Retroperitoneal Spindle Cell Tumor: A Case Report

Spindle cell tumor is very rare. Herein, we report a case of retroperitoneal spindle cell tumor in a 52-year-old female. The patient first presented with a complaint of persistent pain in the right upper abdomen. In the follow-up, a CT scan was performed and showed a retroperitoneal soft tissue density mass measuring 11 cm in diameter. Then, a subsequent operation was performed, and we completely removed the tumor and partially invaded lesions. The tumor was histologically diagnosed as a spindle cell tumor. Therefore, it is imperative for us to enhance the understanding of this seldom found tumor. Surgery remains the best option for treatment.

A tüdő nekrotizáló sarcoid granulomatosisa

Összefoglaló. A nekrotizáló sarcoid granulomatosis a granulomatosus pulmonalis angitisek közé tartozó, ritka kórkép. Egyesek a sarcoidosis variánsának, mások primer pulmonalis vasculitisnek tartják. A kórkép klinikai és patológiai jellegzetességeit két eset bemutatásával ismertetjük. A 20 éves nőbeteg sürgősséggel került pulmonológiai osztályra száraz köhögés, jobb oldali, mély belégzéssel összefüggő mellkasi fájdalom és láz miatt, a 63 éves férfi beteget pedig pneumoniát követő kontroll-mellkasröntgenfelvételen látott elváltozás kivizsgálása során észlelték. Az autoimmun panel vizsgálata, a mikrobiológiai tesztek mindkét betegnél negatívnak bizonyultak, a légzésfunkciós vizsgálat és a bronchoszkópos vizsgálat nem talált eltérést. A mellkas-CT-felvételen lágyrész-denzitású nodulusok látszottak egyoldali dominanciával, a folyamatot nem kísérte a hilusi nyirokcsomók szimmetrikus megnagyobbodása. A nodulusok szövettani vizsgálata vált indokolttá, melyet videoasszisztált torakoszkópos tüdőreszekciós mintavétellel biztosítottak. Mikroszkóposan a tüdőparenchymában gócos nekrózisokat, a környezetükben el nem sajtosodó epitheloid sejtes granulomatosus gócokat, az átfutó artériákban pedig granulomatosus arteritist láttak; a klinikai adatok figyelembevételével a tüdő nekrotizáló sarcoid granulomatosisa diagnózisát állították fel. A tüdőbetegség mindkét betegnél egy év alatt spontán regrediált. Az irodalom adatait és az eseteket összegezve, a tüdő nekrotizáló sarcoid granulomatosisában mikrobiológiai vizsgálatokkal nem igazolható tüdőfertőzés, és az immunológiai kivizsgálás sem tár fel szisztémás autoimmun betegséget; a diagnózis a klinikai kép és a képalkotó vizsgálatok alapján indikált szövettani vizsgálattal állítható fel. A betegség szteroidkezelésre jól reagál, de előfordul spontán regresszió is, az utóbbira láttunk példát. Bár az entitás átmenetet képez a nekrotizáló vasculitisek és a sarcoidosis között, egyre több érv szól amellett, hogy a sarcoidosis spektrumába tartozik. Orv Hetil. 2021; 162(38): 1541–1547. Summary. Necrotizing sarcoid granulomatosis is a rare entity currently classified as a subtype of granulomatous pulmonary angiitis. It is considered to be either a variant of sarcoidosis or a primary pulmonary angiitis. Two cases are demonstrated to present its clinical and pathological features. A 20-year-old female patient was admitted to the department of pulmonology with dry cough, right-sided chest pain during hyperventilation and fever. A 63-year-old male patient was observed with a right-sided lesion on chest X-ray after pneumonia. In both cases, autoimmune panel examination, microbiology tests, spirometry function test and bronchoscopy were unremarkable. Chest CT scans have revealed nodules with soft-tissue density without bilateral hilar lymphadenopathy. In order to clarify the diagnosis, video-assisted thoracoscopic resection (biopsy) was performed. Microscopically, parenchymal focal necrosis with adjacent to non-caseating granulomas and granulomatous angiitis were detected. In both cases, spontaneous remission occurred within a year. Histological examination – integrated with clinical data and radiological tests’ results – is the gold standard form of evaluation to confirm necrotizing sarcoid granulomatosis; furthermore, exclusion of pneumonia and autoimmune diseases are also required. The disease responds well to corticosteroids; moreover, spontaneous remission is often reported, as it happened in both cases. Necrotizing sarcoid granulomatosis is a transition between necrotizing vasculitides and sarcoidosis; although more and more evidence appears supporting the fact that necrotizing sarcoid granulomatosis may belong to the spectrum of sarcoidosis. Orv Hetil. 2021; 162(38): 1541–1547.

