Rare Site Of Involvement Research Articles

Case series of calcaneal osteomyelitis in patients with melioidosis

Background: Melioidosis is an emerging infectious disease caused by the Gram-negative bacterium Burkholderia pseudomallei, which can lead to a range of clinical manifestations including acute septicaemia and chronic localized infections. Osteomyelitis is an uncommon but well-recognized complication of melioidosis, with the calcaneus being a relatively rare site of involvement. Case Description: The authors present a case series of three patients with calcaneal osteomyelitis caused by B. pseudomallei infection. All three patients presented with non-specific symptoms like fever and weight loss, followed by localized ankle/foot pain and swelling. Diagnosis was made based on imaging findings (radiographs and MRI) and isolation of Burkholderia pseudomallei from blood or pus cultures. Treatment involved a combination of initial intravenous antibiotic therapy (meropenem or ceftazidime) for 4-6 weeks, followed by oral trimethoprim-sulfamethoxazole for 6 months. One patient required surgical drainage of a plantar abscess. All patients had a favourable clinical outcome with resolution of osteomyelitis on follow-up imaging. Conclusion: Calcaneal osteomyelitis is a rare but potentially serious complication of melioidosis. Early recognition and prompt initiation of appropriate antibiotic therapy are crucial for favourable outcomes. Clinicians should maintain a high index of suspicion for osteomyelitis in patients with melioidosis, especially in patients from endemic areas as well as in patients with risk factors like diabetes. Close follow-up and monitoring for potential relapse or reactivation are essential, particularly in immunocompromised individuals.

Long-term results of oncological endoprosthetics of the ankle joint: multicenter study

Introduction. The distal tibia and fibula are rare sites of involvement by primary and metastatic tumors. For a long time, amputation remained the standard surgical intervention for this location. Oncological ankle replacement is associated with a number of difficulties, including a lack of soft tissue necessary to cover the implant, close proximity of anatomical structures, a high probability of vascular and infectious complications, and difficulties in restoring the biomechanics of the lost joint. Currently, due to the small number of analyzed groups and short-term observations, there is no generalized data on primary and revision oncological ankle replacement, and there is no developed approach to treatment and prevention of complications.Aim. To generalize the analysis of long-term results, the structure of complications, and functional results in a group of patients after primary and revision oncological ankle replacement in a statistically significant group of patients.Materials and methods. The study included 40 patients with benign bone tumors, primary localized sarcomas of bone and soft tissue, and metastatic lesions, which, since 2008, until 2023 56 primary and revision endoprosthetics of postresection defect of the distal tibia were performed. Over a period of 15 years, the study group included 40 (71.4 %) surgeries in the scope of primary endoprosthetics and 16 (28.6 %) surgeries in the scope of revision oncological endoprosthetics of the ankle joint.Results. Over a 15-year follow-up period, the overall incidence of complications, structured according to the International Society of limb Salvage (ISOLS) 2013 classification, after primary and revision arthroplasty was 50 %. The average time until detection of oncological and non-oncological complications was 16.1 months. The leading complication after endoprosthetics was aseptic instability (Type II; 21.4 %) and tumor recurrence (Type V) – 20 %. The average functional outcome after primary and revision ankle replacement was assessed using the MSTS scale and was 72 % after 6 months and ranged from 43 to 97 %. After 12 months, this figure was 78 % and ranged from 49 to 97 %.Conclusion. An objective assessment and increase in the statistical reliability of the results of oncological ankle replacement requires a larger number of surgeries, a longer observation period and joint consolidation of data from various clinics. Development of indications for such reconstructive operations, careful selection of patients taking into account the effect of conservative treatment allows to reduce the total number of complications, the number of local relapses and achieve a good functional result.

Inguinal lymph node metastasis as the only manifestation of recurrent ovarian cancer: a case report

Background: Ovarian cancer has emerged as one of the most common malignancies affecting women in India. Despite optimal surgery and appropriate first-line chemotherapy, almost 70%–80% of patients with epithelial ovarian cancer will develop recurrence. Inguinal lymph node metastasis is a rare site of involvement in recurrent epithelial ovarian cancer. Case Presentation: Herein, we report the case of a 54-year-old female who presented to us with painless left inguinal lymphadenopathy. She gave history of being diagnosed with malignant ovarian cancer 2 years ago, for which she underwent debulking surgery. She received only one cycle of chemotherapy after which she defaulted. Cytological examination of the inguinal nodes revealed metastatic ovarian cancer. CA-125 levels were elevated despite the lack of any foci of metabolically active tissue on imaging. Conclusion: Inguinal lymphadenopathy is a rare presentation of recurrent ovarian cancer. Despite that, it is essential to examine the inguinal region in the follow up of all the cases of ovarian cancer. It is also necessary to consider ovarian cancer in the differential diagnosis for inguinal lymphadenopathy in a female patient.

