Spindle Cell Lesions Research Articles

Extra-abdominal desmoid tumor located in the axilla.

Extra-abdominal desmoid tumor located in the axilla.

Symplastic Leiomyomas of the Scrotum

Smooth muscle tumors of the scrotum are very uncommon, and those with degenerative-appearing atypia, variably designated as "atypical," "symplastic," or "bizarre" leiomyomas, are extremely rare with only 11 cases in the literature. Given their rarity, the diagnostic criteria and prognosis of symplastic leiomyomas are not well established. We describe 9 cases of scrotal symplastic leiomyomas and compare their histopathologic characteristics to 10 usual leiomyomas and 5 leiomyosarcomas of the scrotum. The preoperative diagnosis was scrotal tumor or cyst in all cases. The mean age was 46 years (range, 32 to 60 y) for usual, 59 (range, 48 to 79) for symplastic leiomyomas, and 57 (range, 49 to 65) for leiomyosarcoma. Submitting diagnoses for symplastic leiomyomas were: atypical spindle cell lesion (n=3); probably leiomyosarcoma (n=1); leiomyosarcoma (n=1); and none given (n=4). Symplastic leiomyomas were diagnosed when there was moderate-severe cytologic atypia, yet was degenerative-appearing with multinucleation or smudged chromatin in the setting of low nuclear/cytoplasmic ratio, low cellularity, and no mitotic activity. The mean size was 1.0 cm for usual leiomyomas, 1.0 cm for symplastic leiomyomas, and 2.0 cm for leiomyosarcomas. Leiomyosarcomas had high nuclear/cytoplasmic ratio, high cellularity, nuclear pleomorphism, and hyperchromasia. Five of 10 usual and 3/9 symplastic leiomyomas showed at least 1 ill-defined border simulating infiltrative growth. Three leiomyosarcomas were grade 1, and 2/5 were grade 2. Resection margins were positive in 5/10 usual and 3/9 symplastic leiomyomas and in 1/5 leiomyosarcoma. Ki67 labeling in usual leiomyomas was on average 2.4% (range, 1% to 5%) and in symplastic leiomyomas was 1.8% (range, 1% to 5%). Mitoses were absent in all cases of usual and symplastic leiomyomas. Mitotic figures averaged 4.7 (range, 1 to 7) and 13.5 (range, 7 to 20) per 10 HPF for the grade 1 and 2 leiomyosarcomas, respectively. None of the symplastic leiomyomas recurred after a median follow-up of 27 months. The 2 patients with grade 2 leiomyosarcoma had no evidence of metastases at 6 and 7 months follow-up, respectively. Scrotal symplastic leiomyomas may have an ill-defined infiltrative border, which along with their atypia mimic malignancy. Ki67 is low in symplastic leiomyomas, which along with their favorable follow-up and experience in other organs justifies a benign diagnosis. High cellularity and high mitotic activity are the most reliable features for the diagnosis of scrotal leiomyosarcoma.

Improve Efficacy with Fine Needle Aspiration in Spindle Cell Lesions of the Upper GI Tract: A Comparative Study with Core Needle Biopsies

Improve Efficacy with Fine Needle Aspiration in Spindle Cell Lesions of the Upper GI Tract: A Comparative Study with Core Needle Biopsies

Fine Needle Aspiration Cytology in Diagnosis of Spindle Cell Lesions of Salivary Glands

lymphoma, 6 follicular lymphoma, and 2 marginal zone lymphoma. The overlap in cytomorphological features between low grade lymphoma and benign reactive lymphocytic lesions was the most challenging in the absence of flow cytometry studies, thus 2/6 follicular lymphomas included one non-diagnostic and the single false negative case which was misdiagnosed as a benign lymphoid process. The overall sensitivity, specificity, positive predictive value, negative predictive value, and accuracy of FNA of salivary glands for the diagnosis of lymphoma without ancillary studies in our study is 88%, 100%, 100%, 89%, and 94%, respectively. Conclusion: Our study demonstrates that even with the lack of on-site evaluation, and flow cytometry studies, a diagnosis of lymphoma based on FNA of a salivary gland can be rendered. However, the diagnostic accuracy is limited in cases of low grade lymphomas were the role of on-site evaluation and flow cytometry studies is most helpful.

