the teratoma. In addition, adhesion between the teratoma and the lower rectum was expected because surgery for anal atresia was performed during childhood. The partial defect of the sacrum, as in our case, also favors the posterior approach. Though there are some reports of laparoscopic en bloc resection of teratoma , 8, ruptAdding coccygectomy to the resection of congenital presacral tumors remains controversial. Traditional treatment of sacrococcygeal teratomas favors removing the coccyx because the totipotential cells are believed to arise from the coccyx . HoweverIn conclusion, although the treatment of the presacral mass with Currarino triad diagnosed in adulthood is challenging, multidisciplinary management can allow feasible removal of the tumor via the posterior approach.


Current treatment options for lymphatic malformations (LMs) are multimodal. Recently, the effectiveness of treating LMs with Eppikajyutsuto (TJ-28) has been reported. TJ-28 is a kind of oral herbal medicine classified as the traditional Japanese Kampo medicine.A 12-year-old girl was admitted to our hospital for intermittent upper abdominal pain. Radiological examinations revealed a large (9.5 × 5.8 × 10.0 cm) retroperitoneal LM, which was suspected to adhering and stretching both pancreas head and duodenum. The large retroperitoneal tumor resection might induce involving complications because of the size and the location. Therefore, we used TJ-28 in order to diminish the tumor size before surgery. The patient received oral doses of 7.5 g/day (2.5 g × 3 times/day) of TJ-28. Six months after the medication, the tumor decreased markedly to 3.5 × 1.5 × 1.2 cm in size. Thereafter, the mass was sub-totally resected (95%) via a 3 cm trans-umbilical incision without any surgical complications.We reported a case of successfully treated retroperitoneal LM with the combination treatment of TJ-28 and surgery. Based on our experience, this TJ-28 treatment option may be very useful in treating cases of LMs having surgical difficulties because of size and/or location. Lymphatic malformations (LMs) usually appear as soft compressive masses at birth and are found in all age groups and in various parts of the body. LMs develop in various spectrums from localized masses to diffuse infiltration. Morphological types of LMs are categorized according to size and location, i.e., macrocystic, microcystic, mixed macrocystic and microcystic, and diffuse types , 2. AlthA 12-year-old girl was admitted to our hospital for intermittent upper abdominal pain, which continued for 3 days. The patient had no past medical histories. On admission, the patient had upper abdominal pain, nausea, and vomiting. The patient had no history associated with antecedent infections or abdominal contusions. A physical examination revealed a smooth and elastic hard mass measuring 10 cm in diameter at the center of her right flank. The patient did not have fever elevation. The patient had spontaneous pain and tenderness on and around the mass. Except for a slight elevation of CRP level, which was 1.22 mg/dl, her laboratory data indicated normal and the levels of tumor makers were also within the normal range. Ultrasound sonography (US) showed a large mass consisting of multi-macrocystic lesions. Contrast CT revealed a well-circumscribed multicystic mass measuring 9.5 × 5.8 × 10.0 cm in the retroperitoneal region. The mass, which is filled with serous fluid without hemorrhage or abscess, was suspected to adhering and stretching both pancreas head and duodenum  consists of six crude drug (herbs) components including Sekko, Mao, Sojutsu, Taiso, Kanzo, and Shokyo. TJ-28 regulates and reduces redundant body fluids and also has anti-inflammatory effects. Therefore, TJ-28 is usually used to treat edema, excessive sweating, thirst, and decreases in urine volume. In addition, recently, several investigators have reported on the effectiveness of TJ-28 in treating LMs , 8. The  consistsOur objectives include realizing a better cosmetic outcome as well as minimizing surgical complications.We reported a case of successfully treated retroperitoneal LM with the combination treatment of TJ-28 and surgery. Based on our experience, this TJ-28 treatment option may be very useful in treating cases of LMs having surgical difficulties because of size and/or location.


Unicystic ameloblastoma is a distinguishable entity of ameloblastomas, characterized by slow growth and being relatively locally aggressive. Three histological types are recognized according to the degree of ameloblastomatous epithelial extension, namely, luminal, intraluminal, and mural types. This classification has a direct bearing on their biological behavior, treatment, and prognosis. However, there is difficulty in determining the most appropriate form of treatment for unicystic ameloblastoma. We present a case of unicystic ameloblastoma that occurred in the right posterior mandible of 19-year-old girl, which was enucleated and did not recur after 12-month follow-up.  Ameloblastomas are the most common form of aggressive benign tumors of the jaws . The ameA 19-year-old girl was undergoing orthodontic treatment, and the panoramic radiograph showed the presence of a unilocular radiolucent lesion in the right mandibular ramus, involving the impacted tooth 48 . There wThe clinical diagnosis was a dentigerous cyst and the patient underwent enucleation of the lesion. During surgery, the cystic lesion, which enclosed a permanent lower first molar, was easily separated from the surrounding bone since it had an evident capsule. Tooth 48 was also extracted. The entire specimen was then submitted for histopathologic examination.Microscopically, cystic cavity lined by epithelium was observed in which the basal cells were columnar, hyperchromatic, and palisaded and with reverse polarity Figures . In someThe patient was followed up and 12-month later no sign of recurrence was detected .Unicystic ameloblastoma is a rare variant of ameloblastoma that was first described by Robinson and Martinez in 1977, referring to those cystic lesions that show clinical and radiologic characteristics of an odontogenic cyst but in histological examination it shows a typical ameloblastomatous epithelium lining part of the cyst cavity, with or without luminal and/or mural tumor proliferation , 5.Based on the character and extent of tumor cell proliferation within the cyst wall, several histologic subtypes of unicystic ameloblastoma are recognized, which include those of simple cystic nature, those with intraluminal proliferation nodules, and those containing infiltrative tumor islands in the cyst walls , 7.According to Philipsen and Reichart, the first two groups of lesions may be treated successfully by enucleation or curettage; it has been suggested that recurrence following conservative surgery is more likely to occur in the third group and that these lesions should therefore be treated by radical resection, as for a solid or multicystic ameloblastoma .However, there is difficulty in determining the most appropriate