Multiple Subcutaneous Masses Research Articles

A Case of Lymphoma with Multiple Identities

Discordant lymphoma (DL) is the coexistence of two or more distinct subtypes in separate anatomic sites. There are limited reports on DL cases especially involving more than two subtypes in more than two sites. We report a 76-year-old man who presented with constitutional symptoms, flank mass and painless lymphadenopathies for six months. Laboratory tests revealed moderate anaemia, markedly elevated serum IgM (13400 mg/dL), IgM Lambda paraproteinemia and Lambda light chain paraproteinuria with unmeasurable serum lactate dehydrogenase due to hyperviscous sample. CT scan showed multiple subcutaneous masses over chest wall and retroperitoneum, with lytic bone lesions, and hepatosplenomegaly. Further biopsy findings with morphological, immunohistochemical and molecular analysis of the tissue sections revealed diffuse large B-Cell lymphoma in the chest wall mass, follicular lymphoma in the inguinal lymph node and lymphoplasmacytic lymphoma in the bone marrow. This case highlights the rare DL. The importance of histopathological evaluation of lymphoma despite the availability of PET-CT scans for disease staging is undeniable.

Familial Multiple Lipomatosis: A Case Report

A 32-year-old man presents to the clinic with multiple subcutaneous masses. Further history and a review of the literature are discussed. This is a case study of a patient with familial multiple lipomatosis and our management. Liposuction was performed after informed consent was obtained to manage familial multiple lipomatosis in an aesthetically pleasing manner without recurrence. Liposuction is an excellent technique for an aesthetically pleasing outcome to manage familial multiple lipomatosis. Additional therapy modalities including laser, chemicals, high-pressure tumescent injection, and Vibration Amplification of Sound Energy at Resonance (VASER) are reviewed. To avoid multiple incisions and scars, liposuction for familial multiple lipomatosis is an aesthetically pleasing, definitive treatment modality with no recurrence of lipomas.

Clinical Images: Giant mass on the forehead in Kimura disease.

The patient, a 68-year-old Asian man with a history of nephrotic syndrome, presented to our department with multiple subcutaneous masses that developed over the past 50 years. The masses were painless, soft, and predominantly located in the head. The mass on the forehead was dome-shaped and had a diameter of 7 cm and a height of 3 cm (A). Blood tests showed eosinophilia and elevated immunoglobulin E (IgE) levels. Serum immunoglobulin G4 levels were normal. Ultrasonography showed heterogeneous masses with marked associated internal hyperemia (B). Magnetic resonance imaging showed T1-weighted isointense and T2-weighted hyperintense masses. A needle biopsy of the forehead mass showed stromal fibrosis, multiple lymphoid follicles, and infiltration of inflammatory cells, mainly eosinophils and lymphocytes. The patient was diagnosed with Kimura disease (KD). The masses shrank rapidly after administration of 30 mg/day of prednisolone for 2 weeks. KD is a rare chronic inflammatory disorder mainly affecting young Asian men. It presents as subcutaneous masses, typically in the head and neck, and is associated with eosinophilia and elevated IgE levels (1). It often affects the cervical lymph nodes and salivary glands (1). It is often associated with renal involvement, such as nephrotic syndrome (2). Glucocorticoids, surgical excision, and radiation therapy are the treatments of choice (3). The diagnosis of KD can be difficult because of its rarity. KD should be considered as a differential diagnosis when patients present with painless subcutaneous masses in the head and neck region with eosinophilia and elevated serum IgE levels.

Complete and durable response of pulmonary large‐cell neuroendocrine carcinoma to pembrolizumab