Clinicoepidemiological profile of extranodal lymphoma: The experience of a tertiary care center in India

Introduction: Lymphoma is a neoplastic proliferation of lymphoid cells at various stages of differentiation and affects lymph nodes, with infiltration into the bone marrow, spleen, and thymus, which form the primary lymphatic organs. Extranodal lymphoma, by definition, involves sites other than lymph nodes, spleen, thymus, and the pharyngeal lymphatic ring. Extranodal involvement is less common with Hodgkin lymphoma (HL) than with non-Hodgkin lymphoma (NHL). Materials and Methods: A single-center retrospective observational study was conducted over the period of 2017–2019. Age, gender, histologic type, location, type of clinical presentation, histologic diagnosis, and presence of specific symptoms were recorded, as were the specialty of the physician initially consulted and of the physician taking the diagnostic sample. Results: Twenty-seven cases of extranodal lymphoma were diagnosed: 12 (44.4%) were male and 15 (56.6%) were female patients. The median age for males was 49.6 years and for females was 45.7 years. In this study, we had varied presentations of the lymphoma with involvement of various structures. We had few patients with very rare site of involvement like an elderly female patient presented with firm swelling over the right forearm which on excision turned out to myeloid sarcoma. Similarly, an elderly woman had presented with pyrexia of unknown origin that was later diagnosed as having primary bone Hodgkin lymphoma (HL) which is a very rare diagnosis and rarely has been described in world literature. The most common site of extranodal lymphoma was gastrointestinal tract (7 out of 27 patients; 25.9%); the other sites reported were testis (14.8%), breast (7.4%) thyroid, and ovary. Few rare sites reported were bone, central nervous system, and mediastinum. We report an extremely rare patient who had presented with anterior chest wall swelling, and on evaluation was detected to have primary mediastinal B cell lymphoma. Conclusion: Extranodal lymphoma is the rare presentation of NHL and extremely rare for HL. As it is a very rare disease, there are very limited studies available for its staging and management. We have presented a case series of extranodal lymphoma with few very rare presentations.

Penile lesion in a dialysis patient: Time to get an X-ray.

Key Clinical MessageCalciphylaxis or calcific uremic arteriolopathy is a rare and serious disorder typically seen in patients with end‐stage renal disease that manifests as skin ischemia and necrosis and histologically characterized by calcification of dermal arterioles. Penis is a rare site of involvement and biopsy is not typically recommended, making the diagnosis clinico‐radiologic.

Primary pulmonary NK/T-cell lymphoma: A case report and literature review.

Extranodal natural killer (NK)/T-cell lymphoma (ENKTL) is an aggressive disease with poor prognosis. The lung is a relatively rare site of involvement. The current study presents a case of primary pulmonary ENKTL with fever and dyspnea, mimicking pneumonia and initially treated with empirical antibiotics. The patient demonstrated rapid deterioration and died shortly following diagnosis. To the best of our knowledge, large-scale investigations referring to primary pulmonary ENKTL are not available. As a result, the exact incidence and clinical features of primary pulmonary ENKTL are unknown. In the current report, a literature review is presented to discuss the clinical characteristics, diagnosis, treatment, and prognosis factors of this malignant disease.

B-Lymphoblastic Lymphoma of the Rectum

Lymphoblastic lymphoma is a neoplasm of immature cells committed to the B-cell or T-cell lineage. B-lymphoblastic lymphoma usually involves lymph nodes and extranodal sites, such as the skin, bone, and soft tissue. The rectum is a very rare site of involvement in B-lymphoblastic lymphoma. We describe a 26-year-old man who presented with bleeding per rectum and fecal incontinence. Endoscopy showed a large nodular friable lesion narrowing the entire rectal lumen. Biopsy was diagnostic of B-lymphoblastic lymphoma. He was started on chemotherapy with the Berlin-Frankfurt-Münster protocol and achieved complete remission after induction chemotherapy. This is the first report of B-lymphoblastic lymphoma presenting as a rectal lesion in the world literature.