form of treatment for these lesions, with the treatment of unicystic ameloblastoma being still very controversial , 9, 10.This happens due to the misapplication of the term “mural” . AccordiThe term “mural” describes the extent to which amelobastomatous changes penetrate the connective tissue layer of a cyst. Just as a mural painting covers only the surface of a wall, a mural ameloblastoma does not penetrate the epithelial lining of a cyst , 11, 12.For Marx and Stern, ameloblastoma “in situ” developing in and limited to the epithelial lining of a cyst and also ameloblastoma “microinvasive” arising from the epithelial lining and proliferating into the connective tissue layer of the cyst should be treated with enucleation. Yet, ameloblastoma “invasive” arising from the epithelial lining and proliferation through the complete thickness of the connective tissue layer of a cyst should be treated with resection .In the present case, microscopically epithelial proliferation was observed into the lumen and into the connective tissue layer of the cyst. The final diagnosis established was of unicystic ameloblastoma with mural proliferation. Based on this diagnosis, the treatment recommended, for most authors, is the surgical resection. However, according to Marx and Stern, our unicystic ameloblastoma is “microinvasively” arising from the epithelial lining and proliferating into the connective tissue layer of the cyst, where the enucleation is considered one viable treatment.Besides, the age of the patient and clinical and radiologic features were considered; thus the enucleation was the treatment of choice. The patient was followed up and one year later there was no sign of recurrence.In literature, the recurrence after conservative treatment of unicystic ameloblastoma is reported to be between 10 and 25% but these reports do not specify the histologic subtypes of the primary lesion. Due to this, many professionals choose resection as treatment, which most often is unnecessary , 13, 14.Therefore, in addition to analyzing the clinical and radiological data of the lesion, histopathological examination is of great importance for an accurate diagnosis and consequently to choose the most appropriate treatment.


Spinal cord glioblastoma (SG) accounts for 1.5% of all spinal tumors and has a poor prognosis with survival ranging from 2 to 26 months from presentation.A 57-year-old male presented with one week of paraparesis and contrasted magnetic resonance imaging (MRI) findings of an epidural enhancing thoracic mass suspicious for an epidural abscess. Intraoperative and pathologic findings revealed SG.Spinal cord tumors can mimic epidural abscess on MRI. When planning to address extradural spinal pathologies, one should be cognizant of the potential for either isolated or concurrent intradural pathologies. When the epidural findings do not correlate with preoperative imaging, intraoperative ultrasound imaging can identify intradural pathologies without violating the dura. Spinal cord glioblastoma (SG) is rare, accounting for 1.5% of all spinal tumors . The proThis case report presents a case of thoracic SG closely mimicking an epidural abscess on contrasted magnetic resonance imaging (MRI). The literature on SG treatments and outcomes will be reviewed and discussed. Use of intraoperative ultrasound imaging to identify intradural pathologies will be discussed.A 57-year-old male patient presented with a one-week history of progressive back pain, paraparesis, and decreased sensation below the umbilicus. His medical and surgical history was unremarkable, including for any previous infectious pathologies or intravenous drug abuse. The patient was afebrile and denied constitutional symptoms. His white blood cell count, erythrocyte sedimentation rate and C-reactive protein levels were not elevated. Physical examination revealed 5/5 bilateral upper extremity strength and 4/5 strength in the lower extremities bilaterally. He had decreased sensation at and below his T10 dermatome. Patellar and Achilles tendon reflexes were 3/4 bilaterally. There were three beats of clonus bilaterally. Muscular tone was normal. Rectal sphincter tone was intact. Enhanced T1 and T2 weighted MRI images of the thoracic spine revealed what appeared to be an enhancing mass within the posterior epidural space at the level of T5 and T6 and extending to the level of the T11 vertebral body (Figures The patient underwent emergent laminectomies from T4 to T7 but no epidural abscess was appreciated. Intraoperative ultrasound demonstrated an intradural mass. A durotomy along the length of the laminectomy was performed and a large tan intramedullary mass with extramedullary extension was encountered. Subtotal resection was pursued due to unclear gross tumor margins, significant vascularity challenging attempted hemostasis, and the presence of an incomplete preoperative deficit. Frozen section sent intraoperatively was reported as metastatic melanoma versus primary glial tumor. The dura was then primarily repaired and the wound was re-approximated in multiple layers.The specimen displayed epithelioid cells with fibrillar cytoplasm, moderate pleomorphism with occasional nuclear pseudoinclusions and a low mitotic rate Figure . There wThe patient reported improved sensation in the immediate post-operative period. Lower extremity strength improved to 4+/5 by the time of discharge. Both chemotherapy and radiation therapy were commenced upon discharge. Full neuraxis MRI with and without contrast was negative for concomitant spine or brain lesions.SG is extremely rare accounting for 1.5% of all spinal tumors and 7.5% of all intramedullary tumors . TypicalLiterature guiding management is limited to reviews, case series and reports due to the paucity of this pathology. Confirmed positive prognostic factors including age less than 60, period of therapy occurring after 1980 and adjuvant therapy have been corroborated by multiple series . Extent In the presented case, high field strength MRI with contrast did not predict an intramedullary mass. Subtotal resection was completed due to the lack of clear tumor margins, the high level of neurological function in the pre-operative physical exam and the presumed poor prognosis.Spinal cord tumors, specifically SG, can mimic epidural pathologies, including epidural abscess, on MRI with contrast. The possibility of a neoplasm should be considered in cases where MRI shows what appears to be an enhancing epidural mass.Operative planning for extradural spinal lesions should incorporate the remote possibility of intradural pathology. Ultrasound guided localization is valuable in this instance as it allows for intraoperative identification of an intradural lesion without violation of the dura.Management of SG remains a challenge due to limited research and guidelines for therapy. Surgical debulking and high dose radiation with chemotherapy affords the longest survival to date. Gene therapy holds some promise for the future but further research is necessary to develop optimal treatment strategies.


