Report of 2 Novel Presentations of Subcutaneous Fat Necrosis of the Newborn

Subcutaneous fat necrosis of the newborn (SCFNN) is a rare form of panniculitis classically affecting healthy full-term infants. There are a number of predisposing factors including perinatal asphyxia. The condition generally has a benign course with spontaneous resolution, but monitoring for metabolic complications, in particular the potentially life-threatening complication of hypercalcaemia, is critical. The authors report 2 cases of preterm infants with perinatal asphyxia with atypical presentations of SCFNN: the first with bony involvement resembling Langerhans cell histiocytosis and with follicular pseudocarcinomatous hyperplasia on histology; and the second presenting with a huge haematoma requiring surgical debridement. Both cases were initially erroneously diagnosed as pyogenic infections.Dermatopathology 2019;6:147 –152
Source: Dermatopathology - Category: Pathology Source Type: research

Related Links:

We report the case of a 68-year-old woman with a cranial pharyngeal tumor and a bone lesion in the tibia. The case was diagnosed as ECD. Pathological analysis showed the typical feature of foamy macrophage accumulation. The macrophages were positive for CD68, and negative for CD1a and S100. The BRAF V600E mutation was identified. In addition, immunohistochemistry was performed for the detailed characterization of the macrophages. The macrophages had low proliferative activity and an M2-like phenotype, and they expressed colony-stimulating factor-1 receptor (CSF1R) on the cell surface. PMID: 33028762 [PubMed - as supplied by publisher]
Source: Journal of Clinical and Experimental Hematopathology : JCEH - Category: Hematology Tags: J Clin Exp Hematop Source Type: research
Conclusion: Combination of neoadjuvant therapy and LT is an effective paradigm in treatment of multisystem LCH with severe liver dysfunction. With advances in chemotherapy regimen for multisystem LCH and LT surgery, perspectives on prognosis for LCH children are promising.
Source: Frontiers in Oncology - Category: Cancer & Oncology Source Type: research
We present an unusual case of aggressive HS presenting in the gastrointestinal tract and gallbladder that progressed after several lines of chemotherapy with a leukemic phase. We review the clinical, pathological, and molecular characteristics of HS in this case and review the literature on HS involving the digestive system as well as on overt leukemic phase of this disease. HS is often diagnosed at an advanced stage, and mortality is high. We discuss the therapeutic approach to patients with HS. We highlight the role of overexpression and somatic alterations in the RAF-MEK-ERK pathway in the pathogenesis of HS and discuss...
Source: Acta Haematologica - Category: Hematology Source Type: research
We present an unusual case of aggressive HS presenting in the gastrointestinal tract and gallbladder that progressed after several lines of chemotherapy with a leukemic phase. We review the clinical, pathological, and molecular characteristics of HS in this case and review the literature on HS involving the digestive system as well as on overt leukemic phase of this disease. HS is often diagnosed at an advanced stage, and mortality is high. We discuss the therapeutic approach to patients with HS. We highlight the role of overexpression and somatic alterations in the RAF-MEK-ERK pathway in the pathogenesis of HS and discuss...
Source: Acta Haematologica - Category: Hematology Authors: Tags: Acta Haematol Source Type: research
This report extends the spectrum of composite hematolymphoid neoplasms and shows thatALK rearrangement should be considered in all histiocytosis subtypes. Moreover, both tumours eradication under ibrutinib suggests that BTK inhibitors may also be effective in histiocytic neoplasms.
Source: Virchows Archiv - Category: Pathology Source Type: research
Anuj SarmaIndian Journal of Anaesthesia 2020 64(10):909-911
Source: Indian Journal of Anaesthesia - Category: Anesthesiology Authors: Source Type: research
We describe here the clinical details of a 2-year-old girl with involvement of unusual sites like the parotid glands and the nails. This child also had multiple cystic lesions in the liver leading to a misdiagnosis of Caroli’s disease. Knowledge about the uncommon manifestations of this rare disorder helps in early diagnosis and treatment.
Source: Journal of Pediatric Hematology Oncology - Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research
We described the case of a 17-year-old girl affected by anorexia nervosa, depression and self-harm with incidental detection of moderate hypercalcaemia. Clinical, laboratory and instrumental tests demonstrated that hypercalcaemia was secondary to primary hyperparathyroidism (PHPT) due to a mediastinal parathyroid adenoma in the thymic parenchyma. After parathyroidectomy with robot-assisted surgery, we observed the restoration of calcium and PTH levels in addition to an improvement in psychiatric symptoms. This case demonstrates that serum calcium concentration should be evaluated in adolescents with neurobehavioural sympto...
Source: Frontiers in Endocrinology - Category: Endocrinology Source Type: research
Abstract Langerhans-cell histiocytosis (LCH) is a rare disease of unknown pathogenesis. To date, there is a lack of FDA-approved treatments in adult LCH to establish optimal first line therapy. We conducted a retrospective, single-center case series evaluating the use of BRAF-inhibitors in adult patients with biopsy proven BRAFV600E - LCH. Our case series is the first to report the use of BRAF-inhibitors as first-line therapy in adults with LCH. We also report the efficacy with single-agent dabrafenib in adult LCH. All but one of our patients had favorable response to targeted therapy. PMID: 32985015 [PubMed ...
Source: The Oncologist - Category: Cancer & Oncology Authors: Tags: Oncologist Source Type: research
ConclusionsLangerhans cell histiocytosis remains an exceedingly rare entity in adults, frequently presenting as multisystem disease with risk organ involvement. Langerhans cell sarcoma represents an aggressive subtype with extremely poor prognosis for which intensive acute myeloid leukemia induction should be strongly considered.
Source: Journal of Medical Case Reports - Category: General Medicine Source Type: research
More News: Dermatology | Histiocytosis | Hypercalcaemia | Langerhans Cell Histiocytosis (LCH) | Pathology | Perinatology & Neonatology