Onsite cytopathology evaluation and ancillary studies beneficial in EUS-FNA of pancreatic, mediastinal, intra-abdominal, and submucosal lesions.

Endoscopic ultrasound-guided fine needle aspiration cytology (EUS-FNA) is considered to be a minimally invasive and safe technique, with low complication rates, for obtaining tissue samples from pancreatic lesions, and mediastinal and intra-abdominal nodes. We retrospectively reviewed the diagnostic accuracy of this method at a tertiary care hospital in Pakistan. The medical records of 393 consecutive patients referred to the gastroenterology service from August 2008 to September 2013 were reviewed in this retrospective study. The mean age of the patients at presentation was 51.2 years (range 6-89; 56.7% males). Major indications for referral were to establish a diagnosis in patients with a pancreatic mass or lymphadenopathy of unknown origin detected on CT scan or PET-CT (n = 235), to exclude lymph node metastasis in those with a known primary tumor (n = 114), and to rule out relapse of lymphoma after treatment or during follow up (n = 44). Of 393 patients, 181 had mediastinal lymphadenopathy, 115 had pancreatic lesions; 79 had intra-abdominal lymphadenopathy and 18 patients had miscellaneous lesions (gastric mass, splenic, retroperitoneal, esophageal, or adrenal lesions). A mean of 2.2 passes per patient were made; 85% of patients had ≤ 3 passes. The initial rapid onsite evaluation (ROSE) was positive in 363 of 393 (92.3%) patients, but on final cytopathological interpretation, 369 of 393 (93.9%) patients had an adequate specimen for diagnosis. Sufficient material for cytopathology was obtained in 369 patients (93.9%), whereas 24 patients (6.1%) had inadequate aspirate on EUS-FNA as assessed by final cytopathological interpretation. The concordance between final cytopathological diagnoses and ROSE was 98.2%. Follow-up data were available for 296 (75.3%) patients, whereas 97 patients were lost to follow up. Of these 296 patients, 283 of 296 patients were true positive, 9 of 296 were true negative, and 4 of 296 patients were false negative. The overall calculated sensitivity, specificity, positive predictive value, and negative predictive value were 98.6, 100, 100, and 69.2%, respectively. The major diagnoses that we obtained were granulomata (n = 114), adenocarcinoma (n = 104), reactive nodes (n = 35), metastatic carcinoma (n = 35), Hodgkin's lymphoma (HL; n = 19) and non-Hodgkin's lymphoma (NHL; n = 11), neuroendocrine tumors (n = 11), spindle cell lesions (n = 7), benign lesions (n = 9), cystic neoplasms (n = 6), solid pseudopapillary neoplasm (n = 3), and miscellaneous (n = 15). Only 3 of 393 patients (0.7%) suffered a complication as a result of EUS-FNA, and all three were successfully managed conservatively. EUS-FNA, combined with ROSE, is a safe and highly effective technique in experienced hands with excellent diagnostic accuracy and low complication rates.

Fine-needle aspiration cytology of histoid leprosy of nipple.