Brain MRI plays an essential role in both diagnosis and follow-up of the JC virus infection of the brain. Recently, MR studies with susceptibility-weighted imaging (SWI) sequences have shown hypointensities in U-fibers adjacent to white matter (WM) lesions of progressive multifocal leukoencephalopathy (PML). This finding has been confirmed with the use of quantitative susceptibility mapping (QSM), allowing to hypothesize a paramagnetic effect in these regions. Here, we report the first longitudinal assessment of QSM and R2* maps in natalizumab-associated PML to evaluate serial changes in susceptibility contrast images and their role in PML diagnosis and follow-up.We report the case of a 42-year-old woman with multiple sclerosis (MS) who eventually developed, after the 28th natalizumab infusion, subacute cognitive decline and received a laboratory-confirmed diagnosis of PML, leading to immediate drug discontinuation. Three months later, she suffered a new clinical exacerbation, with a brain scan revealing significant inflammatory activity compatible with the radiological diagnosis of an Immune Reconstitution Inflammatory Syndrome (IRIS). She was then treated with corticosteroids until the clinico-radiological spectrum became stable, with the final outcome of a severe functional impairment. Quantitative maps obtained in the early symptomatic stage clearly showed increased QSM and R2* values in the juxtacortical WM adjacent to PML lesions, which persisted during the subsequent disease course.High QSM and R2* values in U-fibers adjacent to WM lesions were early and seemingly time-independent radiological findings in the presented PML case. This, coupled to the known absence of significant paramagnetic effect of new active MS lesions, could support the use of quantitative MRI as an additional tool in the diagnosis and follow-up of natalizumab-related PML in MS. Progressive multifocal leukoencephalopathy (PML) is a rare and severe demyelinating disease of the central nervous system caused by the reactivation of JC virus (JCV) in immunosuppressed patients .In recent years, PML has gained increasing attention due to its association with natalizumab treatment in multiple sclerosis (MS) –4 and itNeuroimaging plays a pivotal role in both diagnosis and follow-up of JCV infection and brain MRI has been included in PML diagnostic workflow, due to its high-sensitivity in the screening of suspected patients, even in a presymptomatic stage , 7.Conventional MRI findings of PML are relatively well-known –11. ReceIn contrast to SWI, quantitative susceptibility mapping (QSM) and R2* mapping offer the possibility of a quantitative, and therefore more consistent, evaluation of brain susceptibility changes. Here, we describe the first longitudinal use of QSM and relaxometry maps in a patient with MS who developed a natalizumab-related PML.We report the case of a 42-year-old woman with a 10-year history of relapsing remitting-MS, treated with natalizumab in the last 2 years. She was JCV antibody-positive and had previously received another disease modifying therapy (interferon beta-1a/Rebif 44).After the 28th infusion of natalizumab, she eventually developed subacute cognitive decline, confirmed by neuropsychological tests. Approximately 2 weeks after this clinical onset, a brain MRI scan revealed the presence of multiple new lesions, highly suggestive of PML. Natalizumab was immediately discontinued, she was hospitalized and JCV DNA copies were found in cerebrospinal fluid , leading to a definite diagnosis of PML .Three months later, prompted by further cognitive deterioration and subacute right hemiplegia, another brain MRI scan revealed significant inflammatory activity, compatible with the clinico-radiological diagnosis of an Immune Reconstitution Inflammatory Syndrome (IRIS).per os), until the clinico-radiological spectrum became stable, and she was moved to a neuro-rehabilitation center.She subsequently received corticosteroids .1 = 7.63 ms; TE2 = 22.14 ms; voxel size = 0.65 mm × 0.65 mm × 1.3 mm; 128 axial slices) was acquired with a flip angle of 20° to provide QSM and R2* maps, as already described in detail -hyperintense lesion was present in the left globus pallidus, with no significant paramagnetic effect Figure B.Despite natalizumab discontinuation, a follow-up MRI scan, performed 35 days later, revealed progressive extension of the subcortical white matter (WM) lesions with persistently high QSM and R2* values in the adjacent U-fibers. Besides, the left pallidal lesion showed a mild increase of both susceptibility and R2*, reflecting a more marked paramagnetic effect Figure C.Following a clinical exacerbation, another brain MRI scan performed 91 days after the first diagnostic examination showed further increase in the multifocal extension, with changes in the contrast-enhancement pattern, suggestive of intense inflammatory activity due to IRIS. QSM anomalies of U-fibers adjacent to WM lesions were still present, even if more blurred, while susceptibility changes in the left globus pallidus became more evident  have been proposed as an additional diagnostic tool in this condition –20, and In particular, previous studies have reported the presence, on SWI sequences, of hypointensities in U-fibers adjacent to the WM lesions of PML , 14. ThiTo the best of our knowledge, no studies have been performed using a quantitative MRI method at different time points, covering the entire history of the disease to evaluate possible susceptibility changes that may help in the diagnosis or in monitoring the clinical evolution of these patients.In our case, increased QSM values in U-fibers adjacent to WM lesions, suggesting a paramagnetic effect, were a constant finding. This feature was independent from the clinical course of the disease, being already present in the early phase, when the appearance of new lesions could mimic MS recurrence rather than the occurrence of a PML, and persisting in chronic stages. This finding, coupled with the known absence of significant paramagnetic effect of new active MS lesion , 22, allThe meaning of these susceptibility changes has not yet been fully understood. Indeed, high amounts of iron have been reported within oligodendrocytes and myelin sheaths at the cortical–subcortical junction, in both healthy and MS patients’ brains . In MS pIn addition, we reported the presence of a lesion in the left globus pallidus, characterized by slowly progressive increase of QSM and R2* values. Previous studies have described susceptibility changes in basal ganglia of PML patients, characterized mainly by a diffuse, although asymmetrical, low signal on SWI typically attributed to iron deposition , 14. In Finally, some limitations should be considered in the present report. In particular, histopathological confirmation is lacking in our case, which would have allowed to directly verify our hypotheses. Furthermore, this is a single-subject evaluation, although studied at several time points, with all the associated limits. For this reason, future longitudinal studies including a group of PML patients are warranted, to further corroborate our findings.We support the hypothesis that susceptibility contrast imaging could represent a valid aid in the diagnosis of natalizumab-related PML in MS, even at an early stage, and therefore should be included in the MRI evaluation of patients with such clinical suspicion, or even in the routine imaging surveillance in this population.This study was carried out in accordance with the recommendations of “name of guidelines, name of committee” with written informed consent from the subject. The subject gave written informed consent in accordance with the Declaration of Helsinki. Since this was an observational case study, a formal review by the local Institutional Review Board was not required.GPontillo and SC: study concept and design; analysis and interpretation. RL, AR, and VM: acquisition of data. PB: analysis and interpretation. ET: critical revision; study supervision. GPalma: analysis and interpretation; critical revision; and study supervision.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.


