Anita Nangia

Localized Tenosynovial Giant Cell Tumor of Tendon Sheath

BACKGROUND Localized tenosynovial giant cell tumor of tendon sheath (TGCT-L) is a benign, slowly growing lesion with a peak incidence in the third to fifth decade of life. It is thought to arise from the synovium of tendon sheaths, frequently affecting interphalangeal joints of the hands, feet, ankles and knees. Although the histopathologic appearances are well established, only a few reports describe the cytomorphology of this lesion. CASE A 37-year-old female presented with a slowly growing, nontender mass located near the left ankle joint. The cytologic features of localized tenosynovial giant cell tumor of tendon sheath (TGCT-L) include abundant mononuclear histiocytic cells occurring singly and in three-dimensional tissue fragments, hemosiderin within histiocytes and a few multinucleated giant cells. Subsequently, the histopathologic examination of the surgical specimen was proven to be TGCT-L. CONCLUSION Fine needle aspiration cytology can be used as a diagnostic tool for early and accurate detection of TGCT-L since the cytologic features combined with clinical details are sufficiently distinctive.

Clinico-Haematological Profile of Acute Megakaryoblastic Leukaemia: Report of Five Cases

Acute megakaryoblastic leukemia (AMKL) is a rare subtype of acute myeloid leukemia. Although known as a distinct entity for a very long time, because of lack of distinct clinical features and morphological criteria, it is difficult to diagnose this variant correctly. We herein present the clinical, morphological, cytochemical, and immunocytochemical features of five cases of AMKL. Certain morphological features such as presence of abnormal platelet count, giant platelets, and cytoplasmic blebbing in blasts were found to be important pointers towards the diagnosis. However, none of the features were found to be consistent and thus morphological diagnosis has to be confirmed by cytochemistry and immunocytochemistry.

Malakoplakia of bone: a case report

BACKGROUND Malakoplakia is an uncommon but distinctive granulomatous disease, characterized by an accumulation of histiocytes or Von Hansemann cells containing intracytoplasmic, laminated Michaelis-Gutmann bodies. CASE A 3-year-old male presented with a tender swelling in the left gluteal region that had been present for one month. Smears made from a fine needle aspirate showed large histiocytic cells containing intracytoplasmic, basophilic, laminated, targetoid Michaelis-Gutmann bodies resembling Von Hansemann cells in malakoplakia. Histopathology confirmed the diagnosis of malakoplakia of bone. CONCLUSION This case, histologically proven to be malakoplakia, demonstrated regression of the lesion following therapy. The characteristic cytologic features and presence of Von Hansemann cells may in themselves be diagnostic and obviate the need for biopsy.

Spindle cell lipoma masquerading as lipomatous pleomorphic adenoma: A diagnostic dilemma on fine needle aspiration cytology.

Spindle cell lipoma is a relatively uncommon benign adipocytic tumor that usually presents in subcutaneous fat of adult men. These are a rare form of lipoma, accounting for 1.5% of all lipomatous tumors, with a low rate of local recurrence and no risk of malignant behavior/dedifferentiation. Although few studies addressing the histological findings of spindle cell lipoma have been described, only a few descriptions of fine needle aspiration cytology (FNAC) findings have been documented in literature. We present a case of a 55-year-old male with a nodular swelling over left cheek (in the parotid region), which due to its location as well as prominent myxoid background prompted us to include the lipomatous salivary gland lesions in differential diagnosis. Our objective is to document and delineate the characteristic cytological features of spindle cell lipoma, which may permit a confident diagnosis on FNAC smears.

Segmental vitiligo with segmental morphea: An autoimmune link?

An 18-year old girl with segmental vitiligo involving the left side of the trunk and left upper limb with segmental morphea involving the right side of trunk and right upper limb without any deeper involvement is illustrated. There was no history of preceding drug intake, vaccination, trauma, radiation therapy, infection, or hormonal therapy. Family history of stable vitiligo in her brother and a history of type II diabetes mellitus in the father were elicited. Screening for autoimmune diseases and antithyroid antibody was negative. An autoimmune link explaining the co-occurrence has been proposed. Cutaneous mosiacism could explain the presence of both the pathologies in a segmental distribution.