Sir, Histoid leprosy is considered to be a form of lepromatous leprosy and exhibits characteristic clinical and histological features.[1,2] Patients usually present with nodular lesions over the dorsum of hand, arms, lower back, chest, and buttocks. Diagnosis is usually confirmed on histological examination. Fine-needle aspiration cytology (FNAC) can be a rapid, safe, and effective alternative for diagnosis in a suspected case of leprosy and only a few studies have described this entity.[2,3,4,5] We report the cytomorphological findings that helped us to establish the diagnosis of histoid leprosy in a patient with nipple enlargement. A 40-year-old male patient was referred to the cytology clinic for FNAC of right nipple enlargement. On examination, the swelling of the nipple measured 1 cm in diameter. There was no overlying erythema, discoloration, or ulceration and no discharge. FNAC yielded blood mixed and particulate material. The smears were cellular and showed multiple clusters of oval to spindle shaped cells with elongated nuclei, fine chromatin and scanty to moderate cytoplasm [Figures 1a]. The background showed numerous histiocytes and lymphocytes. Figure ​Figure1b1b and ​and1c1c highlights the negatively stained organisms as singly scatlered or as bundles. The modified Ziehl-Neelson (Z-N) stain for Mycobacterium leprae was strongly positive, with a bacillary index of +6 [Figure 2]. A final diagnosis of histioid leprosy was made on the basis of these clinical and cytomorphological findings. Figure 1 (a) Discrete spindle cells and foamy histiocytes with unstained acid-fast bacilli highlighted as negative staining (May Grunwald Geimsa stain, ×high power), (b and c) Negatively stained organisms as single or narrow bundles (arrows) and thicker ... Figure 2 Abundant discrete and small bunches of acid fast lepra bacilli (Modified Ziehl–Neelson stain, ×Oil immersion) Histioid leprosy is a distinct form of leprosy characterized by nodular tumor like swelling of the subcutaneous tissue in apparently normal skin. The nodule is usually seen in arms, buttocks, thighs and over the knees. On histology section, the lesion shows many spindle shaped cells and foamy macrophages. Lepra stain shows a bunch of acid fast bacilli within the histiocytes and also extracellularly. Fine-needle aspiration cytology of histoid leprosy have been described by occasional studies.[2,3,5] Mehdi et al.[3] described that spindle shaped histiocytes forming whorl like arrangement along with many foamy cells are characteristic of histoid leprosy in FNAC smear. We also noted similar features in FNAC. There were many discrete spindle cells in the FNAC smear along with foamy macrophages. In addition, many rods like negative shadows were also noted. The negative images of the bunch of bacilli raised our suspicion of an infective etiology. Modified Z-N stain confirmed the diagnosis of leprosy [Figures ​[Figures1b1b and ​and1c].1c]. Without proper history, these spindle shaped cells may be mistaken as spindle cell lesions of soft tissue.[3] In brief, FNAC can be an excellent alternative to conventional skin biopsy for the diagnosis of histoid leprosy. It is safe, less invasive, rapid, and inexpensive and permits simultaneous sampling of multiple sites, thereby improving the overall diagnostic accuracy.

Inflammatory Myofibroblastic Tumours of the Urinary Bladder: Multi-Centre 18-Year Experience

Objective: To review a series of inflammatory myofibroblastic tumours (IMTs) of the urinary bladder in 10 hospitals in Hong Kong. Methods: A database search in the pathology archives of 10 hospitals in Hong Kong from 1995 to 2013 was performed using the key words ‘inflammatory myofibroblastic tumour', ‘inflammatory pseudotumour' and ‘spindle cell lesion'. Patient characteristics, clinical features, histological features, immunohistochemical staining results and treatment outcomes were reviewed. Results: Nine cases of IMT of the urinary bladder were retrieved. The mean age was 45.4 ± 22.8 years (range 11-78). Eight patients (88.9%) presented with haematuria and 5 patients (55.6%) had anaemia with a mean haemoglobin level of 6.8 ± 1.3 g/dl. Histologically, the majority of patients (77.8%) had a compact spindle cell pattern. Anaplastic lymphoma kinase staining was positive in 75% of cases. During a mean follow-up period of 43.4 months (range 8-94), none of them developed any local recurrence or distant metastasis. Conclusions: A high index of suspicion of IMT should be maintained for young patients presenting with bleeding bladder tumours and significant anaemia. IMTs of the urinary bladder run a benign disease course, and good prognosis can be achieved after surgical resection.