Midgut volvulus in adults is a rare entity that may present with intermittent colicky abdominal pain mixed with completely asymptomatic episodes. This small bowel twist may result in complications of obstruction, ischemia, hemorrhage, or perforation. With a midgut volvulus, complications may be life-threatening, and emergent surgical intervention is the mainstay of treatment. This current case involves an 80-year-old woman with intermittent abdominal pain with increasing severity and decreasing interval of time to presentation. A CAT scan revealed mesenteric swirling with possible internal hernia. A diagnostic laparoscopy followed by laparotomy revealed a midgut volvulus, extensive adhesions involving the root of the mesentery, and a large jejunal diverticulum. The adhesions were lysed enabling untwisting of the bowel, allowing placement of the small bowel in the correct anatomic position and resection of the jejunal diverticulum. This is a rare case of midgut volvulus with intermittent abdominal pain, associated with jejunal diverticulum managed successfully. A midgut volvulus should be considered in the differential diagnosis of a patient who present with a small bowel obstruction secondary to an internal hernia, especially when a swirl sign is present on the CAT scan. In the adult patient population, small bowel obstruction is a relatively common diagnosis. However, obstruction due to small bowel volvulus is quite rare, and most cases have been documented in newborns. Current literature suggests the annual incidence of small bowel volvulus to be 1.7–5.7 per 100,000 adults in Western countries . Here, wThe patient is an 80-year-old female with a history of recurrent abdominal pain. Permission was obtained from the patient to allow discussion and publication of his case. The patient had a history of coronary artery disease, atrial fibrillation (on anticoagulation), multiple cerebral vascular accidents, chronic obstructive pulmonary disease, an aortic and mitral valve replacement, and a prior hysterectomy. The patient was noted to have presented to the hospital three times in the prior month with similar complaints of vague, diffuse abdominal pain that would last a few hours and resolve. The patient did note nausea and vomiting with these episodes. The patient re-presented to the emergency room, where a CAT scan was performed  which shThe operation was started laparoscopically but was soon converted to an open laparotomy due to a large mass of small bowel swirled onto itself that was adhered together . In mobiSmall bowel obstruction is a fairly common diagnosis in the adult population of the United States, with the most common causes being postoperative adhesions, masses, and hernias. Patients typically experience severe and intermittent abdominal pain, nausea, vomiting, and the inability to pass stool or flatus. A small bowel volvulus can occur if a portion of the bowel twists on itself and its mesentery, effectively causing an obstruction. If the obstruction is a closed-loop obstruction, which includes small bowel volvulus, the CAT scan can demonstrate a swirl sign. This finding represents mesenteric soft-tissue and fat attenuation with adjacent loops of bowel surrounding the rotated intestinal vessels . A relatThe differential diagnosis for a patient presenting with symptoms of a small bowel obstruction may include adhesions, hernia, neoplasm, intussusception, small bowel hematoma, or pathology relating to the patency of the lumen such as a bezoar or a gallstone . VolvuluSmall bowel volvulus may be classified as primary or secondary. Primary volvulus occurs in patients with a virgin abdomen who possess no anatomic abnormalities that would predispose them to develop a volvulus . These rThe primary determinant in reducing the morbidity and mortality of small bowel volvulus is early diagnosis and treatment . If the This is a rare case of midgut volvulus with intermittent abdominal pain, associated with jejunal diverticulum managed successfully. A midgut volvulus should be considered in the differential diagnosis of a patient who presents with a small bowel obstruction secondary to an internal hernia, especially when a swirl sign is present on the CAT scan to minimize morbidity and possible mortality of the patient.


