Un caso singular de granuloma de cèlulas plasmáticas y actinomicosis en encía / A singular case of plasma cell granuloma and actinomycosis in the gum

Antecedentes: El granuloma de cèlulas plasmáticas (GCP) es una patología poco común que se caracteriza por una lesión roja e hiperplasia que presenta una gran afinidad a cualquier tejido blando con mayor frecuencia a nivel pulmonar y de manera poco usual a nivel oral como los labios, lengua y encía como un caso muy singular, histológicamente se caracteriza por un gran número de cèlulas plasmáticas. Objetivo: Reportar un caso poco común de GCP en encía con el abordaje, complicaciones y èxito del tratamiento del mismo. Descripción del caso: paciente femenino de 60 años de edad, acude a consulta por una lesión roja nivel de la encía marginal y adherida del maxilar superior. Al examen intraoral se observa una hiperplasia localizada Con diagnostico presuntivo de granuloma de cèlulas plasmáticas, donde se le realizaron exámenes radiográficos, posteriormente una biopsia, confirmando dicho diagnóstico. Conclusión: GCP se caracteriza clínicamente por presentar una hipertrofia gingival en la encía adherida, una de los lugares menos frecuentes a nivel oral. Esta patología al presentar ciertas similitudes clínica e histológicamente es muy difícil realizar un correcto diagnóstico de dicha patología (AU)

Background: Plasma cell granuloma (PCG) is an uncommon pathology characterized by a red lesion and hyperplasia that has a high affinity to any soft tissue, more frequently at the lung level and in an unusual way at the oral level such as lips, tongue and gingiva as a very unique case, histologically characterized by a large number of plasma cells. Objective: To report a rare case of GCP in the gingiva with the approach, complications and success of its treatment. Description of the case: a 60-year-old female patient comes to the clinic for a red lesion at the level of the marginal and attached gingiva of the upper jaw. Intraoral examination revealed localized hyperplasia with a presumptive diagnosis of plasma cell granuloma, where radiographic examinations were performed, followed by a biopsy, confirming said diagnosis. Conclusion: GCP is clinically characterized by presenting gingival hypertrophy in the attached gingiva, one of the least frequent places at the oral level. This pathology, as it presents certain clinical and histological similarities, is very difficult to make a correct diagnosis of said pathology. (AU)

Inflammatory myofibroblastic tumor of the urinary bladder: a case report.

The inflammatory myofibroblastic tumor (IMT) is a rare lesion, particularly in the urinary bladder. Inflammatory myofibroblastic tumor mainly affects children and young adults. It is unknown neoplastic potential, characterized by spindle cell proliferation with characteristic fibroinflammatory and pseudo-sarcomatous appearance. We describe a 36-year-old Moroccan man, who presented with hematuria the last week. The cystoscopy found a large bladder mass with necrotic-looking floating lesions, located in the trigonal area and left lateral wall on the dome of the urinary bladder. The patient underwent transurethral resection of the bladder tumor (TUR-BT). The histopathology and immunohistochemistry showed an IMT. No evidence of regrowth or residual tumor in 9 months of follow-up cystoscopy. In conclusion, even though, urinary bladder IMT is a rare occurrence, it is associated with a good prognosis. Histopathology investigation and immunohistochemistry analysis are essential to confirm the diagnosis. Complete TUR-BT is the treatment of choice.

Enlarging Liver Mass: Inflammatory Pseudotumor in a Patient With Polymyalgia Rheumatica.

Inflammatory pseudotumors of the liver are rare, non-neoplastic liver tumors. Due to the nonspecific clinical presentation, imaging features, and histopathological findings, they can mimic malignant tumors requiring invasive diagnostics. We present a case of a 61-year-old female patient with a history of type 2 diabetes mellitus, hypothyroidism, hyperlipidemia, and polymyalgia rheumatica who had initially presented with abdominal pain for 3 weeks. Further workup showed normal liver chemistries and tumor markers: AFP and CA 19-9. Magnetic resonance imaging (MRI) of the abdomen showed a segment 6 lesion measuring 4.1 × 4.0 × 3.7 cm. A liver biopsy then confirmed the diagnosis of an inflammatory pseudotumor of the liver with negative IgG4. On follow-up imaging, a rapid growth of this liver lesion was noted. Laparoscopy was done but did not show any distinct liver lesion. Follow-up imaging confirmed a decrease in the size of the mass. Interestingly, the patient had been on a higher dose of steroids for her polymyalgia rheumatic leading up to the follow-up imaging. This is the first case of an inflammatory pseudotumor of the liver in a patient with polymyalgia rheumatica. With this case, we would like to increase the awareness for inflammatory pseudotumors of the liver as a differential diagnosis of liver lesions in patients with underlying autoimmune disorders.

