Delayed Treatment Response in a Neonate with Multisystem Langerhans Cell Histiocytosis : Case report and review of literature

Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of the Langerhans cells, which are part of the mononuclear phagocytic system. The disorder varies in terms of the extent of the disease, its natural course and patient outcomes. While skin rashes are a common presentation of neonatal LCH, other systems or organs may also be involved. Delays in the diagnosis of neonatal LCH may occur due to its non-specific presentation and a lack of awareness of the condition among doctors. We report a two-month-old male neonate who presented to the Chacha Nehru Bal Chikitsalya hospital, New Delhi, India, in 2016 after the onset of pulmonary symptoms. He had been noted to have a generalised rash which had progressively worsened from 15 days of age. Following a skin biopsy and chest imaging, he was diagnosed with multisystem LCH with risk organ involvement. There was a delayed response to combined chemotherapy with no major side-effects

Intraoral Foregut Cystic Developmental Malformations: Three cases with a brief review of literature

Foregut cystic developmental malformations (FCDM) are a type of rare cystic lesion. The occurrence of FCDM is exceedingly uncommon in the intraoral location. We report three cases of FCDM with intraoral location who presented at Chacha Nehru Bal Chikitsalaya, New Delhi, India, in 2016, 2017 and 2018 with symptoms of respiratory distress and feeding difficulties. Two patients were male and one was female with an age range of 29 days to eight years. The clinical differential diagnosis included mucocele, ranula, dermoid, lymphangioma, teratoma, thyroglossal duct cyst, etc. All patients were treated with simple surgical excision and diagnosed, based on histopathology, with FCDM. These should be considered as differential diagnosis of head and neck midline cystic mass lesions. This case report aimed to discuss differential diagnosis and appropriate terminology for these cystic masses as there is varied and ambiguous nomenclature.Keywords: Bronchogenic Cyst; Cyst; Congenital Abnormalities; Oral Cavity; Case Report; India

Malignant melanoma of soft parts with osteoclast-rich giant cells: A rare tumor of the jejunum

Malignant melanoma of soft parts (MMSP), first described by Franz M. Enzinger, is a rare tumor of unknown cell origin. We describe a case of a 45-year-old male who presented with a 1-year history of abdominal pain, weakness, and anaemia. Computerized tomography enteroclysis showed a mass in the jejunum that was suggestive of a gastrointestinal stromal tumor. An ulceroinfiltrative lesion measuring 6.5 x 4 x 2 cm was identified. Microscopy revealed typical features of MMSP with numerous osteoclasts-like giant cells. The diverse histomorphology and immunohistochemical characteristics of this case involving a rare tumor at a rare site is presented.

Margins in skin excision biopsies: Principles and guidelines

Skin biopsies are usually undertaken to confirm a clinical diagnosis, to remove a lesion, and to determine the adequacy of excised tissue margin. A surgical margin is technically defined as the "edge" of the tissue removed. The term is especially pertinent when the tissue excised is suspected of being involved by a malignant process. One of the most important predictive and prognostic factors of a malignant lesion is whether the margins of the resected specimen are involved by the tumor or not. The purpose of this review is to provide an insight into grossing of a skin biopsy specimen with emphasis on techniques and reporting of excision biopsy margins

Metastatic adenocarcinoma of prostate in a 28-year-old male: The outcome is poor in young patients?

Prostate cancer is common in older patients. Rarity in younger population limits the study of natural history and prognosis in this population. Most of the published data has reported poor outcome in younger patients with metastatic prostate cancer. Here, we report a case of prostate cancer in 28-year-old male who presented with bone metastasis. After bilateral inguinal orchidectomy, he was started on anti-androgen therapy and received palliative radiotherapy for bone metastasis. There was only a slight decrease in prostate-specific antigen (PSA) level and pelvic disease post treatment. Subsequently, he was started on opioid analgesics (by World Health Organization, WHO, step ladder) in view of persistent pain. The index case is being presented for its rarity and probable poor outcome in young patients and to stress on the fact that the possibility of primary prostatic adenocarcinoma should be investigated in a male presenting with bone metastasis irrespective of the age

Delayed Treatment Response in a Neonate with Multisystem Langerhans Cell Histiocytosis: Case report and review of literature

Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of the Langerhans cells, which are part of the mononuclear phagocytic system. The disorder varies in terms of the extent of the disease, its natural course and patient outcomes. While skin rashes are a common presentation of neonatal LCH, other systems or organs may also be involved. Delays in the diagnosis of neonatal LCH may occur due to its non-specific presentation and a lack of awareness of the condition among doctors. We report a two-month-old male neonate who presented to the Chacha Nehru Bal Chikitsalya hospital, New Delhi, India, in 2016 after the onset of pulmonary symptoms. He had been noted to have a generalised rash which had progressively worsened from 15 days of age. Following a skin biopsy and chest imaging, he was diagnosed with multisystem LCH with risk organ involvement. There was a delayed response to combined chemotherapy with no major side-effects

Hemophagocytic lymphohistiocytosis in an infant with multiple triggers!!

Hemophagocytic lymphohistiocytosis (HLH) is a disorder of immune dysregulation secondary to a massive unregulated cytokine storm and its downstream consequences. HLH is being increasingly recognized as a cause of pyrexia of unknown origin, unexplained cytopenias, and hepatic dysfunction. However, this potentially treatable condition is often missed due to lack of suspicion, variable, and nonspecific presentations, inability to fulfil all the diagnostic criteria and availability of diagnostic tests in resource limited settings. Both familial and acquired forms of HLH can be triggered by multiple factors in a susceptible patient. We report a 9-month old infant who developed HLH in association with Stevens–Johnson syndrome following massive blood transfusion