Xanthogranulomatous pyelonephritis (XGP) is an uncommon infectious disease of the kidney known to mimic other renal maladies. A rare presentation of this uncommon disease is spontaneous renal hemorrhage (SRH).We report a case of XGP in a 58 year old woman who presented with abdominal pain, hematuria, and radiating left flank pain. CT scan was felt to be consistent with perirenal hemorrhage abutting a fat-containing renal mass. The patient was eventually taken to surgery for left partial nephrectomy. Pathology report returned as XGP, and the patient has no complications from this disease process at 8 month follow up.Our search of the literature shows XGP presenting as SRH to be a rare clinical entity. Furthermore, this is the first such case managed with a nephron-sparing approach. The “great imitator” XGP should be added to the differential for patients presenting with spontaneous renal hemorrhage. XGP is an uncommon infectious disease of the kidney first described in 1918 by Schlagenhaufer and characterized by chronic obstruction and inflammation . SRH is Herein, we reported a female presenting with abdominal pain and hematuria with radiographic findings consistent with SRH who was found to have XGP as the underlying cause of her illness. We also review the epidemiology, diagnosis, and management of these two conditions.A 58 year old female presented with hematuria and left flank pain that radiated to the abdomen. Computed tomography (CT) scan demonstrated a heterogeneous 8.3 X 6.5 X 4.9 cm fat-containing mass arising from the lower pole of the left kidney Fig. . CT alsoPhysical exam was remarkable for enlarged body habitus (BMI 34.7), limited ROM in right shoulder, arm, and neck. Laboratory abnormalities included an elevated glucose (186 mg/dl) and calcium (6.9 mg/dl). White cell count, BUN, and creatinine levels were within normal limits. Urine culture demonstrated no growth in 18–24 h and showed < 10,000 col./ml.The decision was made to treat the patient with open left partial nephrectomy. Intraoperatively, the mass was easily found along the inferior portion of the kidney. The mass had a large desmoplastic reaction surround it from a prior bleeding episode. Adhesions surrounding the vascular pedicle were released. The pedicle was then clamped and cold ischemia was induced after mannitol injection. Resection of the mass was performed by scoring the mass with Bovie electrocautery and carefully dissecting the mass from the normal parenchyma by blunt dissection with a Penfield nerve retractor. All bleeding vessels were ligated. There was 36 min of cold ischemia time and 200 ml of estimated blood loss. After appropriate closure of the surgical sites, the mass was sent to pathology and because of considerable need for pyelocaliceal closure a double J ureteral stent and foley were placed. Postoperatively, the patient experienced urinary retention after foley catheter removal. Once stable, she was discharged back to skilled nursing facility with a catheter in place that was later removed.At 10 day postoperative outpatient follow up, the patient was doing well and asymptomatic. BUN and creatinine levels were within normal limits but calcium remained low. Ureteral stent was easily removed during office cystoscopy. Eight months later the patient reported no signs of flank pain or hematuria and has remained at her pre-operative baseline.Escherichia coli or Proteus mirabilis. [XGP is well known for its capacity to mimic many other disorders, most notably renal neoplasms , 6. Prevrabilis. –9. XGP irabilis. . It is frabilis. . While trabilis. .SRH, also known as Wunderlich Syndrome, is another uncommon urologic condition. The etiology of SRH is most frequently due to renal neoplasm but has also been known to arise due to vascular disease or infection . While t3). Urinalysis (UA) was positive for leukocytes and pyuria. Urine culture was negative.Three of 4 XGP cases presenting with Wunderlich syndrome are summarised and can be reviewed in Table CT scan demonstrated bilateral central staghorn calculi with replacement of renal parenchyma with low attenuated collections in a hydronephrotic pattern. CT also showed a lower pole mass in the right kidney consistent with perinephric inflammatory changes. T1 and T2 weighted MRI images of the mass showed multiple enhancing septa.Two days following the initial presentation the patient was seen again for acute abdominal pain with a red cell count of 6 g/dl, blood pressure of 80/60, and was transferred to the intensive care unit for hemorrhagic shock. CT scan now showed an interval increase in the size of the renal mass and hemoperitoneum. After right nephrectomy pathological examination of the mass revealed abundant foamy macrophage aggregates, neutrophils, fibrosis, and inflammatory cell infiltrations, confirming the diagnosis of XGP .3. UA revealed hematuria only. Abdominal ultrasound and computed tomography displayed a large hypoechoic mass (12 X 7 cm) around the right kidney which extended into the pelvis and paravertebral space. Exploration of the mass revealed a large perirenal hematoma, abscess, and renal rupture. Culture of the abscess grew Proteus mirabilis. Histopathological examination following right nephrectomy revealed foamy, lipid laden macrophages, giant cells, polymorphonuclear cells, granulomatous reaction and fibrosis. A diagnosis of XGP was made [Altinoluk et al., describe a case of XGP with spontaneous kidney rupture in a young female . A 25 yewas made .3). Urine culture was positive for Klebsiella and the patient was subsequently started on IV antibiotics. MRI abdomen demonstrated a hydronephrotic left kidney with an ill-defined mass of the lower pole suggestive of perinephric hematoma. Further, the patient was suspected to have a hemorrhagic angiomyolipoma and was thus surgically explored. During exploration, the kidney was noted to be hydronephrotic with thinned out parenchyma and palpable thickening of the lower pole which warranted nephrectomy. Unlike the previous cases, lipid-laden macrophage aggregates were not seen though further histopathological examination of the mass revealed chronic granulomatous pyelonephritis with dilated vascular channels and no evidence of neoplasia [Lastly, Sharma et al., reported a case of a 60 year old male who presented with left sided flank pain, intermittent fever, and weakness for 15 days . A knowneoplasia .The traditional approach to XGP has been radical nephrectomy, though a nephron-sparing approach has been reported in the management of focal cases of XGP –21. ConsManagement of SRH is first pursued through conservative or minimally invasive approaches. As the underlying cause of SRH may be focally identified by renal arteriography, embolization is an important therapeutic treatment that may preclude the need for surgery. In cases of iatrogenic renal hemorrhage, the combined use of urokinase injections and external drainage has been reported as an effective strategy in patients with large hematomas who nonetheless had stable vital signs . This apAll four previous reports of XGP presenting with SRH were managed by radical nephrectomy. We report the first case of nephron-sparing surgery for XGP presenting with SRH. Our management decision was facilitated by a pre-operative differential heavily weighted towards AML as the etiology of SRH. Prior to this procedure our approach to cases involving a large XGP lesion would have been unlikely to include a nephron-sparing approach, though we are happy to report that such an approach is certainly technically feasible.This report describes a rare presentation of an uncommon infectious disease. Our review of the literature confirms the rarity of XGP presenting with SRH. The importance of a broad differential diagnosis for this condition cannot be overlooked. Nephron-sparing surgery should always be considered as a possibility even in these difficult cases.