BackgroundPulmonary large cell neuroendocrine carcinoma (LCNEC) is a rare and aggressive tumor with a poor prognosis and standard therapy has not yet been established.CaseA 65‐year‐old male with a cough for 2 months presented to our hospital. He was clinically diagnosed with non small cell lung cancer cT3N1M0 stage IIIA and underwent right pneumonectomy. The final diagnosis was pulmonary LCNEC pT3N1M0 stage IIIA. Multiple subcutaneous masses were detected 4 months after surgery, and biopsy revealed postoperative recurrence and metastasis. Chemotherapy with carboplatin plus etoposide was initiated. Subcutaneous masses increased and multiple new brain metastases developed after two cycles. Additional tests revealed that epidermal growth factor receptor and anaplastic lymphoma kinase were negative, and the programmed death ligand 1 (PD‐L1) expression rate in tumor cells was 40% (22C3 clones). The primary cells infiltrating the tumor were CD3‐positive T cells and CD138‐positive plasma cells. Second‐line treatment with pembrolizumab was started. The shrinkage of subcutaneous masses was observed after one cycle, and the tumor had completely disappeared after six cycles. Treatment was continued for approximately 2 years. This response has been maintained for 4 years and is still ongoing.ConclusionPembrolizumab may be used as a treatment option for pulmonary LCNEC.

A Case Report of an Invasive Scalp Actinomycosis

Background: Actinomycosis is a bacterial infection characterized by a chronic, suppurative and granulomatous process. The causative organism was identified in 1891 as Actinomycosis israelii. The disease is usually caused by the introduction of the bacteria through minor wound. Actinomycosis was classified by Cope into 3 different forms: cervicofacial (50%), pulmonothoracic (30%) and abdominopelvic (20%). Actinomycosis is an insidious disease but occasionally the clinical course can be more aggressive, involving underline tissue and organs. Involvement of scalp by actinomycosis is rare causing diagnostic dilemma and could be mistaken for different pathologies, such as tuberculosis or carcinoma. The most common clinical findings are local tumefaction with abscesses and sinuses draining pus that contains the granule of the causative organism. CT scan and MRI are aspecific for diagnosis, but they can help in defining the localization and the extension of the lesion. The certain diagnostic is based on cytology and/or biopsy. Case: A 24 year-old-man was admitted to our neurosurgical department with a 16-month history of multiple scalp ulcerated lesions and swelling of the head. He was complaining of headache. Physical examination revealed multiple scalp subcutaneous nodules and ulcerated lesions with swelling of the head. Our examination of the oral cavity revealed a poor oral hygiene condition with multiple caries. The CT scan revealed multiple scalp subcutaneous masses with chronic inflammatory changes of the skull. Routine laboratory test was normal. Surgical biopsy was performed and the histological result was compatible with actinomycosis. After histological confirmation, parenteral Penicillin 20 million units daily were given for 4 weeks. Clinical improvement was observed after completing a 4-week regimen of intravenous antibiotics. Conclusion: Involvement of scalp by actinomycosis could be mistaken for different pathologies, such as tuberculosis or carcinoma. This emphasizes the importance of biopsy for histological confirmation of the disease.

FINAL DIAGNOSIS OF GARDNER SYNDROME DUE TO STOMATOLOGIC SUPPORT

Gardner syndrome (GS) is an autosomal dominant disease characterized by adenomatous intestinal polyps, and if not treated, shows a great chance of becoming malignant. Multiple osteomas in the skull, maxilla, and mandible, along with multiple cutaneous and subcutaneous masses are also characteristics of GS. A 30-year-old woman reported an epidermoid cyst on the frontal bone and a scar on the left shoulder due to osteoma removal. Tooth absence, malocclusion, and osteoma in the palatal region were observed in the intraoral exam. A panoramic radiography analysis revealed permanent teeth retention, supernumerary teeth, and radiopaque areas suggestive of osteomas. Although there were Gardner syndrome manifestations, only after our evaluation, a colonoscopy examination was ordered and intestinal polyps were removed with a diagnosis of tubular adenoma with low grade dysplasia. Today, the patient is under follow-up with a gastroenterologist and oral and maxillofacial surgeons, orthodontists, and stomatology experts with a common goal of function, esthetic, and quality of life improvements. Gardner syndrome (GS) is an autosomal dominant disease characterized by adenomatous intestinal polyps, and if not treated, shows a great chance of becoming malignant. Multiple osteomas in the skull, maxilla, and mandible, along with multiple cutaneous and subcutaneous masses are also characteristics of GS. A 30-year-old woman reported an epidermoid cyst on the frontal bone and a scar on the left shoulder due to osteoma removal. Tooth absence, malocclusion, and osteoma in the palatal region were observed in the intraoral exam. A panoramic radiography analysis revealed permanent teeth retention, supernumerary teeth, and radiopaque areas suggestive of osteomas. Although there were Gardner syndrome manifestations, only after our evaluation, a colonoscopy examination was ordered and intestinal polyps were removed with a diagnosis of tubular adenoma with low grade dysplasia. Today, the patient is under follow-up with a gastroenterologist and oral and maxillofacial surgeons, orthodontists, and stomatology experts with a common goal of function, esthetic, and quality of life improvements.