Infantile Inflammatory Myofibroblastic Tumor of Spleen.

Background: Inflammatory myofibroblastic tumor (IMT) is a mesenchymal neoplasm with unknown etiology and recurrent potential. They are widely reported in children and young adults. Nearly 50% of inflammatory myofibroblastic tumor harbor rearrangement in anaplastic lymphoma kinase (ALK) gene with the majority expressing ALK protein. ALK-negative IMTs harbor alteration in ROS1 gene in a subset of cases. Few reports have shown association of IMT with Epstein-Barr virus (EBV). Case report: We report a case of IMT of the spleen in an 18-month-old infant presenting with abdominal distention and failure to thrive. Workup for ALK-1, ROS1, and EBV small-encoded RNA in-situ hybridization using immunohistochemistry was negative. Conclusions: IMT can arise in an infant spleen.

Inflammatory pseudotumor-like follicular dendritic cell sarcoma with first clinical manifestation of thrombocytopenia: A case report.

BACKGROUND: Inflammatory pseudotumor-like follicular dendritic cell sarcoma (IPT-like FDCS) is often associated with Epstein-Barr (EB) virus infection. The tumor is commonly found in the spleen and liver, and it has been reported in the literature that it can be associated with paraneoplastic pemphigus, myasthenia gravis, and other diseases. A case of IPT-like FDCS with clinical features of thrombocytopenia has not been reported. PATIENT CONCERNS: A 59-year-old male patient visited our hospital in September 2020 due to bleeding gums and epistaxis. DIAGNOSIS: Splenic lymphoma with marked thrombocytopenia was initially diagnosed. The patient underwent pathological examination after splenectomy. Microscopic examination showed spindle-shaped or oval cells arranged in loose bundles, a large number of lymphocytes and plasma cells infiltrating the interstitium, and fibrin-like changes in the blood vessel wall. Immunohistochemical detection of tumor cells was positive for CD21, CD35, and Epstein-Barr virus in situ hybridization, and the patient was diagnosed with IPT-like FDCS. INTERVENTIONS: The patient underwent a splenectomy. The patient received platelet-raising therapy postoperatively. OUTCOMES: No tumor recurrence or metastasis was found during the 17-month follow-up period, and the platelet count returned to normal. CONCLUSION: IPT-like FDCS is an uncommon tumor, and its first presentation with marked thrombocytopenia is even rarer. The tumor was clinically and radiographically nonspecific. Definitive diagnosis relies on histopathological and immunohistochemical staining. IPT-like FDCS is biologically indolent and has a favorable prognosis.

Inflammatory Pseudotumor in the Nasal Cavity and Sinuses: A Case Report and Associated Literature Review.

Inflammatory pseudotumor (IP) is a clinically aggressive but histologically benign condition of unknown cause. It rarely appears in the nasal cavity and sinuses. Here, we describe a 24-year-old female with the main symptom of right nasal obstruction. Examinations showed dilation in the right maxillary sinus and a pale neoplasm in the nasal cavity. The neoplasm was completely excised under endoscope. Postoperative pathology showed significant proliferation of plasma cells and lymphocytes, indicating the presence of IP. No recurrence was found during 20 months of follow-up. Only 28 cases (10 males and 18 females, average 41.04 years) have been reported on Pubmed. The main clinical manifestations were nasal obstruction, epistaxis, facial swelling and pain, eyeball protrusion, diplopia, and other ocular symptoms. Inflammatory pseudotumor always erodes the surrounding bone and requires active treatments. Surgery is the optimal option with a good prognosis.

Intratumor Abscess in a Posttraumatic Hepatic Inflammatory Pseudotumor Spreading Out of the Liver.