Cytoplasmic expression of Wilms tumor transcription factor-1 (WT1): A useful immunomarker for young-type fibromatoses and infantile fibrosarcoma

There is increasing evidence that Wilms’ tumor transcription factor-1 (WT1) is expressed in the cytoplasm of neoplastic cells from different benign and malignant tumors. Only a few studies on WT1 cytoplasmic immunolocalization are available in pediatric tumors. The aim of the present study was to investigate immunohistochemically the expression and distribution of WT1 in a large series of soft tissue fibroblastic/myofibroblastic lesions occurring in children and adolescents. Notably WT1 was not expressed in nodular fasciitis and desmoid-type (adult) fibromatosis, while it stained diffusely and strongly in several infantile-type fibromatoses, such as fibrous hamartoma of infancy, myofibroma/myofibromatosis, and lipofibromatosis. Interestingly, WT1 cytoplasmic expression was also found in all cases (10/10) of infantile fibrosarcomas examined. The present study shows that a diffuse WT1 cytoplasmic expression is of complementary diagnostic value to conventional myofibroblastic markers (α-smooth muscle actin; desmin) in confirming diagnosis of young-type fibromatoses or infantile fibrosarcoma and in ruling out both desmoid-type fibromatoses and nodular fasciitis. WT1 cytoplasmic expression in infantile fibrosarcoma is a novel finding which could be exploitable as an immunomarker for this tumor. Although highly sensitive, WT1 cytoplasmic immunostaining is not specific for infantile fibrosarcoma, and thus it should be evaluated in the context of a wide immunohistochemical panel when pathologists are dealing with spindle cell lesions of soft tissues in children and adolescents. Accordingly we recommend that a correct diagnosis of fibroblastic/myofibroblastic soft tissue lesion in pediatric patients is usually achieved on the basis of a careful correlation of morphological and immunohistochemical findings in the appropriate clinical context. The different cellular localization of WT1, namely nuclear, cytoplasmic or nucleo-cytoplasmic, in different benign and malignant tumors supports the hypothesis that this transcription factor plays a complex role in tumorigenesis, likely as a chameleon protein functioning as either a tumor suppressor gene or an oncogene, depending on cellular context.

Endometrial Stromal Sarcoma Involving The Urinary Bladder

Endometrial stromal sarcoma (ESS) involving the urinary bladder is very rare, with no prior series reported. We identified 6 cases of low-grade ESS involving the bladder at our institution (1998 to 2013), 5 of them consults. The median age at bladder involvement was 60 years (range, 44 to 77 y). One patient presented with bladder involvement at initial diagnosis of ESS. The remaining 5 cases with bladder involvement presented 7 to 30 years (mean 18 y) after a known diagnosis of ESS (n=2) or after a remote history of hysterectomy with an uncertain diagnosis (n=3). The location of bladder involvement included dome (n=1), trigone (n=2), diffuse (n=1), and unknown (n=2). Two cases demonstrated worm-like infiltrating tumor nests classic of low-grade ESS with little stromal reaction with retraction artifact mimicking vascular invasion. One case originating from the ovary showed focal glandular differentiation in the bladder, resembling endometriosis. Two cases had abundant keloidal collagen formation, arranged haphazardly or in a sunburst pattern. One case showed primitive cells infiltrating entirely hyalinized stroma, after chemotherapy given for a misdiagnosis of urothelial carcinoma. CD31 was negative in all cases, except for 1 case with obvious large vessel invasion. The differential diagnosis included a large nested variant of urothelial carcinoma, carcinoid tumor, synovial sarcoma, solitary fibrous tumor, Ewing sarcoma/primitive neuroectodermal tumors, and endometriosis. CD10 was strongly positive in 5 cases, and 1 case had very focal, moderate staining. Estrogen receptor showed strong and diffuse staining in all 6 cases. Progesterone receptor showed moderate to strong staining in 5 cases and focal staining in 1 case. One case showed PAX8 expression, and 2 cases showed p16 nuclear and cytoplasmic expression. CD56 showed weak to strong staining in 4 cases. Two cases had diffuse synaptophysin, and 1 case had focal p63 positivity. GATA-3, CD34, and CD99 were negative in all cases. The Ki-67 index was 1% to 10% (mean 4%). The mitotic count was 0 to 3/10 HPF (mean <1/10 HPF). Two patients had metastases to pelvic lymph nodes, and 1 had possible lung metastasis. Three patients were treated with Megace and 1 with Arimidex after surgery. Follow-up averaged 19 years (0 to 33 y) after the initial diagnosis of ESS or hysterectomy and 3.5 years (0 to 11 y) after bladder surgery. ESS involving the bladder is extremely rare with a very long interval from onset to bladder involvement. In female patients, low-grade spindle cell lesions involving the bladder should include ESS in the differential diagnosis.