Treatment of consistent BRAF/HRAS gene mutation and MYC amplification radiation-induced abdominal wall angiosarcoma with low-dose apatinib: a case report

BackgroundAn extremely rare condition, radiation-induced angiosarcoma is characterized by a poor prognosis, high recurrence rate and lack of effective treatment. Herein, we present a case report of a 48-year-old female patient with radiation-induced abdominal wall angiosarcoma who showed a dramatic response to low-dose apatinib.Case presentationThe patient, who was diagnosed with cervical squamous cell carcinoma 20 years ago, had received radiotherapy and chemotherapy after operation. Angiosarcomas of the abdominal wall appeared 9 years later. After repeated surgical operations and intravenous chemotherapy for the angiosarcomas, the patient developed tumor recurrence and pulmonary metastasis. The abdominal wall tumors showed repeated rupture and bleeding, with poor wound healing. On evaluation, laboratory findings detected the negative serum tumor markers CEA, CA 125, CA 15–3 and CA 19–9. Imaging showed multiple subcutaneous nodules and masses in the abdominal wall, accompanied by suspected small subpleural nodule at the lower lobe of the right lung. Immunohistochemistry of previous surgical pathology indicated that CD31, ERG and Vim were positive. The result of whole exome sequencing suggested the mutations of BRAF and HRAS, and the amplification of MYC. Based on the above results, the patient was clinically diagnosed with radiation-induced angiosarcoma of the abdominal wall with pulmonary metastasis. The patient was treated with low-dose apatinib and rejected reoperation or chemotherapy.ResultsAt the 6-month follow-up visit, the abdominal wall lesions that had previously ruptured stopped bleeding and showed significant shrinkage. Imaging showed that most of the abdominal wall lesions had partially regressed, and some of the lesions on the abdominal wall and the suspected lesion of subpleural nodule at the lower lobe of the right lung had disappeared.ConclusionsWe described this case and reviewed the literature on radiation-related angiosarcoma. Importantly, this case suggests that apatinib may be an effective and sensitive treatment for radiation-induced angiosarcoma even at the lowest dosage, without aggravating the bleeding of lesions.

Medical management of phimosis associated with generalized Sticker’s sarcoma in an intact mongrel dog

A 3-year-old intact male mongrel dog was presented to the Referral Veterinary Polyclinic, ICAR-Indian Veterinary Research Institute, Izatnagar, with the history of inappetence, multiple subcutaneous swellings, and haematuria for the past 15 days. The dog had a history of mating with stray dogs, a few months ago. Physical examination revealed normal vital signs and generalized lymphadenopathy along with multiple subcutaneous hard masses of approximately 1–5 cm size was observed all over the body including the eyelids, inner canthus, and prepuce; and swelling on the shaft of the penis was also palpated. The haematology revealed microcytosis and mild thrombocytopenia. Fine-needle aspiration cytology (FNAC) of subcutaneous nodules and genital swelling was positive for Sticker’s sarcoma. Based on the anamnesis, clinical symptoms, and FNAC report, the case was diagnosed as a case of generalized cutaneous and genital form of Sticker’s sarcoma. The animal was instituted with vincristine sulphate intravenously at 0.025 mg/kg body weight once a week for 4 weeks along with ascorbic acid 20 mg/kg body weight, orally for 28 days as supportive treatment. At the end of 4 weeks of chemotherapy, the animal recovered uneventfully.