A 45-year-old woman with abdominal pain after minor trauma was referred to our hospital. Computed tomography (CT) showed a hypovascular tumor in the left liver lobe. A tumor biopsy revealed granuloma, although no findings indicated malignancy or infection. A follow-up imaging study showed spread of the hepatic tumor. Her abdominal pain worsened after a second minor trauma. CT revealed an intratumor abscess, and pus overflowed from the patient's umbilicus. The abscess was improved by antibiotics and drainage therapy. In this case, unusual imaging findings and an atypical disease course of a hepatic inflammatory pseudotumor were observed.

Inflammatory Myofibroblastic Tumor of the Urinary Bladder and Ureter in Children: Experience of a Tertiary Referral Center.

PURPOSE: To present the clinicopathological characteristics and outcome of children with bladder and ureteral inflammatory myofibroblastic tumors (IMTs) in our center. METHODS: We reviewed the medical records of patients with bladder and ureteral IMTs from 2010 to 2018. We recorded patients' demographic data, presentation, hemoglobin level, presence of hydronephrosis, tumor size, treatment, and outcomes. RESULTS: Eight patients with bladder IMTs and 3 with ureteral IMTs were treated at our center during this period. The mean age was 7.1 years. Four patients presented with anemia at diagnosis with the mean hemoglobin level 84.5 g/L. Among patients with bladder IMTs, 5 were male and 3 were female. The most common symptom was lower urinary symptoms in 6 patients, followed by hematuria in 4 patients. 2 patients had complications of hydronephrosis and hydroureter. Among patients with ureteral IMTs, 2 were male and one was female. The most common symptom was abdominal pain, and 3 patients presented with upper urinary tract dilation. All patients underwent surgery. A total of 81.8% were positive for anaplastic lymphoma kinase. Cytokeratin (CK) expression was present in all patients with bladder IMTs, while it was negative in 2 patients with ureteral IMTs. During mean follow-up of 43.4 months, all patients survived event-free. CONCLUSION: The presence of hydronephrosis and hydroureter is rare in patients with bladder IMTs. Anemia caused by hematuria should be raised the index of suspicion for IMTs. Children with bladder and ureteral IMTs had excellent prognosis. The expression pattern of CK varied between bladder and ureteral IMTs.

Inflammatory Demyelinating Pseudotumor With Liver Dysfunction: IgG4 Related Disease With Primary Biliary Cholangitis.

Immunoglobulin G4 related disease (IgG4-RD) is a recently recognized immune-mediated disease which is far from understanding. A case of inflammatory demyelinating pseudotumor had been confirmed as IgG4-RD according to pathology features and clinical context. Combined with liver dysfunction, IgG4 related sclerotic cholangitis was suspected. However, primary biliary cholangitis was finally diagnosed by immune marks and histopathological findings. This is the first report in which mass lesions in the brain parenchyma were caused by IgG4-RD while liver dysfunction was due to primary biliary cholangitis. The clinical features of IgG4-RD are miscellaneous, and the accumulation of case reports might enrich clinicians experience and broaden their horizons about this condition.

ALK-1-positive inflammatory myofibroblastic tumor of the thyroid complicated by Hashimoto's thyroiditis: report of a rare case and a literature review.

BACKGROUND: Inflammatory myofibroblastic tumors (IMTs) of the thyroid are extremely rare soft-tissue tumors. In the literature, IMTs are sometimes called plasma cell granulomas (PCGs) or inflammatory pseudotumors, which often causes ambiguity. To date, 17 cases of PCGs and five cases of thyroid IMTs have been reported. These cases reveal that IMTs of the thyroid are often negative for the anaplastic lymphoma kinase (ALK-1) gene. To provide further information on this rare lesion, we present a case of an ALK-1-positive thyroid IMT and a review of IMTs of the thyroid. CASE PRESENTATION: A 34-year-old Chinese woman presented with a painless neck mass that had persisted for over a month. Ultrasonography revealed a 4.28 × 2.53 cm2 hypoechoic mass, in the left lobe of the thyroid gland. Serum levels of thyroglobulin and anti-thyroglobulin antibodies were high. Subsequently, left lobectomy was performed. Macroscopically, the lesion was a gray-brown nodular mass with a partial envelope. Histologically, two different lesion types were observed. The first lesion showed classic spindle cell proliferation, with spindle cells arranged in fascicles, accompanied by mature inflammatory cells. The other lesion showed a large number of infiltrating lymphocytes, with lymphoid follicles in the remaining thyroid gland, which was atrophic. Immunohistochemical staining showed that the spindle cells were negative for CK19, CyclinD1, Gelectin-3, EMA, CD34, S100, Bcl-2, and STAT-6, but strongly positive for ALK-1, vimentin, and TTF1. CK was focally expressed, and the Ki-67 index was 5%. A diagnosis of IMT was proposed according to immunohistochemical findings and morphology. Hashimoto's thyroiditis was confirmed according to serum levels of thyroglobulin and anti-thyroglobulin antibodies and morphology. The patient did not receive adjuvant therapy. She remained alive without disease recurrence for 10 months after lobectomy. CONCLUSIONS: IMTs should be considered in the diagnosis when spindle cell proliferation accompanied by mature inflammatory cells is observed, spindle cells are mildly atypical, and myofibroblast differentiation is present in the thyroid. A uniform diagnostic term is crucial to avoid ambiguity. Clinicians and pathologists should be aware of the necessity for long-term follow-up, especially in ALK-positive cases. The therapeutic potential of ALK-1 positivity should be explored further.