Primary lung sarcoma with gastric metastasis and morphological divergence presenting as melena

A 61-year-old man was admitted to the medical intensive care unit following a 2-week history of weakness, lightheadedness and melena resulting in an acute anaemia. Upper endoscopy revealed multiple large gastric masses without evidence of active bleeding. CT of the chest revealed a large right upper lobe mass with bony destruction of the third rib and invasion into the anterior chest wall and mediastinum, as well as a soft-tissue density in the left kidney. Biopsy and histopathological review of both pulmonary and gastric masses revealed two distinct sarcomatous malignancies that, while both from a primary lung source, differed in their morphology. Natural history and behaviour are not well understood in sarcomas due to their rarity, but abdominal metastasis is considered an uncommon event in the progression of the disease. Gastrointestinal bleeding as the presenting symptom of a primary lung sarcoma is an atypical finding with no previously reported cases.

Invasive Klebsiella syndrome with coexisting fungal endophthalmitis

Endogenous endophthalmitis accounts for approximately 5 – 10% of all endophthalmitis cases. We report a case of a middle-aged gentleman with underlying uncontrolled diabetes mellitus who presented with fever and generalised body weakness for one week. He was diagnosed with invasive Klebsiella syndrome based on blood culture with presence of bilateral pleural effusion, liver abscess, renal impairment and sphenoidal sinusitis. The patient developed sudden bilateral painless reduced vision on day two of admission. Ocular examination revealed bilateral severe anterior chamber reaction and severe vitritis that obscured the view of the fundus. Ocular B-scan ultrasonography showed multiple loculations in the posterior segment in both eyes. There was soft tissue density with calcification in the left sphenoid sinus on computed tomography of the orbit. He was treated for bilateral endogenous endophthalmitis with multiple intravitreal antibiotic injections, but showed no improvement. Functional endoscopic sinus surgery was performed and revealed that the left sphenoid sinus was filled with fungal balls. Following drainage of sphenoidal pus, there was resolution of vitritis and fundus examination showed features of underlying fungal infection with a “string of pearls” present along the vascular arcade of both eyes. The patient was diagnosed with bilateral endogenous endophthalmitis secondary to invasive Klebsiella syndrome with coexisting fungal endophthalmitis secondary to sphenoid mycetoma. In addition to repeated intravitreal antibiotic injections, he was also treated with systemic and topical antifungal therapy. At three months post treatment, the infection resolved and his vision improved from counting fingers to 6/36 bilaterally.

Imaging findings of trichilemmal cyst and proliferating trichilemmal tumour

Purpose The purpose of this study was to evaluate computed tomography and magnetic resonance imaging of benign trichilemmal cysts and proliferating trichilemmal tumours. Methods Nineteen histologically confirmed cutaneous lesions with trichilemmal keratinisation (12 trichilemmal cysts and seven proliferating trichilemmal tumours) were enrolled. Among them, 10 lesions (six trichilemmal cysts and four proliferating trichilemmal tumours) were examined by computed tomography, while 13 lesions (eight trichilemmal cysts and five proliferating trichilemmal tumours) were examined by magnetic resonance imaging. Computed tomography and magnetic resonance imaging characteristics were retrospectively reviewed. RESULTS Sixteen lesions (84%, 10 trichilemmal cysts and six proliferating trichilemmal tumours) occurred on the scalp. Lobulated margins were observed in five lesions (26%, three trichilemmal cysts and two proliferating trichilemmal tumours). With respect to computed tomography attenuation, calcification (>200 Hounsfield units) was observed in seven lesions (70%, five trichilemmal cysts and two proliferating trichilemmal tumours), hyperdense areas (≥80 and ≤200 Hounsfield units) in six (60%, three trichilemmal cysts and three proliferating trichilemmal tumours), and soft tissue density areas (<80 Hounsfield units) in nine (90%, five trichilemmal cysts and four proliferating trichilemmal tumours). On T1-weighted images, intratumoral hyperintensity was only observed in eight trichilemmal cysts but no proliferating trichilemmal tumours (100% vs. 0%, P<0.01). On T2-weighted images, hypointense rim and intratumoral hypointensity was observed in all 13 lesions (100%, eight trichilemmal cysts and five proliferating trichilemmal tumours), and linear or reticular hypointensity was observed in 10 (77%, six trichilemmal cysts and four proliferating trichilemmal tumours). Conclusion Trichilemmal cysts and proliferating trichilemmal tumours predominantly occurred on the scalp with calcification, and usually exhibited linear or reticular T2 hypointensity. Intratumoral T1 hyperintensity may be a useful imaging feature for differentiating trichilemmal cysts from proliferating trichilemmal tumours.