Spindle-Cell Lesions of the Breast with Emphasis on the Differential Diagnosis

Compared to epithelial lesions, spindle-cell lesions of the breast are relatively uncommon and, because of that, may cause diagnostic difficulty. The majority of these spindle cell lesions, such as fibromatosis, nodular fasciitis, spindle- cell carcinoma, inflammatory myofibroblatic tumor and angiosarcoma, resemble their more common non-mammary counterparts. A few others, such as pseudoangiomatous stromal hyperplasia for example, specifically arise in breast. This review discusses these mammary spindle cell lesions with a focus on their salient histological features.

Immunohistochemical Markers in Fibrohistiocytic Lesions

The distinction between dermatofibroma (DF), dermatofibrosarcoma protuberans (DFSP), and other benign and malignant cutaneous spindle cell lesions frequently requires immunohistochemical staining. CD34 and factor XIIIa are the most commonly used immunostains; however, they may exhibit aberrant expression and introduce the potential for misdiagnosis. There is some data supporting that p75 and S100A6 may be additional helpful immunohistochemical markers. We undertook a large case series examining the use of CD34 and factor XIIIa as well as p75 and S100A6 in DF, cellular DF, DFSP, indeterminate fibrohistiocytic lesion, and scar. As expected, CD34 stained DFSP, although it was usually negative in DF. Factor XIIIa was generally positive in DF and negative in DFSP. There were exceptions in both cases of DF and DFSP. S100A6 was routinely negative in all entities studied. P75 was negative in all cases except DFSP, approximately half of which showed weak and/or patchy positivity. We conclude that to date, CD34 and factor XIIIa remain the most reliable immunohistochemical markers for DF and DFSP.

Cytopathologic diagnosis of Kaposi sarcoma in unusual clinical settings

Kaposi sarcoma (KS) is a rare disease that presents as 1 of 4 distinct clinicopathologic subtypes; however, it may present in populations outside those normally encountered. In such cases, it will be important to consider KS in the differential diagnosis, as it may mimic other neoplastic and non-neoplastic entities. We describe 2 cases of KS, 1 in a patient not clinically fitting any of the 4 subtypes and the other in a patient with atypical presentation in human immunodeficiency virus (HIV)-associated disease. The first is an 81-year-old African American (AA) woman with a history of KS of the leg, who presented with groin lymphadenopathy and the second is a 42-year-old AA man with a known history of HIV infection, no skin lesions, and new axillary lymphadenopathy. Fine-needle aspiration of the groin and axillary lymph node, respectively, showed atypical spindle cells in a lymphoplasmacytic background. The spindle cells were positive for human herpesvirus-8 on the cell block and subsequent lymph node excision. In patients with HIV infection, in addition to reactive and lymphoproliferative processes, KS should be considered. In the former case, the demographic of an elderly AA woman without immunosuppression would not cause concern for systemic KS, but for a metastatic tumor or lymphoma. Cytology is a helpful tool in narrowing the differential diagnosis for spindle cell lesions. With a diagnosis of KS, clinicians would be able to query the clinical history for a possible etiology, such as HIV, and exclude the possibility of metastatic disease.

Intranodal Leiomyoma in a Young Child: Report of a Rare Spindle Cell Lesion

ABSTRACT Primary spindle cell lesions of lymph nodes, with the exception of Kaposi's sarcoma, are rare. Intranodal palisaded myofibroblastoma has been described as a spindle cell tumor with prominent amianthoid fibers, intralesional hemorrhage, and intracellular or extracellular inclusions. Another spindle cell lesion, intranodal leiomyoma, has been reported only occasionally. We report the case of a 6-year-old boy with a mass in the neck without other systemic complaints. Excision biopsy of the lymph node revealed a spindle cell tumor with lymph nodal tissue at the periphery. The tumor showed features of smooth muscle differentiation with focally high mitotic index. The classical features of myofibroblastoma were not present. A final pathologic diagnosis of intranodal leiomyoma was rendered. The child has been free of recurrence in the follow-up period. Intranodal leiomyoma is a rare primary spindle cell lesion of the lymph nodes and should be considered in the differential diagnosis of the same.