Generalized crystal-storing histiocytosis with diffuse large B-cell lymphoma and monoclonal gammopathy in a Chinese elderly woman: a case report

BackgroundCrystal-storing histiocytosis (CSH) is a rare lesion characterized by sheets of crystal-laden non-neoplastic histiocytes. CSH shows a prominent association with lymphoproliferative disorders that express monoclonal immunoglobulins, mainly multiple myeloma (MM), lymphoplasmacytic lymphoma (LPL) and monoclonal gammopathy of undetermined significance (MGUS). However, no aggressive B cell lymphoma has been reported to be associated with CSH.Case presentationA 74-year-old Chinese woman presented with multiple subcutaneous masses, abdominal pain, and fever. An IgM kappa type of monoclonal gammopathy (MG) was noted by immunofixation performed on the patient’s serum. Computed tomographic (CT) scan revealed subcutaneous masses on the left upper arm and at the waist and multiple low-density lesions in the spleen. Microscopically, sections of subcutaneous masses revealed sheets of large polygonal and spindle cells with abundant eosinophilic cytoplasm, round to ovoid eccentric nuclei, reticulate chromatin, and median nucleoli. Massive needle-shaped crystals were confined to the cytoplasm. Immunohistochemically, these crystal-containing cells were positive for CD68/PGM1, CD163, IgM, and Igκ. Meanwhile, the splenic tumour was diagnosed as diffuse large B-cell lymphoma (DLBCL), non-germinal-centre B (non-GCB) subtype (Hans algorithm). Immunohistochemistry for IgM was positive in the cytoplasm of some neoplastic cells. Immunoglobulin heavy chain (IgH) gene rearrangement was detected by PCR analysis of the subcutaneous mass and the splenic tumour.ConclusionTo the best of our knowledge, this is the first case of generalized CSH with DLBCL and MG. Although the rarity of CSH and separate locations of CSH and lymphoma led to a diagnostic dilemma, the presence of MG was a clue to appreciate the relation between CSH and DLBCL. This case stressed a full investigation into the underlying lymphoproliferative disorder for integrated diagnosis and correct treatments.

Granulomatous Mycosis Fungoides Presented as Multiple Subcutaneous Masses: a case of 5-year follow-up and Review of the Literature

Granulomatous Mycosis Fungoides Presented as Multiple Subcutaneous Masses: a case of 5-year follow-up and Review of the Literature

Anisometric cell lipoma: Insight from a case series and review of the literature on adipocytic neoplasms in survivors of retinoblastoma suggest a role for RB1 loss and possible relationship to fat-predominant ("fat-only") spindle cell lipoma.

The term "anisometric cell lipoma" (ACL) has been proposed recently by Evans for a lipoma variant characterized by striking variation in size and shape of adipocytes but little or no cytological atypia. One patient with multiple ACL had a history of retinoblastoma. The current study analyzed six patients with ACL (4 males and two females aged 34 to 87years; median, 58); all seen in consultation. Five patients presented with solitary and one with multiple subcutaneous masses measuring 5 to 9cm (median, 7.5cm). Affected sites were upper arm (3), shoulder (2), neck (1), trunk (1) and chest wall (1). Surgical excision was the treatment in all cases. No recurrence was recorded at last follow-up (1-17months). Submitted diagnoses were atypical lipomatous tumor (n=3), lipoma with regressive changes (n=1) and unclassified lipomatous neoplasm (n=2). In all cases, the striking variation in size of adipocytes was mentioned as the most or sole worrisome feature justifying external consultation. Histology was similar in all cases. All fulfilled the features reported by Evans as stated above and lacked any conventional spindle cell lipoma-like areas. Multi-vacuolated (lipoblast-like) cells were seen in three cases. MDM2/CDK4 were negative by immunohistochemistry and MDM2 amplification was absent by FISH in all cases. RB1 immunoexpression was lost in 5/5 cases. Rb1 FISH analysis revealed copy number aberrations in 3 of 4 cases (heterozygous deletions in two cases and homozygous deletion in one). In conclusion, ACL shares similar clinicopathological, demographic and molecular features as spindle cell lipoma suggesting related diseases. In the light of the available literature on adipocytic neoplasms in retinoblastoma survivors (>30 patients with multiple lipomas following retinoblastoma reported), it is probably that retinoblastoma-associated lipomas belong to the ACL category. Thus, it seems that Somatic RB1 loss probably drives sporadic ACL in a comparable way to post-retinoblastoma lipomas which were shown to be driven by LOH of the RB1 wild-type allele.