Inflammatory pseudotumor associated with HSV infection of rectal vascular endothelium in a patient with HIV: a case report and literature review.

BACKGROUND: Herpes simplex virus (HSV) typically infects oral or anogenital squamous epithelium and causes blisters and ulcerations. Here we reported an unusual case of HSV induced exuberant rectal inflammatory pseudotumor with vascular endothelial involvement. CASE PRESENTATIONS: A 52-year old man with HIV presented with abdominal pain, rectal drainage and constipation. Proctoscopy and CT scans revealed an 8 × 5 × 4 cm circumferential, mid-lower rectal mass that was concerning for malignancy. PET-CT showed mild to moderate FDG uptake of the rectal mass. Repeated biopsies showed exuberant lymphoplasmacytic inflammation with rich eosinophils and necrosis in the submucosa and scattered single or multi-nucleated viral inclusions in vascular endothelial cells that were positive for HSV by immunostains. There was no evidence of malignancy on histology or by immunostains. The patient started valacyclovir for three weeks and symptoms resolved after the antiviral therapy. Follow-up CT and sigmoidoscopy with biopsy revealed no rectal mass or drainable collection. CONCLUSIONS: HSV may present as proctitis with exuberant inflammatory response and mass-like lesion, and damages vascular endothelial cells in patients with HIV. The HSV-associated mass-like lesion can be effectively treated by 3-week valacyclovir.

IgG4-related disease with pseudotumor formation in the larynx.

Mikulicz's disease, an immunoglobulin G4-related disease (IgG4-RD) occurs frequently in the head and neck region but rarely in the larynx. We report a case of IgG4-RD with pseudotumor formation in the larynx. A 50-year-old man presented at our facility for a complete physical examination and diagnostic testing of a left arytenoid tumor. On examination, a large supraglottic mass was noted with signs of dyspnea. Movement disorder of the vocal fold was absent. The patient underwent surgery with general anesthesia to improve respiratory symptoms and a resected specimen was submitted for diagnosis. The pathology findings revealed lymph follicle formation, interstitial follicular fibrosis, angiogenesis, and inflammatory cell infiltration with plasmacytosis. Immunohistologic staining at high magnification revealed one hundred or more IgG4-positive plasma cells and fifty percent IgG4/IgG. In addition, obliterative phlebitis was observed. Medical history was positive for retroperitoneal fibrosis with serum IgG4 levels below the diagnostic criteria of IgG4-RD, but histological characteristics of IgG4-RD were met. The diagnosis of IgG4-RD was established, oral treatment with prednisolone (0.6 mg/kg/day) was initiated, and the tumor reduced in size. IgG4-RD may cause irreversible functional impairment. Early diagnosis and treatment are important in reducing the risk of permanent impairment of vocal fold mobility.

Sjögren syndrome-related plasma cell disorder and multifocal nodular AL amyloidosis: clinical picture and pathological findings.