Complex Cellular Composition of Solitary Fibrous Tumor of the Prostate

Solitary fibrous tumors (SFTs) of the prostate are a rare type of spindle cell neoplasm that can demonstrate either a benign or malignant phenotype. SFTs represent a clinical challenge along with other spindle cell lesions of the prostate in terms of both diagnosis and treatment. The present study shows, for the first time, that SFTs of the prostate and other organs can comprise a mixed population of fibroblast, myofibroblast, and smooth muscle cell types. The highly proliferative component demonstrated a fibroblastic phenotype that readily underwent myofibroblast differentiation on exposure to profibrotic stimuli. Consistent with other recent studies, the prostatic SFTs demonstrated NAB2-STAT6 gene fusions that were also present in the fibroblast, myofibroblast, and smooth muscle cell types of the SFT. The results of these studies suggest that benign and malignant prostatic tumors of mesenchymal origin may be distinguished at the molecular and cellular levels, and that delineation of such definingcharacteristics may help elucidate the etiology and prognosis of such tumors.

A case of diffuse infiltrating gastrointestinal stromal tumor of sigmoid colon with perforation

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms of the gastrointestinal tract, and typically present as discrete well-circumscribed but non-encapsulated tumor masses. In this report, we describe a case of colonic perforation caused by an unusual form of GIST. A 72-year-old Japanese woman presented to the emergency department with acute abdominal pain. Under the provisional diagnosis of sigmoid colon perforation, a laparoscopic sigmoidectomy was performed. Although the tumor mass was undetectable during the preoperative examination, a spindle cell lesion with a diffuse longitudinal growth pattern replacing the muscularis propria was revealed by microscopic examination. The spindle cell lesion was exposed at the perforation, suggesting a causal relationship between the lesion and the perforation. The spindle cell lesion was KIT-positive and had a mutation in the C-KIT gene at exon 11. We diagnosed it as diffuse infiltrating GIST. We consider that the lesion would be a cause of the colonic perforation, and emphasize the importance of accurate diagnosis of the lesion by histological, immunohistochemical and genetic examinations.

HER2-Positive Metaplastic Spindle Cell Carcinoma Associated with Synchronous Bilateral Apocrine Carcinoma of the Breast.

Apocrine carcinoma, which is strictly defined as over 90% of tumor cells showing apocrine differentiation, is a rare variant of breast cancer. Here we report an uncommon case in which apocrine carcinomas developed concurrently in both breasts; in addition, a sarcomatoid spindle cell lesion was coincident in the right breast. Both apocrine carcinomas were immunohistochemically negative for estrogen receptor (ER) and progesterone receptor (PgR), but diffusely positive for androgen receptor (AR), GCDFP-15, and HER2. The presence of intraductal components in bilateral carcinomas and the absence of lymph node metastasis suggested that they were more likely to be individual primary lesions rather than metastatic disease. The spindle cell lesion showed a relatively well-circumscribed nodule contiguous with the apocrine carcinoma. HER2 oncoprotein overexpression was observed not only in the apocrine carcinoma, but also in the spindle cell lesion. Since the spindle cell component was intimately admixed with apocrine carcinoma and had focal cytokeratin expression, we diagnosed it as metaplastic spindle cell carcinoma, which was originated from the apocrine carcinoma. To our knowledge, this is the first case report of a patient with synchronous bilateral apocrine carcinomas coinciding with metaplastic carcinoma.

Locally aggressive de novo spinal fibromatosis: case report and review of the literature