Chronic myelogenous leukemia initially presenting with multiple subcutaneous tumors due to extramedullary hematopoiesis

A 53-year-old woman was admitted with right upper-extremity pain and multiple subcutaneous masses. Bone marrow aspirate showed hypercellular marrow with increased myeloid components at all stages of maturation. Cytogenetic analysis of the bone marrow revealed 100% Philadelphia chromosome positivity along with BCR/ABL gene rearrangement, as demonstrated by polymerase chain reaction (PCR). A diagnosis of chronic phase of chronic myeloid leukemia (CML) was therefore made. Biopsy of one of the subcutaneous masses showed proliferation of granulocytes in various stages of differentiation. There were also erythroid cells and megakaryocytes, without p53 and CD34-positive blasts. These results suggested that the subcutaneous masses had developed from extramedullary hematopoiesis, not blastomas. The patient was administered dasatinib (DA) 140 mg, combined with radiation therapy for pain and peripheral neuropathy from the right axial extramedullary tumor. The patient showed complete hematological remission and the subcutaneous masses had disappeared 1 month after starting administration of DA. Because the patient did not achieve a cytogenetic response, the tyrosine kinase inhibitor nilotinib was administered. She will undergo allogeneic stem cell transplantation in the near future. Extramedullary hematopoiesis in the early stages of CML is uncommon. Our case emphasizes the need to elucidate the pathogenesis of extramedullary hematopoiesis in the early stages of CML.

A case of an 8-year-old boy who was strongly suspected of suffering from familial angiolipomatosis

Abstract Familial angiolipomatosis is a rare inherited disease mainly diagnosed in the adult stage. It is usually inherited in an autosomal recessive manner; however, some cases of autosomal dominant mode of inheritance have been reported. The disease may be misdiagnosed as neurofibromatosis type I in some cases due to the similar feature of multiple subcutaneous soft masses. However, familial angiolipomatosis is not associated with malignant tumors and it is very important to distinguish these two conditions. We report here the rare case of a subcutaneous mass that was composed of fat tissues and some fibrous tissue with small venous vessels and capillaries in an 8-year-old boy whose father also has a long history of generalized multiple angiolipomas. These facts strongly suggest that the patient suffers from familial angiolipomatosis and careful follow-up is needed in the future.

CASE REPORT: LOW DOSE CYCLOSPORINE COMBINED KETOCONAZOLE CURES A DOG OF STERILE NODULAR PANNICULITIS

An eight-year-old neutered male Cocker Spaniel with multiple subcutaneous masses on the right flank was presented to National Taiwan University Veterinary Hospital. The masses were excised and submitted for histopathologic evaluation and bacterial culture. A diagnosis of severe panniculitis was made and no microorganism was identified with bacterial culture. The patient did not respond well and showed significant side effects from prednisolone therapy. Later, low-dose cyclosporine was used instead and the lesions resolved. Complete remission was observed when ketoconazole was added in order to increase the blood concentration of cyclosporine in the patient. There were no adverse side effects noted during this combination therapy. The patient has remained free of the disease at the time this report was written. In conclusion, low-dose cyclosporine combined with ketoconazole therapy is safe and effective for treating sterile nodular panniculitis, when glucocorticoid does not provide an adequate clinical result.

Tuberous Sclerosis Complex Associated with Heterotopic Ossification in a Young Girl

Tuberous sclerosis complex (TSC) is a multisystem disorder resulting in hamartomatous lesions. Despite diverse manifestations, skeletal muscular comorbidity is rarely reported in TSC. We report a 2-year-old girl with TSC who suffered from multiple subcutaneous masses over the paraspinal and bilateral scapular areas, which caused disabling pain on any passive movement. Three-dimensional computed tomography scanning revealed multiple calcifications that were consequently diagnosed as fibrodysplasia ossificans progressiva. Such imaging features should be evaluated cautiously to avoid unnecessary surgical intervention and biopsy that may worsen the condition.