Background: Localized nodular deposits of AL amyloid are seen in different tissues/organs; however, the pathogenesis of this form of amyloidosis remains unclear. Recently, Sjögren syndrome combined with localized nodular AL amyloidosis has been noted. Here, we report Sjögren syndrome cases showing multifocal nodular AL amyloidosis and the followed benign course. Materials and methods: We investigated the clinical pictures and histopathological findings of three cases with both presence of Sjögren syndrome and localized nodular AL amyloidosis, paying a special attention to the distribution of amyloidoma. Results: All three cases were middle-aged females. In two of three cases localized deposits of AL amyloid preceded Sjögren syndrome. Amyloidoma was detected in scalp, eyelid, cheek, larynx, trachea, lung and breast, and around these amyloid-deposited lesions infiltration of plasma cells was seen. Pulmonary amyloidosis was consistently accompanied with parenchymal cystic lesions, but this amyloidosis did not produce any significant respiratory symptoms. Some of large pulmonary amyloidomas showed cavity formation and subsequent shrinkage. In two cases amyloid deposition was found on gastric mucosa. Two cases received small doses of oral prednisone, with no further appearance of amyloidoma. Conclusion: Sjögren syndrome-related plasma cell disorder may be responsible for the formation of this unique multifocal nodular AL amyloidosis.

Retro-odontoid Degenerative Pseudotumour Causing Spinal Cord Compression and Myelopathy: Current Evidence on the Role of Posterior C1-C2 Fixation in Treatment.

BACKGROUND: A retro-odontoid pseudotumour compressing the spinal cord and causing myelopathy is often associated with an inflammatory condition such as rheumatoid arthritis. A degenerative non-inflammatory retro-odontoid pseudotumour responsible for clinically relevant spinal cord compression is a rare condition described in small clinical series and is likely associated with craniovertebral junction hypermobility or instability-like conditions. For several years, direct removal of the lesion through an anterior or lateral approach has been advocated as the best surgical option. However, in the last decade the posterior approach to the craniovertebral junction, to perform C1-C2 fixation and C1 laminectomy without removal of the retro-odontoid tissue, has demonstrated its efficacy in reducing retro-odontoid pannus as well as in obtaining improvement of myelopathy. METHODS: In this paper we analyse the clinical and radiological outcomes of seven patients (five males and two females) treated with posterior C1-C2 fixation and C1 laminectomy for a degenerative non-inflammatory retro-odontoid pseudotumour responsible for spinal cord compression. C1 laminectomy provided immediate spinal cord decompression. We also review the relevant literature focusing on associated cervical degenerative conditions that may contribute to triggering or acceleration of atlantoaxial hypermobility or 'instability', causing formation of the retro-odontoid tissue. RESULTS: The mean follow-up period (of six followed-up patients) was 55.8 months (range 10-96 months). In all cases the Nurick score at the latest follow-up visit demonstrated clinical improvement; magnetic resonance imaging during follow-up demonstrated progressive reduction of the retro-odontoid pseudotumour in all but one patient, who died of surgery-unrelated disease in the early postoperative period. No vascular or neural damage secondary to C1-C2 fixation was observed. CONCLUSION: C1-C2 fixation associated with C1 laminectomy is an effective surgical option to treat myelopathy secondary to a degenerative retro-odontoid pseudotumour. In these cases, direct removal of intracanalar tissue compressing the spinal cord is not required, as C1-C2 fixation is sufficient to cause its disappearance.

The inflammatory pseudotumor presenting periodic acid-Schiff-positive inclusions with acute unilateral facial nerve palsy.

Although most acute peripheral facial palsies can be attributed to Bell's palsy, other factors, such as infection, trauma, and neoplasm, can cause facial palsy as well. Among these, facial nerve tumors are rare but should be considered in the differential diagnosis of facial palsy. Palsies due to facial nerve tumors usually present with slow onset but occasionally present as acute episodes. In such cases, facial nerve decompression is the treatment of choice to allow the tumor room to grow without compressing the nerve or its blood supply. We describe a case of severe, acute facial palsy presenting with a spindle-shaped bone erosion on the mastoid portion of the facial canal. Although facial neuroma was suspected preoperatively, emergency decompression surgery revealed that an unusual inflammatory pseudotumor was responsible for the finding. Postoperative histological analysis revealed extensive destruction of the nerve fibers, with extensive infiltration of foamy macrophages containing characteristic, diastase-resistant, periodic acid-Schiff (PAS)-positive inclusions, which are hallmark of the uncommon bacterial infections. This was a case of facial palsy with an unusual etiology. The case shows the benefit of decompression surgery not only as treatment for the palsy but also as exploratory surgery in cases of facial nerve tumor.