Rectal atresia is a rare anorectal malformation, and its association with other anomalies is even more rare. This study presents a unique case of co-twin in which the surviving newborn male underwent surgery due to rectal atresia. Newborn screening tests identified congenital hypothyroidism. The surgical treatment consisted of three stages and thyroid hormones were replaced. Isolated rectal atresia is a rare condition with a reported incidence of 1% of all anorectal malformations.A broad search of the English literature was performed to screen for relevant papers that examined other anomalies associated with rectal atresia, including Down's syndrome, congenital cardiac and renal diseases, tracheoesophageal fistula, choanal atresia, skeletal anomalies, midgut malrotation, presacral masses, pouch colon anomalies, vaginal atresia and vesical, urethral, double urethral, vestibular and labial fistulas of the rectum.In this study, a unique case of rectal atresia that presented as congenital hypothyroidism with isolated rectal atresia is reported in a newborn. The patient was treated successfully by a three stage posterior sagittal approach. This case is presented to demonstrate an uncommon and important therapeutic approach.A 1-day-old male neonate was admitted due to failure to pass meconium and abdominal distention. At that time, prenatal ultrasonic investigations and the family history were reported as normal. During follow-up, the mother was revealed to have had a high-risk pregnancy. A prenatal ultrasonic investigation at 10 weeks of gestation revealed fetal death of one of twin fetuses. A decision was then made to closely monitor the pregnancy to preserve the remaining fetus. After that, pregnancy went well and no pathologic futures were reported in the surviving baby' ultrasounds. The surviving twin was delivered with a birth weight of 2300 g via elective caesarian section to a 23-year-old primigravida mother after a full-term pregnancy. The patient exhibited progressive abdominal distension, vomiting, and failure to pass meconium. A physical examination revealed a normal appearance of the perineum, genitalia and a normally located anal opening; funnel anus was not detected and no signs of fistula were present. After examination of the anal opening with a firm catheter, a blind-ending anal canal was diagnosed. An upside-down invertogram was performed, and a Hegar dilator was passed through the anal opening, which revealed an air column in the distal intestine .Plain X-rays, echocardiography, ultrasound examinations of the newborn did not reveal any associated cardiac, spinal, urinary, or skeletal anomalies; the sacrum and sacral ratio were normal and there was no presacral mass on spinal ultrasound. A diverting descending colostomy was performed on the second day of life . BiopsyA definitive operation was performed at the age of 9 months via the posterior sagittal approach. Before the operation, a distal colostogram revealed rectal atresia without any fistula, and the atretic gap length of the rectum was determined to be ∼1.5 cm . The coVideo 1A muscle stimulator was used during the operation, and good muscle contractions were observed equally on both sides. Online content including video sequences viewable at:www.thieme-connect.com/products/ejournals/html/10-1055-s-0037-1612610-EJPSR-17-0353-v1.mp4.Several classifications are used to define anorectal malformations. Rectal atresia is generally categorized as complex and unusual.Congenital hypothyroidism is the most common endocrine disorder, affects ∼1:2,000 to 1:4,000 newborns worldwide and exhibits a high prevalence of extrathyroidal malformations.Distal intestinal atresia is difficult to diagnose prenatally, and this condition is a very rare cause of intestinal obstruction.Preoperative workup includes plain and cross table or upside-down X-rays, echocardiography, and ultrasonography of the urologic system and spine. Presacral mass should not be overlooked. Hamrick et al reported a 29% prevalence of presacral masses in a rectal atresia/stenosis series.Rectal atresia cases require a preliminary diverting colostomy to minimize postoperative complication.The optimal surgical care for patients with rectal atresia begins with appropriate decision making during the critical newborn period. Many operative approaches are used to correct this unique malformation.Posterior sagittal approach is a useful technique for the surgical correction of rectal atresia according to the largest reported series of rectal atresia/stenosis patients.Postoperative assessment is done with standardized questionnaires by Rintala and Lindahl at the age of 4.5 years.Rectal atresia is very rare, mostly nonsyndromic and occasionally associated with other anomalies. The association of congenital hypothyroidism and rectal atresia is unknown and has not been previously reported. This case report presents the first instance of congenital hypothyroidism and rectal atresia in a male neonate, and the subsequent treatments of both conditions are described. End-to-end rectoanal anastomosis via a posterior sagittal approach is a safe and effective technique for the surgical correction of rectal atresia.


This suggested the presence of HSV in the conjunctival mass. Review of pathology and HSV immunohistochemical staining confirmed the presence of HSV as a coinfection. The likelihood that the mass arose from an abnormal host response to HSV and HPV due to HIES was considered, but coexisting infection with these two viruses and HIES has not been reported in the literature; therefore, such cases require further investigation.Hyperimmunoglobulin E syndrome (HIES) or Job's syndrome is a rare immunodeficiency disease with less than 200 cases reported worldwide, among which few cases are reported with lesions due to herpes simplex virus (HSV) or human papillomavirus (HPV). This case study presents a rare case of HIES with coinfection of HSV and HPV. A 12-year-old boy, previously diagnosed with HIES, presented with a large conjunctival mass lesion. The presence of HPV in the lesion was confirmed by biopsy and by using the line-probe assay method to detect the HPV genome. However, the mass lesion did not respond to anti-HPV therapy with topical interferon- Hyperimmunoglobulin E syndrome (HIES) or Job's syndrome was first described by Davis et al. in 1966 . Only 20γ plays a primary role in the immunopathogenesis of this syndrome, and distorted T-helper (Th1/Th2) cytokine profiles contribute to impaired cellular immunity and specific patterns of infection susceptibility. Chronic refractory viral infection, particularly herpes simplex virus (HSV), molluscum contagiosum, varicella-zoster virus, and human papilloma virus (HPV) due to qualitative T-cell defects, can occur [Most cases of HIES are sporadic, although the disease can be inherited as an autosomal dominant or autosomal recessive trait. The basic immunologic defects of HIES are not clearly defined; however, abnormal neutrophil chemotaxis due to decreased production or secretion of interferon an occur . Presentan occur , 6.In addition to the various unusual manifestations of HIES, HSV infection is observed as recurrent lesions with keratitis  and has A 12-year-old boy presented to the Cornea Clinic of our hospital with a conjunctival mass lesion on his left eye, which had developed over the course of a few weeks. During a slit lamp examination, the lesion was found to be lobulated with corkscrew vessels that infiltrated the temporal, nasal, inferior and superior bulbar, and forniceal conjunctiva Figures . He had His history revealed that he had suffered from eczematous skin lesions, recurrent skin bursts, and sinopulmonary infections since early childhood and recurrent generalized HSV infection. He had been diagnosed with HIES at the age of three based on elevated