Imaging features of hemangioma in long tubular bones

Background To investigate the imaging features of hemangiomas in long tabular bones for better diagnosis. Methods Twenty-four patients with long bone hemangiomas confirmed by pathology were enrolled. Nineteen patients had plain radiography, fourteen patients had computed tomography (CT) and eleven had magnetic resonance imaging (MRI). The hemangioma was divided into medullary [13], periosteal [6] and intracortical type [5]. Results Among 19 patients with plain radiography, eleven patients were medullary, three periosteal, and five intracortical. In the medullary type, the lesion was primarily osteolytic, including five cases with irregular and unclear rims and one lesion having osteosclerotic and unclear rims. In three patients with the periosteal type, the lesion had clear rims with involvement of the cortical bone in the form of bone defect, including two cases with local thickened bone periosteum and one case having expansile periosteum. Five intracortical hemangiomas had intracortical osteolytic lesions with clear margins. Among 14 patients with CT imaging, 8 cases were medullary, three periosteal, and three intracortical. Among 8 medullary hemangiomas, one had ground glass opacity, and seven had osteolytic, expansile lesions like soft tissue density with no calcification. In three periosteal cases, the lesion was osteolytic with thickened periosteum and narrowed medullary cavity. In three intracortical hemangiomas, the lesion was of even soft tissue density with no calcification. Among 11 patients with MRI imaging, seven were medullary, two periosteal, and two intracortical. Among 7 medullary lesions, six were of hypointense signal on T1WI and hyperintensesignal on T2 WI. In two periosteal cases, the periosteum was thickened, with one case being of equal signal, and the other having no signal. Two intracortical hemangiomas were both of slightly low signal on T1WI but hyperintense signal on T2WI. Conclusions The long bone hemangiomas had characteristic cystic honeycomb-like presentations in plain radiograph. CT and MRI imagings are helpful for diagnosis of hemangiomas in long bone.

Cholesteatoma Masquerading as Cerumen

Cerumen is composed of glandular secretions and desquamated epithelial cells. It protects and lubricates the external auditory canal. Impacted cerumen causes canal occlusion and pressure over the tympanic membrane, causing ear discomfort, conductive hearing loss, itching, etc. Up to 6% of the general population are affected with impacted cerumen, which includes 10% of children, more than 30% of the elderly and people with cognitive impairment. Persistent symptoms despite resolution of impaction and patient repeatedly should raise suspicion of alternative diagnosis and prompt for further evaluation. A 20-year-old female patient came with complaints of decreased hearing and ear discharge. On otoscopic examination of left ear, impacted cerumen was seen in the attic region. On removal of wax with the aid of oto-endoscopy, attic region was found to be filled with cholesteatoma debris. On pure tone audiometry, patient had mild conductive hearing loss of 30 db and computed tomography of left temporal bone revealed ill-defined soft tissue density in the middle ear (epitympanum) with erosion of head of malleus and short process of incus. Blunting of scutum was also seen. Attic reconstruction and type III tympanoplasty was performed. On histopathological examination, the section showed keratin flakes with bacterial colonies which were consistent with cholesteatoma. In conclusion, any case of impacted cerumen should not be ignored by otorhinolaryngologist. Further evaluation with thorough examination under otomicroscope/otoendoscope has to be done which can lead to different diagnosis and treatment plan.