Pigmented villonodular synovitis of proximal tibiofibular joint: Rare site of involvement treated with medical management

Pigmented villonodular synovitis of proximal tibiofibular joint: Rare site of involvement treated with medical management

A rare case of metastatic germ cell tumor to stomach and duodenum masquerading as signet ring cell adenocarcinoma

Adenocarcinomas are the most common cancers affecting stomach. However gastrointestinal stromal tumors (GIST), lymphomas and neuroendocrine tumors (NETs) can also affect the stomach. But stomach is relatively rare site of involvement by metastasis. In this case report a rare metastasis of germ cell tumor (GCT) into stomach is described which clinically and endoscopically masquerade as primary gastric cancers. But detailed clinical examination and vigilant histopathological reporting proves the origin of tumor distant from stomach and thereby change the whole approach of management.

The genetic landscape of dural marginal zone lymphomas.

The dura is a rare site of involvement by marginal zone lymphoma (MZL) and the biology of dural MZL is not well understood. We performed genome-wide DNA copy number and targeted mutational analysis of 14 dural MZL to determine the genetic landscape of this entity. Monoallelic and biallelic inactivation of TNFAIP3 by mutation (n=5) or loss (n=1) was observed in 6/9 (67%) dural MZL exhibiting plasmacytic differentiation, including 3 IgG4+ cases. In contrast, activating NOTCH2 mutations were detected in 4/5 (80%) dural MZL displaying variable monocytoid morphology. Inactivating TBL1XR1 mutations were identified in all NOTCH2 mutated cases. Recurrent mutations in KLHL6 (n=2) and MLL2 (n=2) were also detected. Gains at 6p25.3 (n=2) and losses at 1p36.32 (n=3) were common chromosomal imbalances, with loss of heterozygosity (LOH) of these loci observed in a subset of cases. Translocations involving the IGH or MALT1 genes were not identified. Our results indicate genetic similarities between dural MZL and other MZL subtypes. However, recurrent and mutually exclusive genetic alterations of TNFAIP3 and NOTCH2 appear to be associated with distinct disease phenotypes in dural MZL.

Atypical clinical presentations of lymphomas: Two case reports

Chest wall and cheek swelling are rare sites of involvement by lymphoma. We hereby present two such cases with atypical presentations. Case 1: A 48-year-old male patient presented with a painful swelling over right cheek, which was clinically and radiologically suspected to be cellulitis and thus, incised and drained. Later patient presented to our hospital and fine-needle aspiration (FNA) was performed, which revealed cytological features consistent with non-Hodgkin's lymphoma. Case 2: A 30-year-old female patient presented with right anterior chest wall swelling, which was clinically diagnosed as cold abscess. However, FNA revealed cytological features consistent with Hodgkin's lymphoma. Both cases being reported were thought to be of infective etiology, which were however, diagnosed as lymphoma on FNA and thus led to a different management plan.

Spinal epidural cavernous hemangioma: a rare site of involvement

A 38-year-old woman with chronic back pain and hyperesthesia of both legs for the last 5 months was admitted to our hospital. Neurologic examination revealed power loss on both legs. Magnetic resonance imaging showed an ellipticalshaped epidural mass at thoracal 5–7 levels which lies posterior to the spinal cord, deplacing the cord anteriorly, and slightly extends out from the left neural foramina. The lesion was isointense to the spinal cord on T1-weighted images, hyperintense on T2-weighted images, and showed intense homogenous enhancement after contrast administration (Figs. 1

A Case of Focal Eosinophilic Myositis Associated with Hypereosinophilic Syndrome: A Rare Case Report.