Bilateral myoid hamartoma of breast: an exceptionally rare lesion.

Breast hamartomas (BH) or Fibroadenolipoma are extremely rare breast lesions. They are benign, focal malformations that resemble a neoplasm in the tissue of its origin. We describe a case of a 48 year old woman with bilateral breast lumps for the past 18 months and 6 months which were gradually increasing in size. FNAC smears revealed only clusters of benign ductal epithelial cells. Lumps were excised and histopathology revealed predominantly stromal smooth muscle bundles (proved by immunohistochemistry) along with scattered intact terminal duct-lobular units, dilated ducts and entrapped islands of mature fibroadipose tissue. A diagnosis of Bilateral Myoid hamartoma of breast was given. To the best of our knowledge till date no case of bilateral MH of breast has been reported in the literature. We report the first case of bilateral MBH highlighting its morpho-immunophenotypic features.

Myelofibrosis: a cause of increased transfusion requirement in a child with β-thalassemia intermedia.

A 10-year-old girl presented with gradually increasing pallor and abdominal distension for 1 year and fever for 6 months. She required 3 packed cell transfusions during this interval. Investigations done revealed that the child had thalassemia intermedia and parents were carrier of β-thalassemia gene. The increased transfusion requirement in this case prompted further investigations, which revealed the presence of myelofibrosis. Case reports of myelofibrosis and myelodysplastic syndromes with acquired hemoglobin disorders exist in literature, especially α-thalassemia. To the best of our knowledge, this is the first reported case of β-thalassemia intermedia in association with myelofibrosis.

Cilostazol: A novel agent in recalcitrant livedoid vasculopathy

Sir, A 25-year-old woman presented with recurrent erythematous, painful lesions over her lower extremities for 11⁄2 years, some of which turned into slow-healing ulcers. There was no history of preceding drug intake or constitutional symptoms. Examination revealed multiple small (0.5–1.5 cm), extremely tender, linear to angulated superficial ulcers with necrotic margins and pale bases over the lower third of both legs and around the ankles [Figure 1]. Ulcers were interspersed with erythematous to violaceous macules, stellate scars, focal induration and post-inflammatory hyperpigmentation, with associated mild pedal edema. Peripheral pulses were palpable.

Chronic idiopathic myelofibrosis with myeloid metaplasia presenting as refractory ascites.

Chronic idiopathic myelofibrosis (CIM) with myeloid metaplasia is a myeloproliferative disorder characterized by leukoerythroblastosis, tear drop erythrocytes, extra‐medullary hematopoesis (EMH), and varying degree of myelofibrosis. CIM, presenting as refractory ascites secondary to peritoneal hematopoesis, is extremely rare with only six adult cases reported in literature. This is a report of a child with CIM presenting as refractory ascites as a consequence of EMH in the peritoneum. The patient was treated with intermittent hydroxyurea with favorable response over 3 weeks. The patient was thereafter lost to follow up. Pediatr Blood Cancer 2010; 54:151–153.

Cutaneous angiosarcoma in a patient with systemic sclerosis: First case from India

Indian Journal of Dermatology, Venereology and Leprology | Volume 84 | Issue 2 | March-April 2018 214 6. Casrouge A, Zhang SY, Eidenschenk C, Jouanguy E, Puel A, Yang K, et al. Herpes simplex virus encephalitis in human UNC‐93B deficiency. Science 2006;314:308‐12. 7. Burrows J, Nitsche A, Bayly B, Walker E, Higgins G, Kok T, et al. Detection and subtyping of Herpes simplex virus in clinical samples by LightCycler PCR, enzyme immunoassay and cell culture. BMC Microbiol 2002;2:12.