serum IgE levels and other clinical features. He did not have a positive family history of HIES or any other immunodeficiency.At the onset of the lesion discussed here, a differential diagnosis of a conjunctival lymphoid tumor, conjunctival papilloma, and ocular surface squamous neoplasia was given. Because the patient was prone to recurrent HSK, recurrent herpetic lesions were not considered in the differential diagnosis because the patient presented a papillomatous mass lesion of the conjunctiva. Thus, the pathogenesis was suspected to be HPV because the dendritic lesion and stromal infiltration had not be seen in the failed graft. For a specific diagnosis, an incisional biopsy was obtained from the conjunctival lesion, and sections were prepared and stained with hematoxylin and eosin using the periodic acid-Schiff technique to observe the sections under light microscopy . Microscopic examination revealed conjunctival tissue with dense, diffuse, acute, and chronic inflammatory cells that infiltrated the lamina propria and were rich in eosinophil. Eosinophilic conjunctivitis with pseudoepitheliomatous hyperplasia was the pathologic diagnosis. An examination of the specimens for the presence of HPV was requested, and genotyping was performed by a DNA analysis using a line-probe assay method that revealed genotype 52 (high-risk) HPV.α2b (IFN-α2b) , 1,000,000 IU/mL, four times a day and oral cimetidine , 200 mg, four times a day for HPV and was given a low dose of oral acyclovir (400 mg/day) to prevent recurrence of HSK. There was no clinical response to this management at the three-month follow-up. In the next stage of treatment, the patient should have undergone excision and debulking of the mass lesion, but extensive herpetic lesions had recurred on the skin of the patient's head and neck. Therefore, the patient received intravenous (IV) acyclovir for systemic herpetic disease (600 mg/three times a day), prescribed by a pediatrician. The size of the conjunctival mass lesions decreased rapidly after this treatment, and the lesion completely resolved within a few days  and mitomycin-C, with variable success rates [α2b and oral cimetidine, but there was no clinical response to anti-HPV management at the three-month follow-up, although the lesion resolved a few days after administration of 600 mg/three times a day of IV acyclovir. It was concluded that HSV was the main pathogenic agent responsible for lesion formation with a coinfection of HPV, which misled the management process.The patient manifested a papillomatous mass lesion, which was suspected to be an HPV infection. In childhood, papilloma represents 7–10% of conjunctival tumors and possibly plays a role in HPV . The manss rates , 13. Sevss rates . Accordiα injections, rapid improvement in the lesion occurred, while pretreatment with systemic acyclovir and oral methylprednisolone had no effect on the lesion [α; however, systemic IFN-α may be more useful than topical IFN-α.A similar case was reported in the literature of a tumorous HSV blepharoconjunctivitis in a patient with a DOCK8 deficiency. She developed progressive blepharoconjunctivitis of the left upper and lower eyelid. In this case, after the initial IFN-e lesion . This st4 cells was proposed as an immunological mechanism for this unique manifestation of HSV coinfection with HIES. The mass lesions improved promptly under antiviral therapy with acyclovir [Another case of an unusual pathogenicity of the HSV in HIES was reported in a seven-year-old girl who presented with large soft masses rising from her nostril and from behind her ear. The presence of HSV within these lesions was confirmed. A mild decrease in CDcyclovir , which aHPV-6 and HPV-11 are major HPV types that are responsible for conjunctival lesions . In the α treatment was found to be beneficial in the treatment of virally triggered tumors; thus, continuous IFN-α treatment is an option for patients who are ineligible for bone marrow transplantation [Here, an immunological mechanism is proposed for a unique manifestation of coinfection of HPV and HSV in HIES, which has not previously been presented in the literature. No treatment modality is curative for the basic defects of HIES, in terms of cytokine/chemokine derangement, but it is hoped that bone marrow transplants and monoclonal anti-IgE  would be successful future treatments . IFN-α tantation . The rolIdentification of coinfections presented as conjunctival mass lesions in HIES is rare and diagnostically challenging. Such infections should be distinguished from other possibilities, such as primary HPV infection and other tumoral lesions, to ensure appropriate treatment and to avoid unnecessary and ineffective interventions, such as debulking of the lesion. The presence of these viruses in this syndrome, especially in the form of mass lesions, should always be kept in mind.To the best of our knowledge, this is the first description of an unusual and coexisting HSV and HPV related conjunctival mass lesion in a patient with HIES that was resolved promptly after treatment with intravenous acyclovir.


Intranodal palisaded myofibroblastoma (IPM), also known as “intranodal hemorrhagic spindle cell tumor with amianthoid fibers,” is a rare benign mesenchymal tumor originating from smooth muscle cells and myofibroblasts, often with the presence of amianthoid fibers. Usually IPM affects inguinal lymph nodes, but three cases have been described in the submandibular and cervical lymph nodes. We report a new case of a 44-year-old women with submandibular mass. Cervical ultrasound showed a suspect right submandibular adenomegaly. The patient underwent an excision of the submandibular mass. Histological features of the tumor include an encapsulated fusocellular proliferation, with nuclear palisading, amianthoid fibers, hemosiderin pigment, and extravasated erythrocytes. In the light of these results, we made the diagnosis of IPM. No recurrence was found 5 years after surgery. Intranodal palisaded myofibroblastoma (IPM), also known as “intranodal hemorrhagic spindle cell tumor with amianthoid fibers,” is a benign mesenchymal tumor of the lymph node originating from smooth muscle cells and myofibroblasts often with the presence of amianthoid fibers , 2. EighIn this study, we report a new case of submandibular IPM.A 44-year-old-women presented with a 5-year history of a growing right submandibular swelling and 2-year history of anterior basicervical swelling without dysphagia, dysphonia, or dyspnea. On physical examination, the basicervical swelling consisted in a goiter that predominates in the right lobe. The submandibular mass was firm, well circumscribed, and painless measuring 3 cm in greatest diameter. The cervical echography showed a heterogeneous multinodular goiter and a suspect right submandibular adenomegaly. The thyroid function  was normal. The patient had a total thyroidectomy and an excision of the submandibular mass. Grossly, the submandibular lesion, 3 × 2 × 1 cm in size, was well circumscribed, firm, and focally calcified and had a pearly-white appearance with multifocal hemorrhagic areas on the cut surface . The masImmunohistochemical stains showed a strong immunoreactivity for vimentin and Smooth Muscle Actin (SMA) . The tumThe patient was doing well at 5-year follow-up, with no evidence of recurrence.IPM is uncommon. About 89 cases have been reported in the literature . It is aIntranodal palisaded myofibroblastoma (IPM) is a rare benign mesenchymal tumor. It is a problem of differential diagnosis mainly with benign intranodal schwannoma. The treatment is based on complete excision of the lesion. Its evolution is benign. The histogenesis of this entity remains controversial. Further studies of large tumor series using molecular techniques would elucidate the pathogenesis of this lesion.