Dear Editor: Hypereosinophilic syndrome (HES) is classically defined as (i) persistent eosinophilia of >1,500 eosinophils/mm3 for >6 months; (ii) the absence of any other evident cause of eosinophilia, including allergic diseases and parasitic infections; and (iii) signs or symptoms of organ involvement by eosinophilic infiltration. Skin involvement and cutaneous findings are frequently seen in these patients. Although many other organs other than the skin can also be affected by HES, myopathies associated with HES have rarely been reported1. Here, we report a rare case of focal eosinophilic myositis associated with HES. A 49-year-old woman visited our clinic with a solitary ovoid subcutaneous tender nodule on her right palm that appeared 2 weeks before her visit (Fig. 1). She denied any history of an insect bite or trauma at the site. Routine laboratory tests showed marked elevations in the eosinophil counts (6,730/mm3; reference range, 50~500/mm3), platelet counts (562×103/mm3; reference range, 150~350×103/mm3), and C-reactive protein levels (1.97 mg/dl; reference range, 0~0.6 mg/dl); the other test results were normal. Chest radiography showed mild bilateral pleural effusion. Skin biopsy was then performed, and the patient was referred to the department of allergy to check for the cause of blood eosinophilia. Thorough medical history taking, laboratory examinations, and imaging studies excluded any known causes of hypereosinophilia such as allergic diseases, allergic drug reactions, parasitic infections, human immunodeficiency virus infections, and solid tumors. The skin biopsy showed marked infiltration of eosinophils and lymphocytes in the muscle layer, as well as in the dermis and subcutis (Fig. 2A). The patient later developed a localized erythematous patch on her left calf. The skin biopsy at that site also showed moderate infiltration of eosinophils and lymphocytes in the dermis and subcutis (Fig. 2B). The biopsy specimen was insufficient for the evaluation of the muscle layer. The diagnosis of HES was made. Considering the absence of typical histologic findings of a dermal infiltrate of eosinophils, histiocytes, and eosinophil debris between collagen bundles that form flame figures, a diagnosis of eosinophilic cellulitis was less likely. After treatment with 1 mg/kg methylprednisolone for 2 weeks, the blood eosinophil counts decreased to within the reference range and the skin lesion subsided. The lungs could be another organ involved in HES, taking into account the sudden disappearance of pleural effusion after the treatment. Fig. 1 A solitary well demarcated erythematous to skin colored ovoid subcutaneous nodule on the right palm. Fig. 2 (A) The marked infiltrates composed of numerous eosinophis with a slight admixture of lymphocytes in the perimysium (H&E, ×200). (B) The moderate infiltrates of eosinophils and lymphocytes in the subcutaneous fat layer (H&E, ×200). ... Recently, to overcome problems with the above-mentioned old definition, Simon et al.2 proposed a new definition for HES: (i) blood eosinophilia (>1,500 eosinophils/mm3) on at least two occasions, or evidence of prominent tissue eosinophilia associated with symptoms and marked blood eosinophilia; (ii) absence of secondary causes of eosinophilia, such as parasitic or viral infections, allergic diseases, drug-induced or chemical-induced eosinophilia, hypoadrenalism, and neoplasm. Our case is consistent with this new proposed definition of HES. To our knowledge, this is the first case of focal eosinophilic myositis associated with HES in the Korean dermatological literature3. Furthermore, the palm is a very rare site of involvement, as muscle involvement in focal eosinophilic myositis is usually restricted to the lower legs4.

English

We report a rare case of osteogenic sarcoma of zygomatic arch presenting in a 20 year old male patient and a review of world literature on incidence and imaging. Craniofacial osteosarcomas are rare primary malignant bone tumours. This paper presents a case of osteogenic sarcoma of left zygomatic arch, showing erosion of middle aspect of left zygomatic arch with sun-ray spicules noted perpendicular to the cortex with multiple conglomerated new bone formation and associated soft tissue swelling extending inferiorly into the left infratemporal fossa and later diagnosed as osteosarcoma with chondroblastic differentiation. In head and neck, the most common sites of origin are the mandible, maxilla, nasal septum, hard palate and skull vault. More than 50% of craniofascial osteosarcomas arise in the jaw. Mandible is more commonly affected than maxilla. Zygomatic arch is a very rare site of involvement. Osteosarcoma should be considered in the differential diagnosis of lytic lesions with associated soft tissue swelling of zygomatic arch. Only four cases of primary osteogenic sarcoma of zygomatic arch have been reported in the English- language

Lump on back: a rare case of parosteal lipoma of scapula.

Lipomas are benign tumors of mature adipose tissue which can occur in subcutaneous, intramuscular, intermuscular, parosteal, and intraosseous compartments. Parosteal lipoma is a rare type of lipoma, accounting for less than 0.1% of primary bone neoplasms and 0.3% of all lipomas. Parosteal lipomas commonly arise in the femur and extremities. Around 150 cases have been reported in English literature with scapula being a rare site of involvement. They are known to be associated with underlying bony changes like focal cortical hyperostosis, pressure erosion of the underlying bone, and bowing deformity or with underlying osteochondroma. We report a rare case of a parosteal lipoma arising in the scapula with a bony excrescence in a 38-year-old male.