Fibromatosis is a benign lesion, which originates from proliferating fibroblasts. Although fibromatosis is a benign tumor, it is locally aggressive and invasive. Spinal presentation of fibromatosis is very rare, and case reports are the only source of the clinical knowledge. Herein, we describe the oldest patient having spinal fibromatosis with de novo occurrence and aggressive nature. A 68-year-old female patient admitted to our outpatient clinic with low back pain and neurological claudication. On lumbar spine magnetic resonance imaging, she had spinal canal stenosis with a concomitant lesion in the left longissimus muscle. We did our best to resect the tumor, entirely. The pathology result was consistent with spindle cell lesion. The lesion recurred 6 months after the primary surgery. In the second surgery, we did en bloc resection. Pathology and immunohistochemical analysis results were consistent with fibromatosis. After the second surgery, she had adjuvant radiotherapy. There was nothing other than radionecrosis in the operation site, still after 2 years. In conclusion, radiological work-up is usually insufficient to make fibromatosis diagnosis and whole pathological specimen should be carefully evaluated to achieve the accurate diagnosis. Spinal fibromatosis is very rare and its treatment methods have not universally been conceptualized. However, adjuvant therapies are necessary after en bloc resection.

2. Bilateral multiple nodules in lung: Epstein barr virus associated smooth muscle lesions

Background Presence of multiple nodules in lung is concerning for metastatic tumour and triggers prompt evaluation. Such scenario in a transplant/immunocomprimsed patient is further complicated since infectious aetiology and secondary malignancy need to be considered. Epstein-Barr Virus (EBV) associated smooth muscle tumours are rare lesions that occur in immuno-comprimsed patients. They are typically well-differentiated with low mitotic activity and little cytological atypia. They are believed to represent consequence of multiple infection events rather than metastasis. Methods We report a 51-year-old patient with a history renal transplant and immunosuppression whose surveillance showed multiple bilateral lung nodules. Transthoracic, CT guided fine needle aspiration smears and core biopsy materials were evaluated using light microscopy, immunohistochemistry and in situ hybridisation for EBV associated RNA. Results The fine needle aspiration smears showed rare, inconclusive stromal pieces. The core biopsy samples displayed spindle cell lesion with smooth-muscle features. There was mild cytological atypia without necrosis or mitotic activity. Immunohisto-chemical stains showed spindle cells were positive for SMA (smooth muscle actin), desmin, and vimentin. S-100 and MNF-116 (cytokeratin stain) were negative. In situ hybridisation for EBER showed nuclear positivity. Conclusions: EBV associated smooth muscle tumours can present as multiple bilateral lung nodules mimicking metastatic tumour. Histologically, they show spindle cell lesion with bland cytological features and low mitotic activity. Supportive ancillary findings by immunohistochemistry and in situ hybridization are as follows: (1) positive for SMA, desmin and vimentin, (2) negative for S-100 and epithelial marker MNF-116, and (3) positive for EBER.

Juvenile Hyaline Fibromatosis: A Rare Case with Recurrence

Juvenile hyaline fibromatosis (JHF) is a rare autosomal recessive disease. Less than 70 cases of JHF have been reported worldwide and extremely few from India. We present a case of 4-year-old girl who presented with multiple painless nodular masses on the scalp. On fine needle aspiration cytology, a diagnosis of benign spindle cell lesion was provided, the scalp lesions were excised and on histopathology the diagnosis of JHF was made. Retrospectively, the cytology slides were reviewed and the features were consistent with the diagnosis. A diagnosis of JHF was made based on the classical clinical features, Fine Needle Aspiration Cytology (FNAC) findings and the characteristic histopathological features along with a supportive evidence based on special stains. However, the patient had a recurrence within two years. Hence, we present this rare case with recurrence.

Intra–muscular Nodular Fasciitis Presenting as Swelling in Neck: Challenging Entity for Diagnosis

The diagnosis of Nodular Fasciitis is a very challenging on cytological aspirations. Even in histopathological sections, it can be sometimes be mistaken for soft tissue sarcomas due to its high cellularity, presence of mitotic figures, poor circumscription and degenerative myxoid areas. Most commonly, nodular fasciitis is seen on extremities; and sub-cutaneous form is most common. We are hereby reporting a case of nodular fasciitis at a rare site (head and neck area) in its rare form (intra-muscular type: originating within sternocleidomastoid muscle), which was clinically suspected as a malignancy arising from solitary thyroid nodule. Cytological diagnosis was issued as benign spindle cell lesion (where chances of a misdiagnosis as malignant are very high), thereby preventing radical surgery. It was subsequently confirmed as intra-muscular nodular fasciitis after histopathological examination.