Cardiac hemangiomas are rare benign vascular tumors that can occur in any cardiac layer: endocardium, myocardium, or epicardium. Histologically, cardiac hemangiomas may be classified as capillary, cavernous, or arteriovenous; venous hemangiomas are extremely rare.A 46-year-old man reported experiencing precordial discomfort. Computed tomography revealed a massive tumor adjacent to the right ventricle. The right coronary artery was found to be located at the center of the tumor. Cardiovascular angiography showed that the artery was completely encased by the tumor without any obstruction and that the right ventricular lumen was compressed by the tumor. Surgical debulking of the tumor was performed under cardiopulmonary bypass, and the frozen section led to a diagnosis of benign hemangioma. The tumor was debulked as much as possible until the right coronary artery appeared. For decompression of the heart, the pericardium was left open to the thoracic cavity and unsutured. Histopathologic examination revealed a diagnosis of epicardial venous hemangioma.Cardiac hemangioma should be included in the differential diagnosis of mediastinal tumor in reference to the location and flow of the coronary artery. Surgical resection, or at least tumor debulking, is required to confirm the diagnosis and prevent further complications and has a favorable clinical outcome. Cardiac hemangiomas are extremely rare, accounting for 1 to 2% of primary benign cardiac tumors, and may arise from any cardiac layer: endocardium, myocardium, or epicardium . Most caCardiac hemangiomas are histologically classified into three morphologic subtypes: capillary, cavernous, and arteriovenous. Herein, we report a case of venous cardiac hemangioma, an extremely rare subtype of the disease.A 46-year-old man reported experiencing precordial discomfort and was referred to our department. He was a nonsmoker and had no history of any significant illness or surgery. Physical examination and electrocardiography showed no abnormal findings. Chest radiography, however, showed an enlarged right ventricular shadow, leading to a slightly elevated cardiothoracic ratio of 56%. Transthoracic echocardiography showed a mass in the pericardium, 8.0 × 4.0 cm in diameter, that compressed the wall of the right atrium and ventricle Fig. ; left veThe differential diagnosis included sarcoma or primary lymphoma, though the epicardial location would have been atypical for both tumors. Subsequently, biopsy and surgical resection of the tumor were scheduled in order to establish a precise diagnosis and to improve the outflow from the right ventricle and, thus, his symptomatic condition.Median sternotomy was completed under general anesthesia. During surgery, the tumor was determined to be hemorrhagic because incisional biopsy alone caused active bleeding, and cardiopulmonary bypass was initiated. Frozen section led to a diagnosis of a benign vascular tumor, but the right coronary artery was totally encased by the tumor, which was judged to be not amenable to curative resection. Thus, the tumor was resected directly with an electrical scalpel and debulked to the proximity of the coronary artery. The resected tumor specimen appeared as a spindle with a maximum diameter of 6 cm, accounting for approximately 50% of the original tumor volume.Histopathologically, dilated venous vessels with smooth muscle layer were widely observed in the fat layer of the pericardium Fig. –c, leadiThe patient had an uneventful postoperative course and was discharged on postoperative day 7. Follow-up chest CT showed volume reduction of the tumor and improvement of the compression of the right ventricular wall, and no subsequent regrowth Fig. . PresentPrimary cardiac tumors are rare, with a reported incidence of 0.0017 to 0.27% at autopsy. Hemangiomas are thought to represent only 1 to 2% of benign cardiac tumors . HemangiCardiac hemangiomas are generally classified into three histologic subtypes: capillary , cavernous , and arteriovenous . Often, some combination of these subtypes is observed in a tumor. In our case, histopathologic examination confirmed a venous cardiac hemangioma demonstrating small venous vessels in the fat layer of the pericardium. This subtype is not included in the three conventional subtypes and is an extremely rare disease entity.Most cardiac hemangiomas are asymptomatic and found incidentally, though they may present with dyspnea, palpitation, arrhythmia, murmur, pericardial effusion, or thromboembolic events; symptoms usually depend on tumor size and location . In our In many cases, definitive diagnosis of cardiac hemangioma cannot be made preoperatively, as in our case. For cardiac hemangiomas, complete resection is usually the treatment of choice –13. AfteSclerotherapy is frequently performed as first-line therapy . HoweverThe impressive effect of propranolol in treating infantile hemangiomas has provoked a paradigm shift in their management over the past few years , 19. In Treatment for hemangiomas depends on their size, location, and severity. Given the fact that cardiac hemangiomas are benign, it can be expected that our patient will remain asymptomatic for a long time. Thus, therapeutic strategies and surgical procedures for benign cardiac tumors should be considered flexibly with consideration of the positional relationship of the tumor with the coronary artery, the extent of coronary involvement, the rate of tumor growth, and the age or request of the patient. Moreover, in our case, surgery was completed with the pericardium left open. For benign cardiac tumors, it seems that such an innovative technique should be applied to prevent symptoms of heart compression in case of tumor regrowth. Finally, regular screening with various imaging modalities is highly recommended in all cases, especially for those with incomplete tumor resection, such as our case.Cardiac hemangioma is a rare disease entity; however, it should be included in the differential diagnosis of mediastinal tumor in reference to the location and flow of the coronary artery. Surgical resection, or at least tumor debulking, is required to confirm the diagnosis and prevent further complications and has a favorable clinical outcome.