Invasive adenocarcinoma arising in extramammary paget’s disease – a case report and review of the literature

Introduction Extramammary Paget’s disease (EMPD) is a rare entity accounting for only 6.5% of all cases of Paget’s disease. EMPD can be difficult to differentiate from some intraepidermal squamous and melanocytic lesions in H&E stained sections. Case report An 86-year-old male with no significant past medical history presented with a supra-pubic skin rash for the last 14 months which was diagnosed by a dermatologist as an eczema. The patient was on topical steroid, which was unhelpful. The patient underwent an incisional biopsy followed by wide local excision. Pathological findings Macroscopically there was an irregular pale tan patch (60 × 55 mm) with a central depressed region (8 × 7 mm and 5 mm deep). Microscopically there were intraepider-mal and dermal clusters of large cells with abundant pale granular cytoplasm, large vesicular nuclei, prominent nucleoli and scattered mitosis. The lesion extended into the reticular dermis and showed occasional gland formation. There was no lymphovascular or peri-neural invasion. The margins were clear. A diagnosis was made of invasive adnexal adenocarcinoma involving the dermis with extensive overlying extramammary Paget’s disease. Discussion Extramammary Paget’s disease usually affects sites with a high density of adnexal glands, such as the anogenital region. Rare sites of involvement include axilla, the glans penis, buttock, thigh, knee, umbilicus, abdomen, scalp and lower anterior chest. Immunohistochemistry and special stains are helpful in confirming glandular differentiation and excluding a squamous or melanocytic lesion.

A unique variant of intestinal tuberculosis.

Duodenum is a rare site of involvement in intestinal tuberculosis. We report a 19 years old boy who presented with nonbilious vomiting and weight loss. His workup showed stricture in ascending colon, multiple liver abscesses with pneumobilia on CT scan. Upper GI endoscopy revealed stricture in 2nd part of duodenum distally. He was operated and duodenojejunostomy with limited right hemicolectomy (for stricture in this part of gut) were done. Biopsy report was suggestive of tuberculosis. Antituberculous treatment was started. He responded well and gained 20 kg weight at 3 months follow-up.

Use of F-18 FDG PET/CT in Non-Hodgkin Lymphoma With Central Nervous System Involvement

Central nervous system (CNS) is a rare site of involvement by non-Hodgkin lymphoma (NHL). Therapeutic approach for primary and secondary CNS NHL is different and remains challenging. Therefore imaging data are essential at staging to discriminate these 2 clinical entities and during follow-up to assess therapy response. The adjunct role of positron emission tomography using F-18 fluorodeoxyglucose to morphologic imaging is still undefined. We report 2 didactic cases of primary and secondary CNS NHL assessed by F-18 fluorodeoxyglucose positron emission tomography. Metabolic imaging seems to be complementary to conventional imaging techniques for the extent-of-disease evaluation and therapy monitoring of CNS lesions.

Unusual clinical manifestations, rare sites of involvement, and the association of other disorders with hairy cell leukemia

Unusual clinical manifestations, rare sites of involvement, and associations with other disorders and malignancies occurring in patients with hairy cell leukemia (HCL) are uncommon events encountered in a relatively rare disease. The exact prevalence of these associations is difficult to determine accurately in HCL as they are often anecdotal case reports and not always detailed in all larger series of patients. This short review deals with the unusual clinical manifestations and rare sites of involvement of the disease and lists some of the disorders associated with HCL, based on what has been reported in the literature as well as from personal experience. No attempts are made here to establish the true prevalence of these phenomena and only selected references are included. Some details of the coexistence of HCL with other neoplasias, hematological disorders, and ‘paraneoplastic’ autoimmune disorders are provided, while opportunistic infections in HCL, particularly atypical mycobacterial disease, are briefly discussed. For the sake of brevity many of the details are provided in tabular form.

Acute calcific tendinitis of tibialis posterior tendon (TPT): A rare site of involvement

Calcific tendinitis is a benign inflammatory disorder characterised by deposition of calcium hydroxyapatite crystals in tendons and is commonly seen in tendons around shoulder joint but can also sometimes be encountered in other sites including the hip, wrist, elbow, hand, neck and foot. Calcific tendinitis can clinically mimic many other pathologic entities like infection, gout, myositis ossificans or avulsion fracture which frequently leads to misdiagnosis and inappropriate management. Imaging plays an important role in recognizing and evaluating calcific tendinitis and in differentiating this entity from other pathologic conditions. Calcific tendinitis is largely a self-limiting process which usually responds to conservative treatment although in refractory cases invasive options like steroid injection, needle aspiration or surgery can be considered. Early and accurate diagnosis can prevent needless tests and interventions which the patient may be subjected. We report a case of acute calcific tendinitis involving a rare site (tibialis posterior tendon) with its multimodality imaging appearance.