A rare and overlooked cause of massive gastrointestinal bleeding: Distal duodenal GIST

Introduction. Gastrointestinal stromal tumors (GIST) are tumors of mesenchymal origin which originate from the walls of gastrointestinal system (GIS) organs. Aim. In this case report we aim to discuss the clinical, labaratory and radiological presentation of distal duodenal GIST as a rare and overlooked cause of life-threatining GIS bleeding. Description of the case. A 76-year-old male patient was presented to the emergency department with massive gastrointestinal bleeding. Computerized tomography revealed a mass soft tissue density of 4x4cm at the level of the 3-4th segment of the duodenum. At the endoscopy, there was a deep ulcer in the proximal part of the 3rd segment of the duodenum with a diameter of 2 cm with a bleeding vessel protruding into the lumen. After endoscopic treatments, biopsies were taken from the edges of the ulcer. Histopathological examination revealed a sheet-like infiltration composed of mildly pleomorphic cells with oval-spindle nuclei and abundant eosinophilic cytoplasm in the duodenal lamina propria, as the patient was diagnosed of GIST. Conclusion. GIST and its clinical, labaratory and radiological presentation should be kept in mind in the approach to massive duodenal GIS bleeding.

Impossible Airway: A Case Report

Background: Wheezing is a common presentation of asthma and chronic obstructive pulmonary disease (COPD). However, other rare etiologies such as endotracheal tumor should be kept in mind. Case presentation: We report a case of mucoepidermoid carcinoma of carina in which the patient initially presented with wheezing mimicking COPD. Chest roentgenogram showed a soft-tissue density in the tracheal air column. Surgical resection of the tumor was technically hindered by difficulty in airway establishment during anesthesia. Extracorporeal membrane oxygenation (ECMO) was employed to secure adequate blood-gas exchange during operation, after which the tumor was resected smoothly and the patient discharged uneventfully.Conclusions: Endotracheal tumor should be kept in mind when facing a patient presenting with wheezing. Careful auscultation and interpretation of the chest roentgenogram are two important keys for differentiating such lesions from asthma or COPD. If further intervention was hindered by difficulty in airway establishment, ECMO proves effective and safe to “bypass” such an impossible airway and secure the procedures.

Soft tissue density within the foramen magnum, a predictor for surgical intervention in pediatric patients with Chiari malformation type I

OBJECTIVEChiari malformation type I (CMI) is diagnosed as herniation of the cerebellar tonsils by at least 5 mm below the foramen magnum. However, the degree of tonsillar herniation is a poor predictor of the need for decompression surgery. Exploration for an alternative morphological predictor for surgical intervention could provide greater insight into the development of an appropriate treatment plan for these patients. To investigate this issue, the authors calculated the soft tissue density within the foramen magnum as a measure of impaction of the cerebellar tonsils. Soft tissue density within the foramen magnum and degree of tonsillar herniation were then assessed for their correlation with the need for surgical intervention.METHODSThe authors conducted a retrospective, longitudinal chart review of pediatric patients with CMI. Those who had undergone surgical intervention were considered symptomatic and those who had been treated conservatively, as asymptomatic. Soft tissue density was found by dividing the soft tissue occupancy of the foramen magnum (brainstem and cerebellar tonsils) by the total area of the bony foramen magnum. The predictive value of these two measurements for the need of surgery was determined.RESULTSOf the 465 patients seen for CMI at the authors’ institution between July 1, 2011, and May 31, 2017, 80 underwent surgical intervention and 385 were asymptomatic. The average tissue density was significantly greater in the surgical group than in the asymptomatic group (83.3% and 78.6%, respectively, p < 0.0001). The average tonsillar descent for surgical patients was 10.8 mm compared to 9.8 mm for asymptomatic patients (p = 0.140). The point-biserial correlation coefficient was assessed, and soft tissue density was found to positively correlate with the need for surgical intervention (rpb = 0.199, p = 0.0001), whereas tonsillar herniation did not correlate with the need for surgery (rpb = 0.083, p = 0.115). Additionally, the degree of tonsillar herniation did not correlate with soft tissue density (r = 0.09), indicating that soft tissue density is an independent morphological parameter.CONCLUSIONSThe study findings suggest that the need for surgical intervention in CMI patients is positively correlated with increasing soft tissue density within the foramen magnum, whereas the degree of tonsillar herniation did not show a correlation with the need for surgical intervention. Additionally, soft tissue density is a factor independent of the degree of tonsillar herniation. Further investigation of tissue density within the foramen magnum is needed in the hope of discovering a clinically applicable parameter that would indicate a need for surgical intervention in patients with CMI.