Cutaneous Hemangiosarcoma in a Dog

A male golden retriever of unknown age presented with multiple cutaneous and subcutaneous masses from the left elbow to the digits. Histopathologically, multiple tumor foci had formed from the dermis to the subcutaneous tissue. Tumor foci consisted of a vascular structure, alveolar structure and solid proliferative area. The borders among these areas were not clear. Some neoplastic cells resembled a mature endothelium, while others were large pleomorphic cells. Immunohistochemically, the tumor cells were usually strongly positive for CD31 and often positive for PROX-1, the lymphatic endothelial cell marker. Based on these findings, the tumor was diagnosed as a hemangiosarcoma with lymphatic differentiation.

Subcutaneous Panniculitis-Like T-Cell Lymphoma in Two Pediatric Patients: An HIV-Positive Adolescent and a 4-Month-Old Infant

Subcutaneous Panniculitis-like T-cell lymphoma (SPTCL) is a rare subtype of childhood non-Hodgkin lymphoma. Subcutaneous Panniculitis-like T-cell lymphoma has an aggressive variant associated with the hemophagocytic syndrome (HPS). Patients without HPS show resolution of the disease with prednisone or immunosuppressive therapy unlike other T-cell lymphomas. One HIV-positive adolescent and one infant with multiple subcutaneous masses are presented and the literature is reviewed. Lesional cells were consistent with SPTCL alpha-beta type. Our cases, without HPS, showed complete resolution of their lesions when treated with non-aggressive therapies. Patients with SPTCL alpha-beta should be treated conservatively.

Gardner's Syndrome

Gardner's syndrome is an autosomal dominant disease and is a subtype of familial adenomatous polyposis. It is characterized by adenomatous intestinal polyps, multiple osteomas in the skull, maxillae, mandible, and multiple cutaneous and subcutaneous masses (epidermoids and desmoid). Intestinal polyps, if not treated, have 100% chance of becoming malignant. We report a case of a 25-year-old female patient with Gardner's syndrome, with clinical manifestations including impacted supernumerary teeth, odontomes, sebaceous cyst on the scalp, and osteomas. It is important for the general dental practitioners to be aware of the clinical and radiological characteristics of Gardner's syndrome.

PET Appearance of Tuberculous Empyema Necessitans

Abstract: A 28-year-old man with no medical history presented with right-sided swelling of the chest. Findings from the CT scan revealed multiple subcutaneous cystic masses and cavitary lung lesions. FDG PET imaging demonstrated hypermetabolic cavitary lung lesions and cyst walls with no central uptake. Polymerase chain reaction fluid analysis confirmed the growth of Mycobacterium tuberculosis. Operative incision and drainage yielded 2.5 L of purulent material. First described in the 17th century, the hallmark presentation of empyema necessitans is anterior chest wall soft-tissue swelling. M. tuberculosis remains its most common cause. Extraparenchymal collections begin with tuberculosis pleuritis followed by caseous material eroding through the chest wall into fascial planes.

Puffiness of the eyelids and lips with multiple subcutaneous masses in 2-month-old infant

Hematol Oncol Stem Cell Ther 3(4) Fourth Quarter 2010 hemoncstem.edmgr.com 196 Skin involvement can be the initial sympf tom of hematologic diseases in children.1 Lymphoblastic leukemia/ lymphoma (LBL) is a neoplasm of the precursor Iymphoid cells considered to be the lymphomatotis variant of acute lymphoblastic leukemia (ALL). Distinction between LBL and ALL relies on the massive presence in the latter of blast cells in the bone marrow (>25% blasts) and in the blood. According to the Revised EuropeanfAmerican Classification of Lymphoid Neoplasms (REAL clasf silication), ALL and LBL account for 80% and 20% of neoplastic proliferation of lymphoblasts, respectively.2 LBL affects predominantly children and young adults with occasional older patients have been reported.3,4 A 2fmonthfold female presented with multiple subf cutaneous swellings on the scalp, shoulders and upper and lower limbs for the previous week. The swelling prof gressed to the eyelids and lips with intermittent stridor after receiving treatment for a common cold (Figure 1). Angioneurotic edema was suspected. She received stef roids treatment and frequent adrenaline nebulization, but the condition progressed. On examination, she apf Puffiness of the eyelids and lips with multiple subcutaneous masses in 2-month-old infant